First Trimester Medical Termination of Pregnancy

Rajshree Dayanand Katke
Department of Obstetrics and Gynecology, Medical Superintendent, Cama and Albless Hospital, Grant Government Medical College, Sir J. J. Group of Hospitals, Mumbai, Maharashtra, India.

For Article Link:
https://www.researchgate.net/publication/266319267

 

Atypical presentation of uterine choriocarcinoma a case report with review of literature

Rajshree Dayanand Katke
Department of Obstetrics and Gynecology, Medical Superintendent, Cama and Albless Hospital, Grant Government Medical College, Sir J. J. Group of Hospitals, Mumbai, Maharashtra, India.

Volume 4, Issue 6, November-December 2015
Clinical Cancer Investigation Journal
An official publication of the Middle-Eastern Association for Cancer Research

DOI: 10.4103/2278-0513.169114

ABSTRACT
Gestational trophoblastic diseases include complete and partial molar pregnancy, invasive mole, placental site trophoblastic tumor,and choriocarcinoma. Gestational choriocarcinoma is one of the most malignant form of a group of tumors. Although choriocarcinoma has a very high propensity to metastasize to various sites including brain it also has a very high cure rate. Our study represents the case of choriocarcinoma developed after primary invasive mole which was treated successfully with combined surgical and medical management. We now present a course of gestational trophoblastic disease transforming from benign to a malignant condition in due course of management. We are presenting a case of a 26-year-old female who came to us with molar pregnancy which turned out be invasive mole on histopathological examination and converted into choriocarcinoma in due course of treatment even after methotrexate chemotherapy.
Key words: Chemotherapy, choriocarcinoma, gestational trophoblastic disease, invasive mole

INTRODUCTION
Choriocarcinoma also known as chorioblastoma or trophoblastic tumor is a rare form of cancer which occurs in the female genital tract and is commonly associated with pregnancy. It may develop after a normal pregnancy; however, it is usually associated with molar pregnancy, ectopic pregnancy, miscarriage, or abortion. Gestational choriocarcinoma is highly malignant tumor with a very high propensity to metastasize to various sites including lungs, vagina, brain, liver, kidney, and gastrointestinal tract, in descending order of frequency. These cases present with vaginal bleeding, anemia, hyperemesis gravidarum,hyperthyroidism, uterine and ovarian enlargement, and pregnancy-induced hypertension.
The ultimate cause of gestational trophoblastic disease is claimed to be genetic in origin. No environmental etiological factor has been implicated up to now apart from deficient Vitamin A precursor carotene in the diet.[1]
The precise molecular pathogenesis of gestational trophoblastic disease is yet to be elucidated. Genetics has a well-established role.

CASE REPORT
A 26-year-old female married for 9 years G3 P2 L2 with 6 weeks gestation came with complains of bleeding per vaginum since 2 days and pain in abdomen on and off since 1-month. Patient was thin built, vitals were normal,mild pallor with no evidence of any other abnormality found on general examination. Her systemic examination revealed normal findings of cardiovascular and respiratory
system. Perabdominal examination, abdomen was sof with no guarding, tenderness or rigidity. Perspeculum examination, the cervix was congested with minimal bleed and per vaginum examination suggestive of uterine size of 10–12 weeks size and fornices were free. Patient’s ultrasound examination done was suggestive of incomplete abortion. Patient was taken for curettage, and the grossly product of conception appeared to be multiple fluidfilled vesicles, specimen was sent for histopathological examination, which came out to be vesicular mole, with few interspersed myometrial cells suggesting the diagnosis of invasive mole. Patient’s beta human chorionic gonadotropin (B-HCG) levels were more than 200,000 IU/ ml. Patient was kept under observation and serial B-HCG monitoring done, which reduced gradually to 130,000 mIU/ml after 15 days of curettage. As the patients B-HCG levels were in high range, patient was started on prophylactic methotrexate chemotherapy and was given five doses of 50 mg methotrexate with alternate day of folinic acid injections. Postmethotrexate completion the B-HCG levels were 71384 mIU/ml, after 15 days it became 23937 mIU/ml. Postoperative ultrasound showed no evidence of retained products of molar pregnancy and the ovaries also were normal.
Her thyroid-stimulating hormone value was in the range of 0.05 mIU/ml (normal range: 0.3–5.5), hence patient was started on antithyroid drug with serial thyroid profile monitoring.
After 25 days postchemotherapy, the patient suddenly presented with severe bleeding per vaginum and anemia with hemoglobin level of 6.5 g%, hence patient was given 2 units of blood transfusion. B-HCG levels were 49825 mIU/ml. Bulky uterus with fundal mass of 3 × 2 cm size.
Patient was started on the second cycle of chemotherapy with methotrexate, after four doses of methotrexate B-HCG level was 28069 mIU/ml. The ultrasound with color Doppler scan showed entire myometrium replaced by large vascular spaces with supply from the right uterine artery and flow simulating arteriovenous shunting with no surrounding adherence which is suggestive of recurrence or flared up lesion. All these findings were exactly similar to prechemotherapy findings [Figure 1]. Her magnetic resonance imaging findings were highly vascular fundal mass in the endometrial cavity with myometrial invasion reaching up to serosa at some places with no extraserosal spread. Fat planes with surrounding organs were preserved [Figure 2].
Meanwhile the patient’s thyroid profile came to be within normal range hence antithyroid drugs were tapered. As patient appeared to be not responding to treatment and continuously symptomatic and bleeding per vaginum, possibilities of invasive mole or choriocarcinoma explained to the relative. As there was no satisfactory response to chemotherapy and patient had heavy bout of bleeding the decision of hysterectomy was taken. Total abdominal hysterectomy was done under spinal anesthesia. Intraoperative findings were flabby, soft uterus with many blood filled spaces subserosally, no extraserosal spread apparent. Rest of the pelvic organs were normal. Ovaries were normal looking. On gross,the specimen showed bulky, soft, and congested uterus [Figure 3].

                                                Figure 1: Colour Doppler of uteerus (original)

                                                        Figure 2: MRI PELVIS (original)

                                            Figure 3: Gross appearance of specimen (original)

On cut section, it showed polypoidal mass of 2 cm × 2 cm arising from the fundal area of the uterus approaching into the uterine cavity. Histological picture shows the groups of cytotrophoblast and syncytiotrophoblast interspersed with myometrium with areas of necrosis and hemorrhage. Histopathological report confirmed the diagnosis of choriocarcinoma [Figure 4].
Based on the medical oncologist’s consultation the patient received two cycles of chemotherapy, postchemotherapy B-HCG level came out to be 6.0 mIU/ml patient withstood well with surgery and recovered well. Patient discharged from hospital. At present, she is living normal and healthy life. Follow-up B-HCG levels are in normal range and ultrasound showed healthy postoperative status with normal thyroid values.

DISCUSSION
Gestational trophoblastic neoplasia almost always develops with or follows some form of recognized pregnancy. Most follow a hydatidiform mole but neoplasia may follow pregnancy, abortion, or even an ectopic pregnancy.[2,3]
Choriocarcinoma also known as chorioblastoma or trophoblastic tumor is a rare form of cancer which occurs in the female genital tract and is commonly associated with pregnancy. It may develop after a normal pregnancy; however, it is usually associated with molar pregnancy, ectopic pregnancy, miscarriage, or abortion. Invasive mole is locally invasive, but generally lack the pronounced tendency to wide spread metastasis.
Choriocarcinoma is extremely malignant tumor with the incidence of 1 in 30000 pregnancies and only one-third of it develops after a normal delivery and one-third after molar pregnancy. Metastasis often develops early in choriocarcinoma and is generally blood borne and most common sites are lungs and vagina. Ovarian theca lutein cysts are identified in one-third of such cases. Placental site trophoblastic tumors are a rare variant characterized by prolactin producing intermediate trophoblast with relatively low B-HCG, a high proportion of free B-HCG, chemo resistant, and hysterectomy, being the best treatment. Epithelioid tumors are rare characterized by nonconformation of preceding pregnancy, nodular growth, and microscopically resembles placental site tumors, but the cells are smaller and display less pleomorphism. Invasive mole is associated with multiple complications such as hyperthyroidism, hyperemesis gravidarum, and preeclampsia, but early diagnosis and intervention helps in the prevention of these complications.

                                   Figure 4: Histological appearance of choriocarcinoma (original)

Preoperative X-ray chest hemogram, baseline B-HCG,blood grouping, liver enzymes done routinely before suction Evacuation.[4] It is also an important adjunct to the treatment of chemo-resistant tumors. Chemotherapy is now the established method of treatment of choriocarcinoma and hysterectomy and surgical resection of the tumor is rarely required in cases resistant to chemotherapy.[5,6] Nongestational choriocarcinoma also occurs and is usually resistant to therapy.[7]

CONCLUSION
Our case report emphasizes that persistent trophoblastic disease needs to be defined precisely and also the judicious use of methotrexate with surgical intervention at proper time in management of persistent trophoblastic disease is the key to 100% survival in gestational trophoblastic neoplasia. Furthermore, early diagnosis by ultrasound and histopathological examination is the key to avoid associated complications such as hyperemesis gravidarum, hyperthyroidism, and preeclampsia. Our case also proves that the clinical presentation of hydatidiform mole has changed in recent years and fewer current patients as compared to historic control presented with traditional symptoms of molar pregnancy (large uterine size, hyperemesis gravidarum, anemia, preeclampsia, and hyperthyroidism).

Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.

REFERENCES
1. Kajii T, Ohama K. Androgenetic origin of hydatidiform mole.Nature 1977;268:633-45.
2. Cortés-Charry R, Figueira LM, García-Barriola V, Gomez C, Garcia I, Santiago C. Gestational trophoblastic disease in ectopic pregnancy: A case series. J Reprod Med 2006;51:760-3.
3. Nugent D, Hassadia A, Everard J, Hancock BW, Tidy JA. Postpartum choriocarcinoma presentation, management and survival. J Reprod Med 2006;51:819-24.
4. Soto-Wright V, Bernstein M, Goldstein DP, Berkowitz RS. The changing clinical presentation of complete molar pregnancy. Obstet Gynecol 1995;86:775-9.
5. Berkowits RS, Goldstein DP. The management of molar pregnancy and gestational trophoblastic tumors. In: Knapp RC, Berkowitz RS, editors. Gynecologic Oncology. 2nd ed. New York: McGraw-Hill; 1993. p. 328-38.
6. Goldstein DP, Berkowitz RS. Current management of complete and partial molar pregnancy. J Reprod Med 1994;39:139-46.
7. Berkowitz RS, Cramer DW, Bernstein MR, Cassells S, Driscoll SG, Goldstein DP. Risk factors for complete molar pregnancy from a case-control study. Am J Obstet Gynecol 1985;152:1016-20.

For Article Link:
https://www.researchgate.net/publication/283838565

A rare presentation of heteropagus conjoined twin: a case report with review of literature

Rajshree Dayanand Katke
Department of Obstetrics & Gynaecology, Cama and Albless Hospital, Grant Government Medical College and Sir J.J Group of Hospitals, Mumbai, Maharashtra, India



Correspondence:
Dr. Rajshree Dayanand Katke,
E-mail: drrajshrikatke@gmail.com

Received: 04 March 2015     Accepted: 19 April 2015
DOI: 10.18203/2320-1770.ijrcog20150108

ABSTRACT
Conjoined twins are a rare occurrence with an incidence of about 1 in 100000 pregnancies. Our case is that of a 32 year old multigravida patient who came to us at 17 weeks gestation with a scan done at 14 weeks suggestive of severe spinal dysraphism with lumbar meningomyelocele spina bifida at multiple levels with protuberant abdomen. The patient opted for medical termination of pregnancy which was carried out after due to consent. The patient aborted after 12 hours. Further inspection of the abortus revealed it to be conjoined twins with one apparently normal head and thorax with a large omphalocele with 4 lower limbs. There were two caudal halves along with two sets of lower limbs and external genitalia arising from the abortus perpendicular to it on either side of the thorax. One set of arms was attached to the thorax and yet another arm and a limb bud were arising from the other end of the fetus attached to a rudimentary head like structure. Heteropagus tetrapus twins are an extremely rare form of parasitic twins. Inspite of advances in surgical techniques and methods, the morbidity endured by the affected neonates is very high. Thus prenatal diagnosis of conjoined twins along with the option of termination of pregnancy is of utmost importance.
Keywords: Heteropagus, Conjoined twins, Parasitic twins, Prenatal diagnosis

INTRODUCTION
Conjoined or Siamese twins are the rarest form of monochrionic monoamniotic twins. The incidence of conjoined twins worldwide ranges from 1 in 50000 to 1 in 100,000 with a current prevalence of 1.47 in 100000 births1 with a slightly higher incidence in Southwest Asia, Africa and Brazil.2 Conjoined twins are usually of the symmetrical variety but at times they may be asymmetrical called heteropagus or parasitic twins.Parasitic twins constitute only 4.5% of all conjoined twins3 of which epigastric heteropagus twins are the rarest of them all. The parasitic twin usually has supernumerary limbs without a functional heart or brain.4 The pathogenesis and blastogenesis of parasitic twins is not clearly understood as of yet.

CASE REPORT
A 32 year old gravida 3 para 2 living 1 death 1 patient came to the out-patient department of Cama and Albess hospital, Mumbai with 17 weeks gestation with a sonogram done at 14 weeks suggestive of spinal dysraphism opting for medical termination of pregnancy. A repeat Sonogram was done at our institute which revealed a fetus with severe spinal deformities. The fetus had lumbar meningomyelocele and spina bifida at multiple levels with lumbar lordosis and a protuberant abdomen. The patient was married since 8 years and had one living female child of 6 years of age followed by an intrauterine fetal demise at full term 2 years back. She had no significant obstetric complications or any major medical or surgical illness in the past. There was no family history of twinning in either parent. General and systemic examination was carried out. The patient was vitally stable with a 16 weeks sized uterus per abdomen and per vaginum and a patulous cervical os.

After consultation with the pediatrician as well as the pediatric surgeon, the patient and her husband decided to opt for termination of pregnancy. The patient was admitted and all routine investigations were carried out. The patient’s hemoglobin was 10.8 gm%, blood group was B positive and all other investigations were within normal limits. The patient was negative for HIV, hepatitis B, and VDRL. Medical Termination Pregnancy was done with detailed consent. Induction was done with dinoprostone gel followed by injection Pitocin augmentation. The patient aborted after 12 hours uneventfully and completely.

Further inspection of the abortus revealed an apparently normal head with a thorax with two normally formed arms and an omphalocele. The abortus had two caudal ends each with a set of male genitilia and completely formed lower limbs arising laterally from either side of the thorax. One of the caudal ends also had spina bifida. There was yet another arm and a limb bud arising from a rudimentary head like structure at the other end of the fetus. An X-ray was done showing the normal skull of 1 fetus from which arose 1 set of ribs and thoracic vertebrae and two normally formed upper limbs. The lumbar vertebrae continued almost at perpendicular from the right side of the fetus with one set of pelvic bones and completely formed lower limbs with evidence of spina bifida. There was yet another set of lumbar vertebrae, pelvic bones and lower limbs which was fused to the left side of the fetus and one arm arising from the other end of the fetus. There was no evidence of another cranium or thoracic cage. There was a single placenta and cord which did not have any gross abnormalities. The abortus was sent for histopathology which revealed single liver and G-I tract that had herniated into an omphalocele. There was one set of lungs and heart. The parasitic fetus did not have a cranium or thorax and was fused at the epigastrium on left side of the normal fetus.

                                                     Figure 1: Anterior view of the abortus.

                                                    Figure 2: Posterior view of the abortus.

The patient was stable post abortion and was managed with antibiotics. She and her husband refused any further investigations due to financial constraints and she was discharged from the hospital on the third day.

                                                      Figure 3: X-ray of abortus; AP view.

                                                    Figure 4: X-ray of the abortus; PA view.

                                               Figure 5: Pathological dissection of the abortus.

                                          Figure 6: Organs identified from the abortus and sent
                                                        for histopathology.

DISCUSSION
The etiopathogenesis of conjoined twins is not clear till today. Although there are two theories of which one is of incomplete fission due to division of the blastocyst beyond the 13th day of fertilization. The other theory which is more accepted today is that of fusion,5 in which the fertilized eggs are completely separated but stem cells find like stem cells on the other twin and fuse the twins together.6 There have also been reports of conjoined twinning consistent with fusion of two embryos during IVF7 thus recommending cytogenetic testing of all conjoined twins .Conjoined twins maybe symmetrical or asymmetrical. Symmetrical conjoined twins have two identically formed twins which are fused to each other at a certain site on both bodies. They may share viscera at that site and the nomenclature is according to the site of fusion i.e. thoracopagus (fusion at chest; most common variety), omphalopagus (fusion at anterior abdominal wall),pyopagus (Buttocks/sacrum),ischiopagus (Pelvis/ischium), craniopagus (Cranium) or a combination of any of the above such as thoracoompalopagus or ischio-pyopagus.
 Asymmetrical conjoined twins are called as heteropagus or parasitic twins in which the ‘parasite’ is not completely formed and is attached to the normally formed foetus or the ‘autosite’ at a certain point. The parasite is not completely formed and may only be evident by supernumerary limbs. The spectrum of heteropagus twinning may present depending on the site of union and the extent of damage of one embryo resulting in (1) an externally attached parasitic twin, (2) an enclosed fetus in fetu, (3) an internal teratoma, or (4) an acardiac connected via the placenta.1 It is believed that heteropagus twins occur because of some form of ischemic insult in early gestation that leads to selective atrophy and resorption of the cranial part of one of the monozygous twins. Of all the heteropagus twins, epigastric heteropagus twins are among the least
documented cases in literature. The parasite usually does not have a functional brain or heart and does not share the host twin’s G-I tract or liver but may have a rudimentary G-I tract or Urinary system of its own with connection to that of the host.8 Thus surgical separation can be done safely in such patients.
The prenatal diagnosis of conjoined twins is of utmost importance. The diagnosis is usually done by a good sonogram between 14-16 weeks or if necessary an MRI to confirm diagnosis.9 However the diagnosis can be completely missed as was in our case where termination was done for other congenital anomalies. The delivery of conjoined twins beyond 20 weeks irrespective of the type is usually done by Caesarean section to avoid invariable obstruction of labour and its complications which will occur if trial of vaginal delivery is given.

CONCLUSION
The theory behind conjoined twinning is as of yet not understood. Hence it is recommended that all cases of conjoined twins should be reported in literature. There have been numerous case reports and studies of conjoined twins both homopagus and heteropagus twins being delivered at term and undergoing surgical separation of the twins or successful excision of the parasitic twin.However this requires a multidisciplinary approach with good obstetric, anaesthetic, paediatric surgery and NICU setup. Inspite of advances in surgical techniques and resources today, the medical and surgical management of conjoined twins still has chances of great morbidity and mortality to the neonates and also a great financial as well as emotional burden to the parents. Hence early prenantal diagnosis of conjoined twins is of utmost importance and termination of pregnancy should be offered to all affected mothers as an option if detected on time.

Funding: No funding sources
Conflict of interest: None declared
Ethical approval: Not required

REFERENCES
1. Spencer R. Parasitic conjoined twins: External, internal (fetuses in fetu and teratomas), and detached
(acardiacs). Clin Anat. 2001;14:428.
2. Carnevale FC, Borges MV, Affonso BB, Pinto RA, Tannuri U, Maksoud JG. Importance of angiographic
study in preoperative planning of conjoined twins: case report. Clinics (Sao Paulo). 2006;61(2):167-70.
3. Edomonds LD, Layde PM. Conjoined twins in the United States, 1970-1977. Teratology. 1982;37:111-8
4. Cunningham FG, MacDonald PC, Gant NF.Williams’s obstetrics. In: Cunningham FG, MacDonald PC, Gant NF, eds. A Book. 23rd ed. New York: McGraw-Hill; 2010.
5. Machin GA. Conjoined twins: implications for blastogenesis. In: Opitz JM, Paul NW, eds. Blastogenesis: Normal and Abnormal. 9th ed. New York: Wiley-Liss; 1993: 141-75.
6. Le Tao, Bhushan Vikas, Vasan Neil. First aid for the USMLE step 1. In: Le Tao, Bhushan Vikas, Vasan
Neil, eds. A Book. 20th Anniversary ed. USA: The McGraw-Hill Companies, Inc.; 2010: 121.
7. Logrono R, Garcia-Lithgow C, Harris C, Kent M, Meisner L. Heteropagus conjoined twins due to fusion of two embryos: report and review. Am J Med Genet. 1997;73:239-43.
8. Abubakar AM1, Ahidjo A, Chinda JY, Tahir C, Abubakar S, Adamu SA, et al. The epigastric heteropagus conjoined twins. J Pediatr Surg. 2011;46(2):417-20.
9. Chen Pei-Lin, Kyuran Ann Choe. Prenatal MRI of heteropagus twins. Am J Roentgenol. 2003;181(6):1676-8.

A Huge Retroperitoneal Neurofibroma mimicking broad ligament fibroid in postmenopausal women: An extremely rare report with review of literature

Rajshree D. Katke
Department of Obstetrics & Gynaecology, Cama & Albless Hospital, Govt. Grant Medical College Mumbai, Maharashtra, India

Correspondence:
Dr. Rajshree D. Katke,
E-mail: drrajshrikatke@gmail.com


SEAJCRR SEPT-OCT 4(5) ISSN: 2319-1090

ABSTRACT
The neurofibroma is a tumour of neural origin. This kind of neoplasm, though, is
generally skin located. Rare cases in deep organs or in the peritoneal cavity are also reported in
the literature. There are two types of neurofibromas, localized and diffuse; the latter is associated
with von Recklinghausen disease and always occurs together with skin neurofibromas. Here we
report the case of a 62-year-old woman affected by neurofibroma, but not associated with von
Recklinghausen disease.
A computed tomography (CT) scan described a retroperitoneal pararenal lesion with no
clear involvement of adjacent viscera. We describe the diagnostic modality, treatment planning
and the timing of treatment of this neoplasm, reviewing also the literature.
Keywords: benign tumour of kidney, neurofibroma, rare tumour, retroperitoneal tumour

INTRODUCTION
Neurofibromas, in general, are rare neoplasm and arise in patients with von Recklinghausen disease, but a solitary variant has been observed in rare cases and its splanchnic location is very uncommon Paraaortic–Pararenal Neurofibroma is an exceedingly rare tumour location. To our knowledge, only six such cases have been reported worldwide to date . Although their diagnosis and location is similar to that of Neurofibroma, different diagnostic and therapeutic approaches (surgery) have been used. In all cases, except ours and another one, patients underwent radical nephrectomy. In all likelihood, such an approach is due to the fact that preoperative imaging staging does not often allow one to diagnose these neoplasias.As a matter of fact, only histology can diagnose them and, if it is performed preoperatively, it can influence treatment. In particular, it is capital to discriminate between malignant and benign lesions thus modifying a surgical approach, conservative versus aggressive ones. In our case, the absence of mitotic activity, lack of necrosis, pleomorphism and infiltrative pattern of
growth allow us exclude malignity.

CASE REPORT
A 62 yr old female with para 3 living 3 tubal ligation done 33 yrs came with c/o abdominal discomfort nausea, anorexia since 1-2 months. Patient is postmenopausal since 15 years and had history of 1 lower segment caeserian section and 2 vaginal birth after caeserian section and last delivery was 33 years back.
Patient is a known case of hypertension and diabetis mellitus and taking medicines since last 6-7 yrs.her vitals were stable.her systemic examination revealed nothing abnormal. On per Abdominal examination there was a huge mass of approximately of 16cm*9cm*8cm felt with diffuse margins and hard in consistency which was extending from the right sided of iliac region upwards.mobility restricted. Scar of LSCS was healthy. there was no guarding , tenderness or rigidity , on per speculum examination the cervix was flushed with vagina. On per vaginal examination the same size mass was felt on per vaginal examination and was very high up with restricted mobility and hard in consistency.patient’s tumour markers were within normal range like AFP - 2.38 iu(0.5- 5.5),beta HCG 4.53 (0-5.3),ca 125-12.7 u (0-35). Ultrasound examination suggestive of right adnexal mass of size 6.5 cm x 10.2 cm which appears to be subserosal fundal fibroid or right ovarian mass . magnetic resonance imaging studies showed it as\ broad ligament fibroid of size 12.5cm x 6.7 cm separate from right ovary well preserved surrounding fat planes.it also showed few small to prominent heterogeneously enhancing lymph nodes at the level of aortic bifurcation which needed further. evaluation.patient undergone

                                                                           (Figure 1)

                                                                           (Figure 2)
Laparotomy in situ patient had dense multiple adhesions between anterior abdominal wall and parietal peritoneum.whole things were stucked up.bowels was adherent which was separated with fine dissection.the bladder was very much stucked and adherent to the mass arising from retroperitoneum.uterus and adnexae were not seen due to the mass and adhesions.

                                                                          (Figure 3)
Adhesions were separated by blunt and sharp dissection and adnexal mass isolated .Grossly the mass was a size of 17cm*10cm*8cm roughly.hard in consistency,whitish in colour with smooth and shiny surface. After dissecting and separating the tumour mass sent to frozen section.

                                                                           (Figure 4)
The frozen section report was suggestive of spindle cell tumour most likely neurofibroma. after the removal of mass then the atrophic uterus and ovaries seen clearly.Total abdominal hysterectomy with bilateral salpingoophorectmy done.postoperatively patient was managed on antibiotics and analgesics with injection insulin for diabetic management .Patient recoverd well with suture removal done on day 10 .Final histopathology report was suggestive of neurofibroma .

                                                                           (Figure 5)

                                                                            (Figure 6)
DISCUSSION
First described by Garre et al [2]. in 1892, glandular differentiation is the rarest form of divergent differentiation seen in peripheral nerve sheath tumors (PNST) which also includes cartilage, bone, chondrosarcoma, osteosarcoma and rhabdomyosarcoma. Around 40 cases have been reported so far in world literature.
Diagnostic criteria includes evidence supporting a finding of nerve sheath tumor, presence of true glandular epithelium, not entrapped glands or pseudoepithelium. The vast majority of PNST harbouring these glands have been malignant peripheral nerve sheath tumor (MPNST) [3]. The glandular epithelium is usually benign though there are sporadic case reports of PNST with malignant glandular component [4].
Glandular neurofibromas are rare and in an extensive review by Woodruff and Christenstein, only 2 out of 25 analyzable cases of glandular peripheral nerve sheath tumors were neurofibromas. Both of these cases occurred in young females, were associated with NF Type 1 and were finally.
diagnosed as plexiform neurofibromas [3].No history of neurofibromatosis was present in the other reported cases of glandular neurofibromas .
Most important differential diagnostic consideration in a case of glandular neurofibroma is schwannoma with entrapped adnexal structures. Schwannomas usually display typical Antoni A and B areas along with presence of Verocay bodies. Trapped adnexal glands are generally seen
in clusters and are connected to each other.
In the absence of typical morphological features, differentiation between these entities may be difficult and immunohistochemistry (IHC) may be employed. Glands in true glandular PNST are devoid of myoepithelial cell lining and are hence non reactive for muscle common actin (HHF-35). This glandular epithelium is not only reactive for cytokeratins but also for neuroendocrine cell markers (chromogranin, serotonin and somatostatin). Entrapped adnexal glands in schwannoma show the presence of myoepithelial cell layer and are non reactive for neural markers. Stromal cells show S-100 positivity.
Though IHC was not employed in our case (case number 1), the tumor was diagnosed as neurofibroma as characteristic features of neurofibroma (spindle cells with short, curved nuclei embedded in a collagenous stroma) were seen on histology sections. Moreover, history of NF-type 1 in the patient corroborated the diagnosis of neurofibroma. The glands seen in case number 1 resembled mucus secreting glands rather than eccrine glands of skin adnexa. It is interesting to note that adnexal glands have also been reported in neurofibroma and it has been proposed that the tumor appeared in the nerves around the eccrine glands and grown to the subcutaneous tissue, and the glands might have been left behind rather than entrapped by the growing tumor.
The histogenesis of glandular PNST is still not clear and it may be attributed to the metaplastic potential of Schwann cells or the presence of primitive neural crest cells that migrate with Schwann cells along the peripheral nerves [4].
Neurofibroma with rosette like structures is exceedingly rare. Enzinger reviewed a unique case of neurofibroma showing presence of mucus secreting glands and focal rosettes . A recently described variant of neurofibroma with rosettes is dendritic cell neurofibroma with
pseudorosettes (DCNP) . This tumor is seen in adults and has been described mostly in patients without a history of NF-1,. though cases arising in association with NF-1 have also been described.The lesion is well circumscribed and occurs in superficial dermis of head, trunk and extremities. It is comprised of two types of cells. Type 1 cells are small lymphocyte like cells with slightly cleaved nuclei which are concentrically arranged around larger type 2 cells having vesicular nuclei and copious cytoplasm. These type 2 cells have dendritic extensions which form the core of these pseudorosettes. On IHC, type 2 cells and most type 1 cells stain for CD57 and S-100 .
Another important differential diagnostic consideration is Schwannoma with neuroblastoma like rosettes. In this tumor, small round to oval cells are layered around a central eosinophilic fibrillary material. These cells may also show presence of intranuclear cytoplasmic inclusions.different methods of immobilizing NGF in poly (DL‐lactic acid‐co‐glycolic acid) conduit for peripheral nerve regeneration by

CONCLUSION
Even though neurofibroma are rare in postmenopausal age group it should be considered as one of the differential diagnosis in case of solid ovarian mass. Preoperative suspicion of retroperitoneal neurofibroma help in preventing intraoparative complications. Our case report also emphasizes that preoperative thorough investigations like CT scan, MRI are necessary in case of mass of uncertain origin.

REFERENCES
1.Corbellini, C., Vingiani, A., Maffini, F., Chiappa, A., Bertani, E., & Andreoni, B. (2012). 1Retroperitoneal pararenal isolated neurofibroma: report of a case and review of literature. ecancermedicalscience, 6.
2.Freund, M. E., Crocker, D. W., & Harrison, J. H. (1967). Neurofibroma arising in a solitary kidney. The Journal of urology, 98(3), 318-321.
3. Borrego, J., Cuesta, C., Allona, A., Navio, S., & Escudero, A. (1995). Myxoid neurofibroma of the renal sinus. Actas urologicas españolas, 19(5), 415.
4.Hsieh, S. C., Tang, C. M., Huang, W. T., Hsieh, L. L., Lu, C. M., Chang, C. J., & Hsu, S. H. (2011). Comparison between two.EDC/NHS/MES and genipin. Journal of Biomedical Materials Research Part
A, 99(4), 576-585.


For Article Link:

https://www.researchgate.net/publication/296396416

A huge benign mucinous cystadenoma of ovary: a case report and review of literature

Rajshree D. Katke 
Department of Obstetrics & Gynaecology, Cama & Albless Hospital, Govt. Grant Medical College Mumbai, Maharashtra, India

Correspondence:
Dr. Rajshree D. Katke,
E-mail: drrajshrikatke@gmail.com

Received: 15 March 2014  Revised: 19 March 2014  Accepted: 23 March 2014

DOI: 10.5455/2320-1770.ijrcog20140636

ABSTRACT
Ovarian tumour is not a single entity, but a complex wide spectrum of neoplasms involving a variety of histological tissues. The most common are the epithelial tumours forming 80 % of all tumours. 80% are benign tumours, 10% borderline malignant and 8-10% malignant. Mucinous tumours represent about 8-10% of the epithelial tumours, they may reach enormous size filling the entire abdominal cavity.1 Here we would like to present a case of huge benign mucinous cystadenoma in a 50 year old female where the patient could not access medical care, and presented with huge tumour which lead to breathlessness and responded remarkably to surgical excision. The patient could go back to her normal life following the procedure.
Keywords: Huge benign mucinous cystadenoma, Mucinous cystadenoma

INTRODUCTION
Benign ovarian mucinous tumors are rare at the extremities of age, before puberty and after menopause. They are common between the third and the fifth decades. Mucinous cystadenomas may reach huge size as a matter of fact; many of the largest human tumours belong to this group. Grossly these tumours appear as rounded, ovoid or irregularly lobulated growths with a smooth outer surface of whitish or bluish white hue. The wall in many areas is so thin as to be translucent. Although adhesions to surrounding organs may be
present, they usually represent inflammatory adhesions and do not connote malignant extension. They are attached to the infundibulopelvic ligament by a relatively narrow pedicle that contains the markedly increased supply for the tumour. The content of the cyst is generally a clear viscid fluid, sometimes very thick at other times thin; a mixture of blood elements may give it a chocolate or brownish hue. This fluid is usually rather thin and flows freely at body temperature, but congeals and becomes gelatinous as it cools. The cut surface shows the cavity to be divided by septa into varying number of compartments or locules, these tumours have therefore often been spoken as a multilocular cyst. Microscopically, the distinctive feature of mucinous cysts is the characteristic single layer often undulating outline, of tall, pale-staining secretory epithelium, with nuclei placed at the basal poles of the cells, goblet cells are often seen, on occasion, even paneth and argentaffin cells are noted. Pseudomyxomaperitonii is often associated with mucinous cyst of ovary and mucocele of appendix and carcinoma of large bowel.
 
CASE REPORT
A 40 year female married since 30 years P1L1,
postmenopausal since 15 years presented on 30/11/13
with complaint of abdominal distension and pain since 6
months. Patient consulted at municipal hospital thane, but
symptoms were not relieved. On 25/09/13, CA 125 was
7.2 U/ml, CEA was 79 U (raised) and AFP - 3.3 U/ml.
On examination, her pulse rate was 82 beats/min, BP -
124/80 mmHg. She was mildly pale. 
No lymphadenopathy on general examination. Breast
examination was normal findings. On per abdomen
examination - abdomen was markedly overdistended all
over. The skin was overstretched with prominent veins on
it. On palpation there is a very large palpable mass
arising from the pelvis extending to the epigastrium and
extended diffusely in both the flanks and all over the
abdomen. Consistency was cystic and at some places
firm. With large palpable lump with diffuse ill-defined
margins arising from pelvic region to epigastric region
with lower margins not palpable. Fluid Thrill was
positive. On 03/12/13, all haematological investigations
were normal; Pap smear done on 5/12/13 was suggestive
of atrophic smear with no evidence of malignancy. On
06/1 CT SCAN done on 09/12/13 was suggestive of

predominantly cystic large lesion 18.4 cm x 30 cm x 32
cm in abdominopelvic region with minimal thickened
septae, probably serous / mucinous cystadenocarcinoma.

                                           Figure 1: Shows huge mucinous cystadenoma of the
                                                          ovary extending all over the abdomen.

                                           Figure 2: The rectus sheath, muscle and peritoneum
                                       adherent to the cyst wall and fused to become one layer.

                                           Figure 3: Shows huge mucinous tumour of ovary 
                                                         (20kg in total).

                                           Figure 4: Left ovarian tumour weighing 3 kg.

                                           Figure 5: Huge ovarian tumour of 20 kg.


ON 13/12 /13, exploratory laparotomy with tumour mass
excision with frozen section with total abdominal
hysterectomy with bilateral salpingo-oophorectomy done
under general and epidural anesthesia. Abdomen was
opened by taking midline incision. After opening the
rectus sheath the rectus muscle adherent to the posterior
rectus sheath with cautery the adhesions separated. After
that the thin out peritoneum completely fused with the
wall of the cyst all over and at some places the posterior
rectus sheath, peritoneum and cyst wall fused completely
and formed like one layer of the cyst wall. At some
places the peritoneum wall was very much thinned out
because of overstretching. There was no way to go to the
tumour so decompression of the tumor was done by
taking out the mucinous jelly like material of 10 liters
drained. After that we were able to go the peritoneal
cavity. There was a huge tumour extended to all over the
abdomen 60 cm x 40 cm x 35 cm. It is soft, cystic,
yellowish tumour spreading all over. Posteriorly it was
adherent to the colon the adhesions were separated with
fine dissection and cautery. There was another extension
of the tumour which is cystic mucinous tumour of size 25
cm x 30 cm x 28 cm from the left ovary adherent to the
left ureter and below to urinary bladder. Careful
dissection helped in separating tumour from the left
ureter and urinary bladder. The huge tumour separated all
the sides then delivered out and confirming the position
of the both the ureters keeping them away the pedicle
sealed and cut with cautery. Taken out the tumour
successfully. The total weight of the tumour measured as
20 kg. (Twenty kg) Haemostasis achieved by ligating the
vessels and ligating the pedicles. Total abdominal
hysterectomy with right sided salpingo-oophorectomy
with removal of huge ovarian tumour done. The
omentum was yellow jelly like. Surgeon explored the
bowel to see any other site of involvement of intestine.
Frozen section report came as Benign Mucinous
cystadenoma of the ovary, so pelvic lymph node
dissection was not done. Thorough wash was given.
Haemostasis achieved completely, after checking the
counts of instruments and sponges. Abdomen closed in
layers after keeping the drain in situ. Blood loss during
surgery was average. Her postoperative period was
uneventful. Stiches were taken out. Bilateral ureteric
stents removed and patient discharged. Patient came for
follow up; she was fine and resumed the work.

DISCUSSION Mucinous tumours of the ovary are usually evaluated
using ultrasound, computerized tomography scan, or
magnetic resonance imaging .These ovarian tumors may
be multi-septated, cystic masses with thin walls. They
may contain varying amounts of solid tissue which
consists of proliferating stromal tissue, papillae, or
malignant tumor cells. Tumour markers may also aid us
in telling us the origin of the tumour.3 Benign mucinous
cystadenomas comprise 80% of mucinous ovarian tumors
and 20% - 25% of benign ovarian tumours overall. The
peak incidence occurs be-tween 30 - 50 years of age.
Benign tumors are bilateral in 5% - 10% of cases.
Borderline mucinous cystadenomas make up about 10%
of mucinous ovarian neoplasm’s and are bilateral in 10%
of cases.
 

                                          Figure 6: Computerized tomography imaging pictures 
                                                         of the ovarian tumour.

In the modern era of medicine, such huge mucinous
ovarian tumours have become rare in the current medical
practice, as most of the cases are diagnosed early during
routine gynaecological examinations or incidental finding
on the ultrasound examination of the pelvis and abdomen.
Most of the patients who have large tumours they present
mainly with the pressure symptoms over the
genitourinary system leading to urinary complaints and
also pressure over respiratory system leads to respiratory
embarrassment. The role of imaging modalities like CT
scan and MRI gives better idea about the extension of the
tumour in the various quadrants of the abdomen and
consistency of the tumour. Management of ovarian cysts
depends on the patient’s age, the size of the cyst and its
histo-pathological nature. Conservative surgery as
ovarian cystectomy and salpingo-oophorectomy is
adequate for benign lesions.4 Frozen section is very
important to know the malignant variation of this tumour
and that helps in the management of the patient. As in the
huge tumours, the anatomical planes get distorted, so the
surgical expertise is required to prevent the
complications.

Funding: No funding sources
Conflict of interest: None declared
Ethical approval: Not required

REFERENCES
1. Wilfred Shaw, John Howkins, Gordon Lionel
Bourne. Disorders of the ovary and benign tumours.
In: Wilfred Shaw, John Howkins, Gordon Lionel
Bourne, eds. Shaw’s Textbook, 14th ed. London:
Churchill Livingstone; 2008: 334.
Howard W. Epithelial ovarian cancer. In: Jones,
Anne Colston Wentz, Lonnie S. Burnett, eds.
Novak’s Textbook of Gynaecology. 11th ed. UK:
Williams & Wilkins; 1988: 806-809.
3. Crum CP, Lester SC, Cotran RS. Pathology of
female genital system and breast. In: by Kumar V,
Abbas A, Fausto N, Mitchell R, eds. Robbins’ Basic
Pathology. 8th ed. USA: Elsevier Company; 2007:
Chapt. 19.
4. Alobaid AS. Mucinous cystadenoma of the ovary in
a 12-year-old girl. Saudi Med J. 2008;29(1):126-8.


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