|Year : 2015 | Volume
| Issue : 3 | Page : 650
Rhabdomyosarcoma of vulva in a young lady: A rare case report with review of literature
Tapesh Bhattacharyya1, Firuza D Patel1, Radhika Srinivasan2, Bhavana Rai1, Pradeep Saha3, R Nijhawan2
1 Department of Radiotherapy and Oncology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Gynaecology and Obstetrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||9-Oct-2015|
Department of Radiotherapy and Oncology, PGIMER, Chandigarh
Source of Support: None, Conflict of Interest: None
A 21-year-old lady presented with a rapidly progressive vulvar swelling with inguinal lymphadenopathy for 7 months. Pathological evaluation revealed it as a case of rhabdomyosarcoma. The disease behaved aggressively and she was treated with multimodality treatment.
Keywords: Rhabdomyosarcoma, vulva, radiotherapy, chemotherapy
|How to cite this article:|
Bhattacharyya T, Patel FD, Srinivasan R, Rai B, Saha P, Nijhawan R. Rhabdomyosarcoma of vulva in a young lady: A rare case report with review of literature. J Can Res Ther 2015;11:650
|How to cite this URL:|
Bhattacharyya T, Patel FD, Srinivasan R, Rai B, Saha P, Nijhawan R. Rhabdomyosarcoma of vulva in a young lady: A rare case report with review of literature. J Can Res Ther [serial online] 2015 [cited 2019 Nov 22];11:650. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/650/138116
| > Introduction|| |
Rhabdomyosarcoma (RMS) is one of the most common soft tissue sarcoma among young adults. It is most commonly seen in head and neck region followed by paratesticular region, retroperitoneum and extremities. Sarcomas arising from vulva comprise only 1.5-5% of all the vulvar malignancies and leiomyosarcoma is the most common histological variety among those sarcomas. We report a rare case of vulvar RMS presenting with an aggressive behavior in a 21-year-old girl.
| > Case report|| |
The present case report is about a 21-year-old girl presented with rapidly progressive vulvar swelling for 7 months. It was painless to start with, but gradually became painful with increase in size of the lesion. Her medical history was unremarkable. Clinical examination revealed a 12 × 6 cm swelling involving right labia majora and labia minora associated with right inguinal lymphadenopathy 3 × 3 cm in size, hard and fixed. Her routine blood investigations were within normal limit. Contrast enhanced computed tomography (CT) showed a heterogeneously enhancing lobulated soft tissue mass of size 6.7 × 5.1 cm in relation to right labia majus; the mass had ill-defined fat planes with the adjoining muscles of the adductor compartment of the right thigh [Figure 1]. Multiple enlarged lymphnodal masses (largest measuring 3.7 × 3.7 cm) were seen in right inguinal region [Figure 2]. Multiple enlarged discrete and conglomerate mediastinal lymphnodes were also seen as well. There were multiple well defined bilateral lung nodules largest in left mediobasal segment measuring 14 × 12 mm likely representing lung metastasis [Figure 3]. Fine needle aspiration cytology (FNAC) of right vulvar mass came out to be myxoid RMS and FNAC of right inguinal region showed metastatic involvement. She received six cycles of chemotherapy based on injection vincristine 1.2 mg/m 2 intravenous (iv) day 1, injection adriamycin 50 mg/m 2 iv day 1 and injection cyclophosphamide 600 mg/m 2 iv day 1 at 3 weekly interval. As patient showed good response after six cycles of chemotherapy she was given further two more cycles of same chemotherapy regimen. However even after eight cycles of vincristine, actinomycin d, cyclophosphamide (VAC) based chemotherapy, she had local residual disease and her chest X-ray showed bilateral clear lung fields. She had been given local external beam radiotherapy (EBRT) 50 Gy in 25 fractions delivered over a period of 5 weeks. There was a very good clinical response to radiotherapy with almost complete disappearance of the mass and only a small 2 × 2 cm mild indurated area was left behind. Patient was kept on follow-up. Within 2 months of follow-up, she developed recurrence in the form of mediastinal and left supraclavicular lymphadenopathy. She was planned with second line chemotherapy based on injection ifosfamide 1400 mg/m 2 iv day 1-day 3, injection cisplatin 30 mg/m 2 iv day 1-day 3 and injection etoposide 100 mg/m 2 iv day 1-day 3 at 3 weekly interval for six cycles. After six cycles of ifosfamide, cisplatin, etoposide based chemotherapy clinically, there was a 3 × 3 cm hard swelling present over right labia majora. A repeat CT scan was done which showed well defined; heterogeneously enhancing soft tissue lesion measuring 3.4 × 3.9 × 3 cm is noted in the subcutaneous tissues on the right side of pubic region. There was no significant mediastinal or hilar lymphadenopathy. Bilateral lung fields were clear. She underwent wide local excision and surgical histopathology specimen of the vulval mass revealed tumor in subcutaneous plane. The tumor showed an alveolar pattern with tumor cells having round nuclei with clumped chromatin and abundant eosinophilic cytoplasm. Large areas of necrosis were seen. Overall features were of alveolar rhabdomyosarcoma (ARMS) [Figure 4]. The deep resection margin, circumferential resection margins and overlying skin are all free of tumor. She is currently on follow-up and is leading a good quality of life for last 6 months.
|Figure 1: Contrast enhanced computed tomography showed a heterogeneously enhancing lobulated soft tissue mass of size 6.7 × 5.1 cm in relation to right labia majus; the mass had ill-defined fat planes with the adjoining muscles of the adductor compartment of the right thigh|
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|Figure 2: Multiple enlarged lymphnodal masses (largest measuring 3.7 × 3.7 cm) were seen in right inguinal region|
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|Figure 3: Multiple well defined bilateral lung nodules largest in left mediobasal segment measuring 14 × 12 mm likely representing lung metastasis|
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|Figure 4: Histopathology showing a tumour with alveolar pattern and focal necrosis. The tumor cells are pleomorphic and have vesicular nuclei and prominent nucleoli. Inset shows immunoreactivity for desmin (H and E stain, original magnification ×200; inset immunoperoxidase for desmin, original magnification ×200)|
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| > Discussion|| |
Mesenchymal tumors of the vulva are rare. DiSaia et al.  in 1971 reported 12 cases of sarcoma of vulva over 20 years. Davos and Abell  in 1976 gave a detailed pathological description of the various histologic types in 15 cases of sarcoma collected over 39 years. Primary vulvar sarcomas are very rare and approximately account for 1-3% of all vulvar malignancies. Among vulvar sarcomas leiomyosarcoma is the most common entity. RMS of vulva has very rarely been reported. Copeland et al.  in their study have reported eight cases of ARMS of female genitalia with vulva being primary sites in two. Behranwala et al.  reported 17 cases of vulvar neoplasm over a period of 16 years of which only one was RMS. A summary of different cases of vulvar RMS including ours is shown in the [Table 1]. RMS of vulva has favorable outcome and considered one of the most curable form of RMS.  With increasing efficacy of combination chemotherapy the importance of radical surgery has been reduced over the past decades. Approaches to local tumor control, including conservative surgery or radiotherapy have been developed in an attempt to limit long-term sequelae without jeopardizing survival.  Initial surgery is used primarily for diagnosis, although gross tumor resection is occasionally possible without cosmetic or functional deformity. These tumors are often quite sensitive to chemotherapy and many may not require radical surgery or radiation therapy for local tumor control.  Wide excision with clear margins may suffice. The tendency of the tumor toward early metastases encourages the use of adjuvant therapy, radiotherapy for the local disease in either adjacent soft-tissue or lymph nodes and chemotherapy for occult systemic disease.  VAC based chemotherapy remains the standard chemotherapy regimen used in those patients.  Radiation therapy usually is reserved for patients with residual disease after resection or as part of a pre-operative treatment regimen to help limit the extent of surgery. EBRT ranging from 40 to 50 Gy have been used in few case series and some anecdotal case reports to control microscopic disease.  However, there is a paucity of data regarding the optimum treatment of vulvar RMS. More prospective studies are required to decide the optimum therapy. Our report may throw some light in the management of this rare tumor.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]