Sarcoma botryoides

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and Keywords: Vaginal sarcoma; Embryonal vaginal rhabdomyosarcoma; Botryoid tumor

Overview

Sarcoma botryoides is a rare subtype of embryonal rhabdomyosarcoma. The most commonly affected sites is the vagina, and it occurs more frequently among young girls. The most common clinical finding is vaginal bleeding. Sarcoma botryoides was first discovered by Alexander Brunschwig, an American physician in 1946. There is no classification system for sarcoma botryoides. Genes involved in the development of sarcoma botryoides, include K-ras gene and P53 oncogene.[1] There are no established causes for sarcoma botryoides.[1] Sarcoma botryoides is more commonly observed among girls aged 3 to 8 years old.[2] The median age at diagnosis is 3 years (approximately 38.3 months). If left untreated, patients with sarcoma botryoides may progress to develop direct extension to pelvic soft tissue structures. The mainstay of therapy for sarcoma botryoides, include: vincristine, cyclophosphamide, and actinomycin D. Surgery is the mainstay of therapy for sarcoma botryoides.[1][2]

Historical Perspective

  • Sarcoma botryoides was first discovered by Alexander Brunschwig, an American physician in 1946.[3]

Classification

  • There is no classification system for sarcoma botryoides.[1]

Pathophysiology

  • The pathogenesis of sarcoma botryoides is not fully understood.[1]
  • Genes involved in the development of sarcoma botryoides, include:
  • On gross pathology, characteristic findings of sarcoma botryoides, include:[1][2]
  • Grape-like morphology
  • Fleshy nodular polypoid projections
  • On microscopic histopathological analysis, characteristic findings of sarcoma botryoides, include:[2]
  • Rhabdomyoblasts that may contain cross-striations
  • Tumor cells are crowded in a distinct layer beneath the vaginal epithelium (cambium layer)
  • Spindle-shaped tumor cells that are desmin positive
  • Randomly arranged small cells
  • Myxoid matrix
  • Tadpole-like morphology
  • Positive for Desmin, MyoD1

Causes

  • There are no established causes for sarcoma botryoides.[2]

Differentiating Sarcoma Botryoides from Other Diseases

  • Sarcoma botryoides must be differentiated from other tumors that cause vaginal bleeding, such as:[1]
  • Squamous cell vaginal carcinoma
  • Vaginal adenocarcinoma
  • Melanoma metastasis

Epidemiology and Demographics

  • Sarcoma botryoides is very rare.
  • The prevalence of sarcoma botryoides remains unknown.[2]

Age

  • The median age at diagnosis is 3 years (approximately 38.3 months).
  • Sarcoma botryoides is more commonly observed among patients aged 3 to 8 years old.[1]
  • Sarcoma botryoides is more commonly observed among young children.

Gender

  • Sarcoma botryoides affects females more frequently than males.

Race

  • There is no racial predilection for sarcoma botryoides.

Risk Factors

  • There are no risk factors associated with the development of sarcoma botryoides.[4]</nowiki>

Natural History, Complications and Prognosis

  • The majority of patients with sarcoma botryoides remain asymptomatic.
  • Early clinical features include vaginal discharge, and vaginal bleeding.[2]
  • If left untreated, patients with sarcoma botryoides may progress to develop direct extension to pelvic soft tissue structures.
  • Common complications of sarcoma botryoides, include:
  • Prognosis is generally poor, and the median survival rate of patients with sarcoma botryoides is approximately 10 to 35%.[1]

Diagnosis

Symptoms

  • Sarcoma botryoides may be initially asymptomatic.
  • Symptoms of sarcoma botryoides may include the following:[1]

Physical Examination

  • Patients with sarcoma botryoides usually are well-appearing.
  • Physical examination may be remarkable for:[1]
  • Soft nodules that fill and sometimes protrude from the vagina
  • Polypoid mass

Laboratory Findings

  • There are no specific laboratory findings associated with sarcoma botryoides.

Imaging Findings

  • There are no specific imaging findings associated with sarcoma botryoides.[2]

Treatment

Medical Therapy

  • The mainstay of therapy for sarcoma botryoides, include:[1]

Surgery

  • Surgery is the mainstay of therapy for sarcoma botryoides.[1]

Prevention

  • There are no primary preventive measures available for sarcoma botryoides.[1]

References


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