International Journal of Health Sciences and Research www.ijhsr.org ISSN: 2249-9571

Case Report

Endometrial Stromal Sarcoma Mimicking Adenomyosis with Ovarian Carcinoma - A Case Report

Dr. Gritachi Das1, Dr. Sanjay M. Khaladkar2

1Post Graduate Student, 2Professor, Dept. of Radiology, Dr. D.Y. Patil Medical College and Research Centre, Dr D.Y. Patil Vidyapeeth Pune, Maharashtra.

Corresponding Author: Dr. Gritachi Das

ABSTRACT

Endometrial stromal sarcoma is a very rare malignant tumour that constitutes 0.2 % of all uterine malignancies. It affects slightly younger woman as compared to otherwise uterine malignancies with mean age of 42-52 years. Usual presentation is in perimenopausal age with abnormal uterine bleeding, uterine enlargement. It mimics endometrial carcinoma, leiomyoma, leiomyosarcomas, adenomyosis and ovarian tumour. A high index of suspicion is needed for its diagnosis. Hysterectomy is the treatment of choice. We report a case of 40 year old female patient presenting with lower abdominal pain, menorrhagia and abdominal distension. Ultrasound and CT abdomen and pelvis revealed enlarged uterus with heterogeneous myometrium, subserous fibroid and multilocular cystic mass with solid mural nodules. A diagnosis of adenomyosis with malignant ovarian neoplastic mass was made. Hysterectomy was performed. Histopathological diagnosis was entrometrial stromal sarcoma with bilateral ovarian infiltration.

Key words - Endometrial Stromal Sarcoma, Adenomyosis, ovarian carcinoma, leiomyosarcoma.

CASE REPORT were not transmitted to mass. Bilateral A 40 year old female patient adnexa could not assessed due to extent of presented with pain in lower abdomen since the mass. Rectal mucosa was free on per 5 months, increased PV bleeding during rectal examination. Mass corresponded to menses and abdominal distension since 3 size of 36 weeks gravid uterus. A months. There was no history of fever, differential diagnosis of ovarian neoplastic weight loss, bowel bladder disturbance. On mass, uterine fibroid with cystic per abdominal examination, there was degeneration or retroperitoneal mass was uniform distension of abdomen made. Laboratory investigations were corresponding to size of 36 weeks gravid within normal limits. RFT’s, LFT’s were uterus. The mass was both firm and cystic in normal. CA 125 -52.7 U/ml. consistency, side to side mobility of mass Ultrasound (USG) abdomen pelvis was restricted and lower pole of the mass showed an enlarged and bulky uterus with could not be reached. On PV and per marked heterogeneous echotexture of speculum examination, vagina was healthy myometrium with a subserous fibroid. and cervix was pulled up. Mobility of the Endometrium was not distinctly visualised. mass was restricted. Cervical movements A large multilocular cystic mass was noted

International Journal of Health Sciences & Research (www.ijhsr.org) 320 Vol.7; Issue: 9; September 2017 Gritachi Das et al. Endometrial Stromal Sarcoma Mimicking Adenomyosis with Ovarian Carcinoma-A Case Report superior to uterine fundus with multiple showing minimal vascularity. Both ovaries septations and solid components, measuring were not visualised separately. Both kidneys approximately 30 (Transverse) X25 showed mild fullness of pelvi-calyceal (Cranio-caudal C) x18 (Antero-posterior) system due to compression of both ureters cm with multiple internal septations and by the mass. Diagnosis of adenomyosis of solid components. It was seen occupying uterus with CA ovary was made. almost entire abdomen and pelvis in midline

A B C D

Figure 1 (A-D)- Axial CECT abdomen showing multilocular cystic mass in pelvis superior to uterine fundus with solid mural nodules (A,B); enlarged and bulky uterus with heterogeneous myometrium with subserosal fibroid on right side(C,D).

A B C D

Figure 2 (A-D)- Sagittal CECT abdomen showing multilocular cystic mass in pelvis superior to uterine fundus with solid mural nodules , enlarged and bulky uterus with heterogeneous myometrium with obscuration of intervening fat planes.

A B C D

Figure 3(A-D)- Coronal CECT abdomen showing multilocular cystic mass in pelvis superior to uterine fundus with solid mural nodules , enlarged and bulky uterus with heterogeneous myometrium with obscuration of intervening fat planes and mild ascites in bilateral paracolic gutters.

International Journal of Health Sciences & Research (www.ijhsr.org) 321 Vol.7; Issue: 9; September 2017 Gritachi Das et al. Endometrial Stromal Sarcoma Mimicking Adenomyosis with Ovarian Carcinoma-A Case Report

CT abdomen and pelvis (Figures- multiple tumour emboli (Figure-5D). A 1,2,3) showed enlarged and bulky uterus diagnosis of endometrial stromal sarcoma of with mild heterogeneous myometrium with uterus with multiple tumour emboli, fundic subserosal fibroid. A large bilateral ovarian infiltration, with no multilocular cystic mass measuring perineural invasion was made. Parametrium, approximately 30 (T)X25 (CC)X18(AP) cm omentum and surrounding tissues were free was noted in pelvis just superior to uterine from tumour cells Figo staging III A, TNM fundus with multiple thin and thick staging 3A NO MO / stage IIIA. septations and solid mural nodules showing heterogeneous enhancement on contrast study. No calcification was seen. Fat plane between the mass and uterine fundus was obscured. Mild ascites was noted in bilateral lower paracolic gutters. Endometrial cavity was not distinctly visualised. There was no abdominal or pelvic lymphadenopathy. No hepatic metastasis. A diagnosis of adenomyosis with fundic subserous fibroid and malignant neoplastic ovarian mass was made. Intraoperative a large cystic mass was noted arising from uterine fundus which was densely adherent to omentum and bowel. Figure 5-Histopathological examination- Total abdominal hysterectomy with bilateral A) High power view-show small oval to spindle cells resembling endometrial stroma, tongue like infiltration salpingo-oophorectomy with omentectomy between muscle bundles of myometrium, multiple tumours with lymphnodes resection was done emboli. (Figure 4).

B) Ovary-Scanner view- Sections from both ovaries showed Figure 4- Postoperative specimen showing uterus along with infiltration by tumour cells and multiple tumour emboli cystic mass superior to uterine fundus.

INTRODUCTION Histopathological examination Uterine neoplasm can be (Figure-5 A,B) showed small oval to spindle endometrial or mesenchymal type. cell resembling endometrial stroma, tongue Endometrial carcinoma is the most common like infiltration between muscle bundles of endometroid tumour of the uterus. myometrium, multiple tumours emboli, Mesenchymal tumours are subclassified as infiltration of serosal surface of the uterus smooth muscle, endometrial stromal and by tumour cells. Mitotic figures of 4 to 5/ 10 mixed epithelial mesenchymal tumours. [1] hpf were seen. Sections from both ovaries Uterine sarcomas represent 2 to 5% showed infiltration by tumour cells and of all uterine malignancies. They are of 3 International Journal of Health Sciences & Research (www.ijhsr.org) 322 Vol.7; Issue: 9; September 2017 Gritachi Das et al. Endometrial Stromal Sarcoma Mimicking Adenomyosis with Ovarian Carcinoma-A Case Report types- carcinosarcoma (mixed mullerian are complicated. Latest WHO classification tumours) which is the most common (40- is not made on mitotic comment but on the 70%) followed by leiomyosarcoma (40- basis of unclear pleomorphism and necrosis. 50%) and endometrial stromal sarcoma UES (Undifferentiated endometrial which is the least common (<10%) of sarcoma) represent a high grade sarcoma uterine sarcomas. [2,3] which bears no histological resemblance to Endometrial stromal sarcoma is a endometrial stroma. Abnormal uterine very rare malignant tumour that constitutes bleeding is present in 90% of woman while 0.2 % of all uterine malignancies. Its annual 70 % cases show uterine enlargement. incidence is 1 to 2 per million woman. It About 30 -50% of ESS has intrauterine affects slightly younger woman as spread at the time of diagnosis. The main compared to otherwise uterine malignancies tumour mass is intramyometrial, most ESS with mean age of 42-52 years. 10 to 25 % of involve the endometrium and hence uterine affected woman are premenopausal. It is an curettage is useful in preoperative diagnosis. indolent tumour with local recurence and Sometimes the lesion is completely within distant metastasis even after 20 years of the myometrium when uterine curettage is initial diagnosis. Mean age at presentation is not diagnostic. Since ESS has great 42 to 53 years. Patient usually present with similarity with normal endometrium, it may pain and abnormal vaginal bleeding. They not be possible to diagnose with certainty are typically soft and polypoid and may fill and curettage fragments. Hence definitive the endometrial cavity. Necrosis and diagnosis can be made only on haemorrhage are common. Histologic hysterectomy specimen. Rarely it can grades are – endometrial stromal nodal present at extrauterine site -commonly (ESN, benign), low grade, high grade and ovary. It can be primary or metastatic lesion undifferentiated. The staging of uterine from an occult tumour of the endometrium sarcoma is like endometrial carcinoma. or from a previous undiagnosed case when Stage I limited to uterine body, Stage II- hysterectomy was done for uterine spread to the cervix, Stage III- spread leiomyoma. [4] It can extend into adjacent outside the uterus but within the pelvis, structures like fallopian tubes, ligaments, Stage IV- distant metastasis. [4] ovaries and vessels. Differential diagnosis is uterine DISCUSSION leiomyoma, endometrial polyp, ESN is a benign tumour composed adenomyosis, mullerian adenocarcinoama. of cells which resemble normal proliferative Extrauterine ESS mimic sex cord stromal phased endometrial stromal cells. ESN is tumours of ovary, gastrointestinal stromal considered as a long as finger like tumours, hemangiopericytoma, protrusion into the adjacent myometrium are lymphangiomyomatosis and mesenchymal <3mm in depth and<3 in the number with cystic hamartoma of the lungs. [4] no vascular invasion. More extensive On microscopy, ESS arises from myometrial invasion and endolymphatic and endometrial stroma (connective tissue) invasion vascular is suggested of LGESS. rather than the glands. On macroscopy, it Low grade ESS is distinguished from high shows polypoid mass extending into broad grade ESS by a mitotic rate of <10 mitotic ligament, ovaries and fallopian tubes. figures (MF) /10 ppf and clinically by a Lymphatic tumour plaques are seen as more protracted course. Recurrence occurs yellow, ropy and ball like masses. On late with local recurrence more common microscopy, it shows monotonous ovoid than distant metastasis. [6,7] cells to spindly cells with minimal The pathologenesis of ESS is cystoplasm, prominent arterioles with unknown. Exposure to unopposed estrogen, angiolymphatic invasion, upto 10-15 mitotic tamoxifen and polycystic disease of ovaries figures per 10 hpf in active areas, tongue

International Journal of Health Sciences & Research (www.ijhsr.org) 323 Vol.7; Issue: 9; September 2017 Gritachi Das et al. Endometrial Stromal Sarcoma Mimicking Adenomyosis with Ovarian Carcinoma-A Case Report like infiltration in myometrium between well defined or ill- defined. They can be muscle bundles, may have foam cells or hyperechoic with respect to myometrium or hyalinization in the stroma and may exhibit heterogeneous with multiple anechoic cystic epitheloid, myxoid and fibrous change. [8] areas. Low grade ESS has four patterns on Immunoreactivity with antibodies to USG – predominantly solid mass with cystic CD-10 and smooth muscle actin and desmin areas, predominantly unilocular cystic mass, are used to differentiate between ESS and a well defined solid mass and an ill defined leiomyoma. Usual treatment is total infiltrative solid mass mimicking abdominal hysterectomy with bilateral adenomyosis. A more nodular or coarse salpingo-oophorectomy and pelvic and peri- appearance of the myometrium is suggestive aortic selective lymphadenectomy. In low of low grade ESS rather than adenomyosis. grade ESS and recurrent disease hormonal [5] therapy tamoxifen, gonadotrophin releasing hormone (GnRH) analogoues CONCLUSION medroxyprogestron and aromatase As it a rare entity and mimic inhibitors is suggested. As gynaecologist endometrial carcinoma, leiomyoma, and radiologist are not much familiar with leiomyosarcomas, adenomyosis and ovarian this tumour, it may be mistaken for tumour (with intrauterine) involvement, a endometrial polyp, leiomyoma, high index of suspicion is needed. Usual adenomyosis, subserous fibroid, presentation is in perimenopausal age with leiomyosarcoma of uterus. High index of abnormal uterine bleeding, uterine suspicion is needed in a rapidly enlarging enlargement. Hysterectomy is the treatment uterus with heterogeneous myometrium and of choice diagnosis is done on histological extaruterine mass. [4] examination. Grades of ESS can be detected On MRI, ESS has typically high by studying characteristics varying of the signal intensity on T1WI and tumours. Prognosis is better in low grade heterogeneously highly signal intensity on ESS. T2WI. The tumour margin are typically irregular with marginal nodular lesion. REFERENCES Intramyometrial worm like nodular 1. Szklaruk J, Tamm EP, Choi H, extension are often seen with areas of Varavithya V.MR imaging of common hemorrhage and necrosis. Most of them and uncommon large pelvic masses. Radiographics. 2003 Mar- show marked enhancement then normal Apr;23(2):403-24. PMID: 12640156. myometrium. They have to be differentiated 2. Gandolfo N, Gandolfo NG, Serafini G, from endometrial carcinoma, leiomyomas MartinoliC. Endometrial stromal with cystic degeneration, leiomyomas. sarcoma of the uterus: MR and US Endometrial carcinomas do not show findings. Eur Radiol. 2000;10(5):776-9. irregular marginal lesions, intramyometrial PMID: 10823632 nodules or marginal nodules and do not 3. Sahdev A, Sohaib SA, Jacobs I, show extensive enhancement as ESS. Shepherd JH, Oram DH, Reznek Leiomyomas usually have regular margins RH.MR imaging of uterine sarcomas. and appear hypointense on T2WI. [9] AJR Am J Roentgenol. 2001 Leiomyosarcomas may show irregular Dec;177(6):1307-11.PMID: 11717072 4. Puliyath G, Nair MK Endometrial margins. Adenomyosis affects the stromal sarcoma: A review of the myometrium diffusely. It appears literature. Indian J Med Paediatr Oncol. hypointese with respect to myometrium on 2012 Jan;33(1):1-6 PMID: 22754201 [9] both T1WI and T2WI. PMCID: PMC3385272 On USG, it can be seen as intramural 5. Park GE, Rha SE, Oh SN, Lee A, Lee mass protruding into the endometrial cavity KH, Kim MR. Ultrasonographic or as intramural mass. The margins can be findings of low-grade endometrial

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How to cite this article: Das G, Khaladkar SM. Endometrial Stromal Sarcoma mimicking adenomyosis with ovarian carcinoma- a case report. Int J Health Sci Res. 2017; 7(9):320-325.

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International Journal of Health Sciences & Research (www.ijhsr.org) 325 Vol.7; Issue: 9; September 2017