Genital rhabdomyomas are extremely rare benign tumors of skeletal muscle origin, majority of them being reported in vaginal location. Extensive literature search revealed only three such cases reported in cervix. We hereby report fourth such case of cervical rhabdomyoma in a 35-years-old female patient. The diagnosis was confirmed by histomorphology with desmin and myoD1 positivity on immunohistochemistry. Due to paucity of cases no definite treatment guidelines are available. Differentiation from identical and more common malignant counterpart which is rhabdomyosarcoma is essential to avoid unnecessary aggressive therapy.

Rhabdomyosarcoma is the most common sarcoma diagnosed in childhood and adolescence, arising from the bladder/prostate in only 5%-10% of cases. Treatment-induced cytodifferention of tumor cells into mature rhabdomyoblasts has been reported following chemoradiation and is thought to suggest a more favorable outcome. We report a case of embryonal rhabdomyosarcoma of the bladder/prostate that exhibited extensive cytodifferentiation with downregulation of myogenin and MyoD1 gene expression in rhabdomyoblasts following treatment with chemoradiation therapy. The downregulation of myogenin and MyoD1 expression in rhabdomyoblasts following chemoradiation treatment has not previously been described in the literature and its significant remains uncertain.

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This report presents a case of primary pleomorphic rhabdomyosarcoma arising in the duodenum. A 63-year-old male with persistent melena was referred for a solid tumor in his right upper abdomen detected using ultrasonography. Gastrofiberscopy revealed a protrusion in the upper part of the duodenum, with a large ulcer on the top of it. Enhanced computed tomography showed that the tumor extended to the pancreas. Pancreaticoduodenectomy was performed, despite the absence of malignant cells in the biopsy specimen, with a preoperative diagnosis of duodenal cancer. The tumor consisted of multiple cell types, and immunohistochemical staining was positive for desmin, HHF-35 and alpha smooth muscle actin. Electron microscopy revealed primitive Z-band structures in the tumor. The final diagnosis was pleomorphic rhabdomyosarcoma of the duodenum. This is the first report of primary rhabdomyosarcoma occurring in the duodenum, confirmed by immunohistochemical staining and electron microscopy.

Alveolar soft part sarcoma (ASPS) is a distinctive mesenchymal tumor of uncertain histogenesis, which is exceedingly rare in the uterine cervix. We herein report the smallest (1.6 mm) ever published case of ASPS of the endocervix with the characteristic histomorphology and immunohistochemical findings. One-year follow-up was uneventful. Because of its seemingly much better prognosis, it is important in routine practice to distinguish ASPS from adenocarcinoma of the cervix. This is facilitated by the awareness that ASPS can occur in this location and if there is doubt about the diagnosis on routinely stained sections, the appropriate immunohistochemical study should be performed. Owing to the small number of published ASPSs in the cervix, the optimal treatment strategy has yet to be determined.

Sclerosing spindle cell rhabdomyosarcoma (SSRMS) is a newly recognized entity in adults. The authors report a new case of SSRMS in a 31-year-old woman who presented with a large right leg mass. Biopsy revealed a malignant spindle cell neoplasm with focal sclerotic areas. A diagnosis of monophasic synovial sarcoma was favored initially. The tumor cells in the resection specimen were positive for myosin, myogenin, and MyoD1. Fluorescence in situ hybridization performed on the resection specimen showed no evidence of SYT gene rearrangement in the neoplastic cells, ruling out monophasic synovial sarcoma. A diagnosis of SSRMS was established. The patient succumbed to widely metastatic disease 16 months after initial diagnosis. This case highlights the utility of skeletal muscle markers and cytogenetic testing in distinguishing SSRMS from its mimic, monophasic synovial sarcoma. It is hoped that this case will expand the literature on adult SSRMS and help clinicians and pathologists better understand this newly described entity.

Non-hematopoietic malignancies infiltrating bone marrow have always been a source of erroneous diagnosis. Among these, the small round cell tumors like neuroblastomas and rhabdomyosarcomas mimick the hematopoietic blasts. Several case reports of rhabdomyosarcoma mimicking acute leukemia, clinically and morphologically at presentation have been reported in the literature. To the best of our knowledge such an entity has not been reported in Indian literature. We report here one such case of alveolar rhabdomyosarcoma masquerading as acute leukemia. A thorough clinical examination with high degree of suspicion on bone marrow morphology and judicious use of appropriate immunohistochemistry markers will solve many of these cases.

Rhabdomyosarcoma is the most common soft tissue malignancy in children but is rare in adults. The latest World Health Organization classification of soft tissue tumors recognizes embryonal, alveolar, and pleomorphic rhabdomyosarcomas. More recently, a sclerosing variant of rhabdomyosarcoma has been recognized and reported in seven adult patients. We describe a pediatric case of sclerosing rhabdomyosarcoma presenting as a sacral mass in a 3-year-old girl. Morphologically, the tumor showed a prominent sclerosing hyaline matrix and demonstrated pseudovascular and microalveolar architectural foci. Focal positivity was seen with desmin, smooth muscle actin, and myogenin. MyoD1 showed uniform diffuse nuclear staining. Fusion transcripts were not demonstrated by reverse transcriptase-polymerase chain reaction analysis. The histology, immunohistochemistry, and molecular genetics matched those reported in the seven adult cases of sclerosing rhabdomyosarcoma. This is the first case report, to our knowledge, of this rare tumor arising in the pediatric age group, and we compare the features with those reported in adult sclerosing rhabdomyosarcoma.

Sarcoma botryoides, which usually occurs in the vagina in childhood, is rare in the female reproductive tract of adult women, especially those over the age of 40. We present a case of sarcoma botryoides of the uterine cervix that was an incidental finding in a 46-year-old woman. The tumor formed a grape-like cervical polyp that was locally excised, but rapidly recurred. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. The myxoid tumor had a cambium layer and was composed of spindle-shaped myoblastic cells without apparent cross-striations. The tumor cells were immunoreactive for MyoD1 and myogenin. Postoperative chemotherapy (vincristine and actinomycin D) was given according to the Intergroup Rhabdomyosarcoma Study protocol, and the patient was alive without recurrence 45 months postoperatively. This is the only the fourth case reported in detail of a sarcoma botryoides of the uterine cervix in a patient over the age of 40.