A rare case of leiomyosarcoma of the penis with a reappraisal of the literature

Dominici, A; Rose, A. Delle; Stomaci, Niceta; Pugliese, Lavinia; Posti, Alessandro; Nesi, Gabriella

doi:10.1111/j.1442-2042.2004.00806.x

Cited by 22 publications

(19 citation statements)

References 8 publications

(19 reference statements)

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“…Distant metastasis is rarely observed [2,3,8,10]. The deep type of leiomyosarcoma requires more aggressive treatments, including radical penectomy, radiation therapy, and chemotherapy, although the outcome of these treatments is still poor.…”

Section: Discussionmentioning

confidence: 99%

“…Excision of the regional lymph nodes in deep type leiomyosarcoma has no definite benefits for a patient's survival contrary to squamous cell carcinoma of the penis. Also, radiation therapy and chemotherapy produce little effect in treating leiomyosarcoma of the penis, but they might decrease the risk of local and distant recurrence postoperatively [2,3]. Accepted poor prognostic factors of leiomyosarcoma include deep tumor depth, a tumor size of greater than 5 cm, a high meiotic rate, and necrosis [2,3,8,10].…”

Section: Discussionmentioning

confidence: 99%

“…Among the cases of penile sarcomas, vascular sarcomas are the most common type, followed by rhabdomyosarcomas, leiomyosarcomas, and fibrosarcomas, which also include soft tissue sarcomas. Other types are much more rare [3]. Sixteen cases of epithelioid sarcomas and only 1 case of undifferentiated sarcoma have so far been documented in the English language literature [1,4].…”

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Primary Undifferentiated Penile Sarcoma in Adolescence

et al. 2012

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We report a case of primary penile undifferentiated sarcoma. A 16-year-old adolescent man visited Pusan National University Hospital complaining of a painless mass on his penis that was increasing in size. Magnetic resonance images revealed a 5×5-cm mass and pathological examinations revealed small round cell sarcomas with neuroendocrine differentiation. The tumor, which had metastatic pulmonary nodules, was treated by tumorectomy and systemic chemotherapy. Thirty-four months after the initial diagnosis, the patient was still alive without evidence of local recurrence or metastatic disease. This is our second case of an undifferentiated penile sarcoma.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Primary Undifferentiated Penile Sarcoma in Adolescence

et al. 2012

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“…Rhabdomyosarcoma is a common childhood malignancy with rare occurrence in adults and more than 50% cases are diagnosed in first 2-decades of life. These sarcomas appear to have male predominance with a ratio of 3:1 [3]. Normally, microscopic examination shows tumor cells arranged in sheets and lobules.…”

Section: Introductionmentioning

confidence: 99%

“…They represent only 1-2% of neoplasia in this system [2][3]. Rhabdomyosarcoma is a common childhood malignancy with rare occurrence in adults and more than 50% cases are diagnosed in first 2-decades of life.…”

Section: Introductionmentioning

confidence: 99%

Unveiling Penile Rhabdomyosarcoma

AK¹

2016

JCPCR

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Stated purpose: Rhabdomyosarcomas are a heterogeneous group of malignant tumors representing the most common soft tissue sarcoma in lower genitourinary tract mostly occurs in infants and children in the first 2 decades of life. It is a rare and potentially deadly malignant mesenchymal neoplasia mainly originating from immature striated muscle. Rhabdomyosarcoma cells tend to appear rod-shaped, having an identifiable striated muscular differentiation with rhabdomyoblasts cells. Clinical guidelines and treatment of these tumors are very difficult because rhabdomyosarcomas behave extremely different and requires an early diagnosis and therapeutic modalities involving surgery, chemotherapy and radiation therapy. Material & Methods:In the present study, the authors report a rare case of Grade-II Penile Rhabdomyosarcoma diagnosed in a 2-year male child and have explored the histopathological literature and treatment options for this rare entity. Conclusion:We summarized the results from the available data to the published findings which have recently reported for the proven case of grade-II rhabdomyosarcoma of the penis with treatment options for this rare entity. Considering the limited number of cases reported till now, the conclusions about treatment and prognosis are equivocal. Finally the authors concluded that penile sarcomas are rare tumors with poor prognosis when they involve deep-seated lesions.Keywords: Penile rhabdomyosarcoma; Mesenchymal neoplasia; Neoplasm metastasis histopathological appearance and immunohistochemical profile of the biopsy tissue confirmed it to be a grade-II rhabdomyosarcoma of the penis.With this diagnosis, the patient underwent nine cycles of three weekly intravenous neo-adjuvant chemotherapy with VAC i.e. Inj Vincristine 7 mg, Inj Doxorubicin 20 mg and Inj Cyclophosphamide 200 mg. Initially patient showed good regression up to 6-cycles of VAC chemotherapy but the tumor was still unresectable and hence it was decided to give 3-more courses of VAC, however later the tumor has progressed and in view of the inoperable size of the tumor, it was decided to give second line three weekly intravenous combination chemotherapy with Inj Carboplatin 150 mg and Inj Etoposide 50 mg. Despite three cycles of 2nd line chemotherapy, the disease was progressive and inoperable and the patient further developed multiple lung metastases as reported in MRI chest and abdomen in form of round lesions seen in bilateral lung peripheries. Patient later left the hospital follow-up. Figure 4C) and Vimentin ( Figure 4D). Results and DiscussionRhabdomyosarcoma is an exceedingly rare and highly malignant tumor of childhood with a typical locally invasive neoplasm that accounts for 3.8% of solid tumors in children [4][5][6]. The diagnosis of RMS can be difficult with conventional histological techniques and extensive immunocytochemical staining is strongly recommended to establish the diagnosis. Prognostic factors include mainly lesion size (larger or smaller than 5 cm), extension of invasion (superficial...

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