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Malignant peripheral nerve sheath tumor of the uterine corpus presenting as a huge abdominal neoplasm. ABSTRACT. A 45‑year‑old P3L3, referred to us with ...

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Malignant peripheral nerve sheath tumor of the uterine corpus presenting as a huge abdominal neoplasm ABSTRACT A 45‑year‑old P3L3, referred to us with abdominopelvic mass for further management. Vaginal examination was suggestive of uterine mass. Magnetic resonance imaging (MRI) of abdomen‑pelvis disclosed a uterine mass with equivocal invasion of the fat plane with the sigmoid colon. Coelomic antigen (CA) 125 was 120.2 U/ml (normal range, 0–35 U/ml). On exploratory laparotomy entire pelvic cavity was filled with a mass that was seen arising from the uterus and involving the sigmoid colon. Hence, a total abdominal hysterectomy with bilateral salpingo‑oopphorectomy (TAH BSO) was performed, along with resection anastomosis of the rectosigmoid and excision of omental and pelvic peritoneal nodules. Histopathology and immunohistochemical analysis, including S100‑P positivity confirmed diagnosis of a malignant peripheral nerve sheath tumor (MPNST), with tumor deposits in the right parametrium, omentum, sigmoid colon, and pelvic peritoneum. This case is presented in view of its rarity and associated diagnostic and therapeutic implications. KEY WORDS: Abdominal neoplasm, hysterectomy, leiomyosarcoma, malignant peripheral nerve sheath tumor, S100‑protein, uterine sarcoma

INTRODUCTION MPNSTs are aggressive soft tissue sarcomas that invariably arise from a peripheral nerve or in the extraneural soft tissues and display nerve sheath differentiation.[1,2] These tumors rarely involve the genitourinary system.[3] Primary uterine MPNSTs are extremely rare and till date, only three cases of MPNST of the uterine corpus have been reported, to the best of our knowledge.[4‑6] CASE REPORT A 45‑year‑old, P3L3, Hepatitis B surface antigen positive lady presented with abdominal distension, decreased appetite, and significant weight loss for 2 months. She had regular, but heavy menses. Her abdominal examination revealed 24–26 weeks sized firm mass extending from right to left iliac fossae with restricted mobility. Vaginal examination showed a pulled up but healthy cervix with a uterine mass. Papanicolaou (Pap) smear was normal. There was no evidence of neurofibromatosis type 1 (NF1). She had no previous exposure to radiation. Abdominal and transvaginal ultrasonography (USG) and magnetic resonance imaging (MRI) revealed a large abdominopelvic mass infiltrating the myometrium of fundus and posterior wall and encasing the corpus with loss of fat plane between

the mass and the rectosigmoid [Figure 1]. In addition, a 3.3 cm × 2.5 cm sized nodule was identified, compressing the lumbar inferior vena cava. Chest computed tomography (CT) scan showed few subcentimeter sized nodules in right lung. Two years ago, her pelvic USG showed a 1.4 cm × 1.1 cm sized fundal fibroid that increased in size a year ago, when it measured 7 cm × 4.3 cm × 4.3 cm. Serum tumor marker levels for coelomic antigen (CA) 125 were 120.2 U/ml (normal range, 0–35 U/ml) and for lactate dehydrogenase (LDH) were 270 U/L (normal range, 81–234 U/L). Hepatitis B virus (HBV) deoxyribonucleic acid (DNA) viral load was 93 IU/ml (normal value, less than 3.8 IU/ml). The patient underwent an exploratory laparotomy, during which a large tumor was seen arising from the posterior wall and fundus of the uterus, infiltrating into rectosigmoid [Figures 2 and 3]. Bilateral adnexa were unremarkable. Features were suggestive of a uterine sarcoma. Total abdominal hysterectomy with bilateral salpingo‑oophorectomy (TAH BSO) and rectosigmoid resection anastomosis was performed along with excision of pelvic peritoneal deposits and an omental cystic lesion measuring 2.5 cm × 2.5 cm. She had an uneventful postoperative recovery.

Anju Rani Sengar (Hajari), Arundhati G. Tilve, Jagdeesh N. Kulkarni1, Rekhi Bharat2 Department of Gynecology, Dr. Babasaheb Ambedkar Hospital, Central Railway, Mumbai, 1Department of Genito‑Urinary and Gynecological Oncosurgery, Asian Institute of Oncology, Mumbai, 2Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India For correspondence: Dr. Anju Rani Sengar (Hajari), Dr. Babasaheb Ambedkar Hospital, Central Railway, Byculla, Mumbai ‑ 400 027, Maharashtra, India. E‑mail: [email protected] gmail.com

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Pathological findings Grossly, the resected specimen measured 19 cm × 17 cm × 8 cm. On serial sectioning, a

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Sengar, et al.: Malignant peripheral nerve sheath tumour

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Figure 1: Multiplanar (a‑d) T2W images show a large exophytic mass surrounding the uterus, with focal attachment to the fundus and posterior wall. Note a metastatic node (e) compressing the common iliac vein and an omental nodule (f) T2W = T2‑weighted

Figure 3: Posterior surface view showing large uterine mass (m), both ovaries (o), and cervix (c) Figure 2: On laparotomy, exteriorized uterus (u) and mass (m)

proliferative tumor was seen involving the fundus and body of the uterus, measuring 14 cm × 14 cm × 7.2 cm. Cut surface of the tumor was grey‑white and fleshy with focal areas of hemorrhage, necrosis, and myxoid change. Hematoxylin and eosin (H and E) stained microsections revealed near total effacement of the endomyometrium by a spindly sarcomatous tumor composed of cells arranged in palisades and storiform pattern, along with focal areas of myxoid differentiation and geographic areas of tumor necrosis. Interspersed were numerous mitotic figures, varying from 20 to 30 per 10 high power fields, including atypical forms. Tumor was seen involving more than half the thickness of the myometrium. Immunohistochemically, the tumor cells were positive for S100‑P in prominent areas and negative for desmin, smooth muscle actin (SMA), h‑Caldesmon, pan cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), and HMB‑45. Diagnosis of a MPNST was formed [Figure 4]. Besides uterus, tumor deposits were also noted in the right parametrium, omentum, sigmoid colon, and pelvic peritoneum. Cervix, bilateral fallopian tubes, and ovaries were unremarkable. 2

Patient was given adjuvant chemotherapy (gemcitabine + docetaxel) with lamivudine. She tolerated two cycles well. The response to the chemotherapy was minimal. A repeat MRI performed 2 months later showed a well‑defined soft tissue mass in the precaval region at and below the level of inferior pole of right kidney measuring 6.6 cm × 4.2 cm, compressing the inferior vena cava and upper portion of right ureter causing hydroureter and hydronephrosis. Multiple other soft tissue lesions in the omentum, along the peritoneum, sigmoid colon, and anterior to urinary bladder were identified. In view of progressive disease, further chemotherapy was not offered and patient was kept on palliative care. DISCUSSION Leiomyosarcomas and endometrial stromal sarcomas are the most common sarcomas arising in the uterine corpus in peri‑ and postmenopausal women. Adenosarcoma and rhabdomyosarcoma are other uncommon uterine sarcomas. Herein, we present a rare case of huge MPNST arising from the uterus. The most important known risk factor for MPNST Journal of Cancer Research and Therapeutics - ??? - Volume ?? - Issue ?

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Sengar, et al.: Malignant peripheral nerve sheath tumour

planning. Careful histopathological examination and immunohistochemical analysis helped in the exact diagnosis of MPNST. On histopathology, lack of epithelial element ruled out an adenosarcoma; lack of carcinomatous elements ruled out a malignant mixed Müllerian tumor (MMMT) and lack of immunohistochemical expression of smooth muscle markers ruled out a leiomyosarcoma. Absence of expression of epithelial markers objectively ruled out a MMMT and a synovial sarcoma. S100‑P positivity reinforced diagnosis of a MPNST. HMB‑45 negativity militated against a melanoma. In view of this tumor being extremely uncommon in the uterus, the treatment is not standardized. There is insufficient data on diagnosis, differential

is NF1; up to 29% of patients with NF1 suffer from MPNST in the course of their life; 50-60% of MPNSTs occur in patients with NF1. The second known risk factor for developing MPNST is radiation exposure decades prior to occurrence of MPNST, recorded for 10% of patients.[7] After extensive review of literature, we found only 10 documented cases of MPNST of the uterine cervix[2,4] and three cases of uterine corpus MPNST [Table 1]. Clinical examination clearly indicated lesion of uterine origin. Additionally, a detailed MRI report helped in surgical

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Figure 4: (a) Cellular tumor showing spindly cells in focal palisading pattern (hematoxylin and eosin (H and E), ×200) (b) Sarcomatous cells with interspersed several mitotic figures  (arrows)  (H  and  E, ×400)  (c) Focal myxoid differentiation within the tumor  (H  and  E, ×200). (d) Polygonal sarcomatous cells with an interspersed atypical mitotic figure (arrow head) (H and E, ×400) (e) S100‑P positivity in prominent tumor areas (diaminobenzidine (DAB), ×200). Inset: Distinct, S100‑P positivity in the spindly and polygonal tumor cells (DAB, ×400)

Table 1: Literature review of reported cases of MPNST of uterine corpus Authors Age Site (years) Gustavo 56 Uterine et al.[4] corpus

Molina et al.[5] Gulati et al.[6]

62 22

Present 45 case

Clinical presentation Abnormal uterine bleeding

Uterine corpus Uterine Abnormal corpus uterine bleeding Uterine Large pelvic corpus mass

IHC profile

Treatment

Vimentin +, Neurofilaments TAH BSO CD56.(kindly check) −CD10, h‑caldesmon, desmin, myoglobin, cytokeratins (AE1/AE3), CD117, CD34, HMB‑45, chromogranin, S‑100 protein, and GFAP Vimentin +, S100‑P +, CK−, CD57− TAH BSO S100‑P +, vimentin +, inhibin−, desmin−, synaptophysin−, myogenin−, GFAP−, chromogranin−, HMB‑45−, Melan A−, and CK− +S100‑P, −desmin, smooth muscle actin (SMA), h‑caldesmon, pan cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), and HMB‑45

Outcome Not known

Not known

TAH BSO

Vaginal metastasis after 6 months of treatment TAH BSO with Multiple abdominal rectosigmoid resection metastasis at 2 month anastomosis and excision follow‑up. Alive till of omental nodules date, that is, 6 months

+=Positive, −=Negative, TAH BSO=Total abdominal hysterectomy with bilateral salpingo‑oophorectomy, MPNST=Malignant peripheral nerve sheath tumor, IHC=Immunohistochemical

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diagnosis, treatment and prognosis. Goertz et al.[7] in evaluation of 65 patients with histologically proven MPNST concluded that initial diagnosis has to be revised in 32% of cases, the common sites were proximal upper and lower extremity. Therapy consists mainly of surgery. Irradiation and chemotherapy are only used for select patients. The only significant negative prognostic factor for survival was occurrence of metastasis.[7] Our case presented with intraabdominal metastasis. In published literature there are only three uterine MPNSTs reported till date. Two of them presented as abnormal uterine bleeding, our patient presented with abdominal distension. All three were treated by Total abdominal hysterectomy and bilateral salpingooophorectomy. In two of them outcome is not known and one presented with vaginal metastasis after six months. Further follow up is not known. There is no evidence on whether a more radical surgery or adjuvant chemo/radiotherapy can improve the overall survival or disease free survival. In this patient surgical findings indicated peritoneal, intra-abdominal disease and a 3.3 cm × 2.5 cm sized nodule compressing and encasing the lumbar Inferior vena cava. Decision to give chemotherapy was individualised based on a high chance of early symptomatic recurrence in this patient, with the hope of delaying it. Regarding the origin of the tumor in the present case, probably an earlier small fibroid, identified on USG might have progressed. The prognosis for patients with MPNST remains guarded as it is fraught with local recurrences. Sixteen percent patients have metastatic lesions at the time of diagnosis,[3,8] as also noted in our case. The 5‑year survival rate has been reported to be varying from 47 to 65% (average 24–48 months).[6,8‑10] In conclusion, our case reinforces the value of a dedicated multidisciplinary team for evaluation of suspected uterine sarcomas. MRI is a useful tool for planning treatment of large abdominal and pelvic neoplasms. Careful assessment of histopathological features and immunohistochemical analysis at a tertiary center are helpful in objective confirmation of a MPNST, including at rare locations like the present case. Surgical excision remains the treatment mainstay, adjuvant therapy should be individualized.

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ACKNOWLEDGEMENT We acknowledge the support provided by Dr Ganesan Karthik, Consultant Radiologist, Mumbai, for providing expert radiological opinion and MRI images.

REFERENCES 1. Nielesen GP, Antonescu CR, Lothe RA. Malignant peripheral nerve sheath tumour. In: Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F, editors. World Health Organization (WHO) Classification of Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2013. p. 187‑90. 2. Rekhi B, Ingle A, Kumar R, De Souza MA, Dikshit R, Jambhekar NA. Malignant peripheral nerve sheath tumours: Clinicopathological profile of 63 cases diagnosed at a tertiary cancer referral center in Mumbai, India. Indian J Pathol Microbiol 2010;53:611‑8. 3. O’Brien J, Aherne S, Buckley O, Daly P, Torreggiani WC. Malignant peripheral nerve sheath tumour of the bladder associated with neurofibromatosis I. Can Urol Assoc J 2008;2:637‑8. 4. de A Focchi GR, Cuatrecasas M, Prat J. Malignant peripheral nerve sheath tumor of the uterine corpus: A case report. Int J Gynecol Pathol 2007;26:437‑40. 5. Molina CP, Putegnat BB, Logrono R. Fine needle aspiration cytology and core biopsy of malignant peripheral sheath tumor of uterus: A case report. Diagn Cytopathol 2001;24:347‑51. 6. Gulati N, Rekhi B, Suryavanshi P, Jambhekar NA. Epithelioid malignant peripheral nerve sheath tumor of the uterine corpus. Ann Diagn Pathol 2011;15:441‑5. 7. Goertz O, Langer S, Uthoff D, Ring A, Stricker I, Tannapfel A, et al. Diagnosis, treatment and survival of 65 patients with malignant peripheral nerve sheath tumors. Anticancer Res 2014;34:777‑84. 8. Sordillo PP, Helson L, Hajdu SI, Magill GB, Kosloff C, Golbey RB, et al. Malignant schwannoma‑‑clinical characteristics, survival, and response to therapy. Cancer 1981;47:2503‑9. 9. Doorn PF, Molenaar WM, Buter J, Hoekstra HJ. Malignant peripheral nerve sheath tumors in patients with and without neurofibromatosis. Eur J Surg Oncol 1995;21:78‑82. 10. Wanebo JE, Malik JM, VandenBerg SR, Wanebo HJ, Driesen N, Persing JA. Malignant peripheral nerve sheath tumors: A clinicopathologic study of 28 cases. Cancer 1993;71:1247‑53. Cite this article as: ??? Source of Support: Nil, Conflict of Interest: None declared.

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