Foetiform teratoma : A Rare Variant of Mature Cystic Teratoma Arshad Khan M Pathan*, Sangita R Margam**, Madhu Chaturvedi***, Manisha S Khare****

Abstract Foetiform teratoma is a rare form of mature cystic teratoma. A 23 year old unmarried female presented in emergency with acute abdomen. On ultrasonography bilateral cystic ovarian masses were seen. An exploratory laparotomy with bilateral cystectomies was performed. Based on the gross and microscopic finding, the left ovarian cyst was diagnosed as Foetiform teratoma. This has to be distinguished from Foetus in foetu. Here we present a rare case of Foetiform teratoma and discuss the differentiating features from Foetus in foetu.

Introduction oetiform teratoma is a term that F has been given to a rare variant of mature cystic teratoma that is highly developed and organised; resembling a malformed foetus. This is a rare entity presenting most often as ovarian mass in women of reproductive age group. Case Report A 23 year old unmarried lady presented with pain in abdomen. Her past medical and surgical history was insignificant. On examination there was abdominal tenderness. USG Abdomen revealed bilateral ovarian cysts, right measuring 6 Fig. 1 : External surface showing arrow x 5 x 4 cm and left measuring 7 x 6 x 5 cm head cranial pole and arrow limb buds respectively. CA-125 was 35 IU/ml. Patient underwent bilateral ovarian cystectomy. through the mass showed fatty yellowish areas We received two cystic masses. Right with bone, cartilage and tooth. X-ray of specimen measured 6 x 5 x 4 cm and left sided cyst showed a mass resembling malformed foetus with measured 7 x 6 x 5 cm, externally smooth with vertebral body (Fig. 2). congested blood vessels. On cut opening right Microscopically right sided ovarian cyst ovarian cyst was unilocular thin walled and filled showed features of dermoid cyst. Multiple with sebum and tufts of hairs, whereas left sections through left sided ovarian mass showed ovarian cyst revealed a structure macroscopically tumour composed of disorganised mature tissue resembling an ill formed foetus with globular mass of all the three germ layers viz. skin, adipose like elevation resembling head and two small limb tissue, cartilage, bone, teeth, gastrointestinal buds, each measuring 0.6 cm (Fig. 1). Cut section epithelium, respiratory epithelium, thyroid, choroidal tissue, mature glial tissue. Thus based *Resi. Pathologist, **Asst. Prof., ***Asso. Prof., on clinical history, gross, radiological findings ****Prof., Dept. of pathology, Lokmanya Tilak and microscopic examination, we diagnosed this Municipal College and Lokmanya Tilak Municipal Hospital. as Foetiform teratoma.

544 Bombay Hospital Journal, Vol. 53, Special Issue, 2011 cases have been reported in the English literature.1,2 Age distribution of the patients ranged from 2.5 yrs to 65 yrs.1,2,3 However most presented in the third or fourth decade of life. This entity needs to be distinguished from Foetus in foetu (FIF). Most reported cases of the FIF have been discovered in infancy as an abdominal mass and no cases have been reported within an ovary. The most common location is retro peritoneum. In contrast, Fig. 2 : X-ray showing malformed foetus foetiform teratoma are most commonly with vertebral body. found in women of reproductive age and discovered as ovarian masses. Other common sites being testis and mediastinum, less commonly midline region, sacrococcygeal region and GIT especially caecum is involved.2 It has been proposed that foetiform teratoma and FIF can be distinguished based on zygosity. Most ovarian teratomas are homozygous at loci, where the host n o r m a l t i s s u e d e m o n s t r a t e s heterozygosity, but FIF is genetically identical to its host.1,2,3,4 In our case Fig. 3 : Microscopic examination showed 1) cytogenetic study was not carried out. Thyroid 2) GIT 3) Ovarian tissue 4) Skin 5) Many previous reports have Brain 6) Choroidal tissue. suggested that FIF and foetiform Discussion teratoma can be differentiated based on Mature cystic teratoma are presence of axial skeleton and spine, common benign ovarian tumours that but according to recent reports occur most commonly in women of organogenesis or axial skeleton is used reproductive age group composed of as diagnostic criteria for diagnosing 5 disorganised mature tissues of one or FIF. A spine can also be seen in a 4 more of the embryonic germ layers : foetiform teratoma. In our case though ectoderm, mesoderm and endoderm.1 axial skeleton was seen but organized organogenesis with respect to spine was A foetiform teratoma is a rare not seen and hence the diagnosis of variant of mature cystic teratoma that foetiform teratoma was made. shows some degree of organoid differentiation and appears grossly to Interesting pathological and be shaped like a foetus. Less than 30 radiological findings and rarity of lesion, prompted as to report this case.

Bombay Hospital Journal, Vol. 53, Special Issue, 2011 545 References Fetiform teratoma. Pediatr Radiol 2008; 1. Weiss JR, Burgess JR, Kaplan KJ. Fetiform 38:336-339. teratoma (homunculus). Arch Pathol Lab Med. 3. James AG, Thomas EC. Fetiform teratoma 2006;130:1552-1556. (homunculus). Rev Obstet Gynecol. 2008; 2. Tiffany LR, Krishna S, Peter MS. Intracecal 1(3):95-96.

PRIMARY PREVENTION OF CARDIOVASCULAR DISEASE Severe hypertension and familial hypercholesterolaemia are single risk factors for cardiovascular disease, but blood pressure and cholesterol are measured on a continuous scale and risk of cardiovascular disease is multifactorial. Most events occur in people with modest values of cholesterol (or low density lipoprotein-cholesterol) that overlap with those seen in people without cardiovascular disease and Hingorani and Hemingway debate whether to target high risk people or screen whole populations. Extended screening to everyone aged 40-75 years for cardiovascular disease and metabolic factors to be done. Screening may do no physical harm but some people find it emotionally distressing. Perhaps half of asymptomatic men would be eligible for statins in their last 25 years of life, which raises concerns about “medicalising” such large numbers of people. JOHN P D RECKLESS, BMJ 2011; 342:291-292

546 Bombay Hospital Journal, Vol. 53, Special Issue, 2011 Twin Gestation with One Vesicular Mole and One Normal Foetus Sonal P. Yadav*, Nagendra Sardeshpande**, Pratima Chipalkatti***

Abstract Gestational trophoblastic disease refers to pregnancy related trophoblastic proliferative abnormalities. Hydatidiform mole is the most common type of gestational trophoblastic disease. Very rarely Hydatidiform mole can occur in association with a live twin foetus the incidence being 1 per 22,000 and 1 per 100,000 pregnancies.1 This is a case report of twin gestation with complete mole in one sac along with a normal foetus in the other which progressed to term followed by delivery of a normal neonate.

Introduction Case Report estational trophoblastic disease is a A 30 year old lady gravida 3, para 1, living 1, abortion 1 married since 7 years with 28 weeks of Gspectrum of disorders ranging from pregnancy following intrauterine insemination

Hydatidiform mole to post molar presented at our antenatal clinic with complaint gestational trophoblastic neoplasia and of one episode of spotting per vaginum without i s c h a r a c t e r i s e d b y a b n o r m a l associated abdominal pain. Patient gave history of a similar episode of spotting per vaginum in trophoblastic proliferation with varying first trimester for which she was investigated and risk of metastasis. Hydatidiform mole is diagnosed with subchorionic haemorrhage. divided into complete vesicular mole Patient had responded to conservative with absence of identifiable embryonic management during that episode. tissue and partial mole wherein the Her first pregnancy was a spontaneous abortion at 2 months of pregnancy. The molar tissue coexists with a foetus pregnancy was also conceived following w h i c h o f t e n h a s g e n e t i c a n d intrauterine insemination. Her second pregnancy morphological abnormalities. Twin was again conceived following intrauterine gestation with one molar pregnancy and insemination. The pregnancy continued to term and elective caesarean delivery was done in view other live foetus is an extremely rare of a breech presentation. The current pregnancy condition. Differentiation between was her third pregnancy. partial mole and a multifoetal gestation On examination her general condition was with complete mole with normal foetus fair. She was afebrile with vital parameters is of utmost importance. Studies have normal. Per abdomen examination revealed shown higher risk persistent gestational uterus corresponding to 30 weeks of gestation with the foetus in longitudinal lie and cephalic trophoblastic disease in patients with presentation. The foetal heart sound was twin gestation and complete mole localised on Doppler. The uterus was relaxed. On pregnancy.2 per vaginal examination, the external os was patulous and the internal os was closed. Her haemoglobin was 11.6 gm%, complete *Sr. Resident, **Asso. Consultant, ***Consultant blood count 12200/cmm, platelets adequate, and Head of the unit, Dept. of Obstetric and urine routine normal, fasting blood sugar 89 gm%, Gynaecology, Bombay Hospital Institute of TSH 1.3 U/L and her HIV, HBsAg and VDRL Medical Sciences, Mumbai - 400 020.

Bombay Hospital Journal, Vol. 53, Special Issue, 2011 547 fibrinogen concentration (569 mg%). Other coagulation parameters (PT, PTT, platelet count, D - d i m e r ) w e r e w i t h i n n o r m a l l i m i t s . Haematologist’s opinion was taken and diagnosis of pregnancy-induced hyperfibrinogenaemia was

Fig. 1 : Ultrasound Showing Molar Pregnancy with A Normal Foetus nonreactive. Coagulation parameters (PT, PTT, serum fibrinogen) were within normal limits. S e r u m b e t a h C G w a s 3 1 , 4 0 0 U / L . Ultrasonography revealed twin gestation with one sac filled with multiple cystic structures and a second sac showing a live intrauterine foetus corresponding to 21 weeks of gestation with no obvious congenital anomaly (Fig.1). A diagnosis of twin gestation with one molar pregnancy and other normal live foetus was made. Fig. 3 : Histopathology Picture of Complete After counselling and consultation with the Mole patient and her husband, it was decided to made. continue the pregnancy. Her antenatal checkup was undertaken every week with clinical Patient underwent an elective caesarean monitoring of foetal and maternal well being. delivery at 39.1 weeks of gestation giving birth to Ultrasonography was repeated every two week. a female foetus weighing 2.5 kg. Second sac was Follow up scans showed normally growing live filled with vesicular mole (Fig. 2). The neonate was foetus along with sac filled with vesicular moles. morphologically normal and had a normal There was no repeat episode of antepartum karyotype. haemorrhage. At 32 weeks of gestation, Both placenta and vesicular mole were sent coagulation profile revealed raised serum for histopathology examination. Molar tissue weighed 350 gm and microscopic diagnosis of a complete mole (Fig. 3) was made as there was no evidence of umbilical cord, membranes or foetus. Post partum beta hCG was 38,000 U/L. The beta hCG was repeated at weekly intervals and, seven weeks following delivery, serum beta hCG had reached non-pregnant levels and a chest X-rays (PA and lateral view) were normal. Discussion Gestational trophoblastic disease is a spectrum of interrelated conditions including complete and partial hydatidiform mole, placental site Fig. 2 : Gross Picture of Molar Tissue

548 Bombay Hospital Journal, Vol. 53, Special Issue, 2011 t r o p h o b l a s t i c t u m o u r a n d twin gestation with vesicular mole c h o r i o c a r c i n o m a . C o m p l e t e compared to a singleton complete hydatidiform moles have a 46 XX vesicular mole.4 k a r y o t y p e w i t h b o t h s e t s o f Successful outcome of twin chromosomes derived from the male gestation with vesicular mole and gametes. Partial mole pregnancies are foetus have been reported in studies.5 In triploid and extra set of paternal view of the complications associated chromosomes. Complete moles are with a complete vesicular mole (PIH, usually diagnosed earlier in pregnancy. e m b o l i z a t i o n , a n t e p a r t u m The incidence of complete and partial haemorrhage, torsion of theca lutein mole was found to be 1 per 1,945 and 1 cysts, thyrotoxicosis and risk of per 695 pregnancies respectively. It is persistent and metastatic disease), important to differentiate between a continuation of pregnancy should be partial mole with viable pregnancy from only considered after extensive a twin gestation with one molar counselling of the patient. Careful pregnancy and other normal foetus in monitoring of the pregnancy, maternal view of different foetal prognosis parameters, coagulation profile and (increased risk of foetal abnormalities TSH has to be undertaken. Delivery has in partial mole) and risk of persistent to be conducted in a tertiary centre in gestational trophoblastic disease view of risk of embolisation and (increased in complete vesicular mole) postpartum haemorrhage. Monitoring in each condition. of beta hCG levels till normalisation is 3 Stellar et al have described 8 cases necessary. Chemotherapy is not of twins with molar pregnancy. They warranted except in the presence of found five out of eight patients factors suggesting an increased risk of developed persistent gestational persistence or recurrence of gestational trophoblastic neoplasia. trophoblastic disease. However, a recent study in U.K. References showed no increase in risk of persistent 1. Veherslev LO. Clinical management and gestational trophoblastic neoplasia in a diagnostic possibilities in hydatidiform

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