A Case Series on Malignant Peripheral Nerve Sheath Tumour of Cervix: an Institutional Report
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CASE REPORT
A Case Series on Malignant Peripheral Nerve Sheath Tumour of Cervix: an Institutional Report Aswathy G. Nath 1
&
P. Rema 1 & S. Suchetha 2 & Neelima Radhakrishnan 3
Received: 15 May 2020 / Accepted: 1 October 2020 # Indian Association of Surgical Oncology 2020
Introduction Cervical sarcomas are rare malignancies of the cervix with incidence of less than 1 percentage. Malignant peripheral nerve sheath tumour (MPNST) is a rare pathological variant of sarcoma. Of these, MPNST of the cervix is very rare, and we could find only 15 cases in literature. In this report, we present our experience with three cases of MPNST of cervix.
Case Reports Case 1 A 44-year-old parous premenopausal lady presented with complaints of abnormal uterine bleeding of 1-month duration with history of weight loss and loss of appetite. On clinical examination, there was a proliferative growth arising from anterior lip of the cervix and filling vagina. MRI showed enlarged uterus with multiple fibroids and 9 × 9 × 6 cm hyperintense mass arising from the anterior lip of the cervix with bilateral parametrial involvement and no enlarged nodes. Punch biopsy was taken which showed only necrotic tissue, but patient presented to the emergency department with profuse vaginal bleeding. Patient was started on concurrent chemoradiation with a clinical diagnosis of carcinoma cervix IIB
* Aswathy G. Nath [email protected] 1
Division of Gynaecological Oncology, RCC, Thiruvananthapuram, Kerala, India
2
Division of Gynaecological Oncology, RCC, Thiruvananthapuram, Kerala, India
3
Division of Pathology, RCC, Thiruvananthapuram, Kerala, India
with profuse bleeding. Patient received EBRT 40GY in 20 fractions with weekly cisplatin 40 mg/m2. Post-chemoradiation, she had persistent tumour clinically which was confirmed in MRI. The case was discussed in a multidisciplinary tumour board and decided for radical hysterectomy, and patient underwent type B radical hysterectomy with bilateral salpingo-oophorectomy. Histopathology report came as a spindle cell sarcoma suggestive of malignant peripheral nerve sheath tumour with MIB signalling index 18–20%. Patient was advised adjuvant chemotherapy, but she was not willing so kept under follow-up. Patient presented to OPD after 11 months with complaints of breathlessness and on evaluation detected to have massive right-sided pleural effusion and multiple pulmonary metastasis. Intercostal drainage for pleural effusion was done followed by pleurodesis with 60 units bleomycin. In view of her poor performance status, she was started on single agent Adriamycin. Patient developed progressive disease and died after 2 months (Figs. 1, 2, 3, 4, 5, 6, 7, 8, and 9).
Case 2 A 50-year-old parous postmenopausal lady presented with complaints of postmenopausal bleeding. On clinical examination, a proliferative lesion 4 × 4 cm was seen arising from both lips of the cervix without parametrial infiltration. Punch biopsy from lesion was suggestive of mesenchymal tumour with epithelial elements with S-100 positive, HM
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