Vagina
Malignant tumors
Malignant mixed tumor

Author: Shweta Gera, M.D. (see Authors page)
Editor: Arzu Buyuk, M.D.

Revised: 28 September 2017, last major update July 2014

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Malignant mixed tumor vagina

Cite this page: Gera, S. Malignant mixed tumor. PathologyOutlines.com website. http://pathologyoutlines.com/topic/vaginaMMT.html. Accessed October 23rd, 2017.
Definition / general
  • Primary vaginal biphasic tumor with malignant epithelial and malignant spindle cell elements with atypia and mitoses
  • No evidence of another primary malignancy that may give rise to vaginal metastases
Terminology
Epidemiology
Sites
Pathophysiology
  • Not clearly known - theories include collision, combination, composition, metaplastic
  • Collision: carcinosarcoma has biclonal origin with two separate but synchronous neoplastic clones fusing to form a "collision" tumor
  • Combination: neoplasms are monoclonal and the carcinomatous and sarcomatous elements share a common stem cell
  • Composition: mesenchymal component is not truly neoplastic but reactive; not a valid theory because this component is always malignant on histology
  • Metaplastic: recent theory that favors a common cell origin like the combination theory but suggests that the epithelial or the mesenchymal component gives rise to the other via metaplasia of a subclonal population (Arch Gynecol Obstet 2005;271:264)
    • HPV 16 has been detected in both epithelial and sarcomatous elements, supporting metaplastic theory of histogenesis (Am J Surg Pathol 2001;25:338) although the role of HPV has not been clearly defined
  • May originate from any site in Müllerian tract as well as ovary and peritoneum and may arise from glandular or squamous precursors (Gynecol Oncol 1998;70:303)
Etiology
Clinical features
Diagnosis
  • Diagnosis of primary MMMT of the vagina should be based on the following:
    1. Location in the vagina without involvement of the cervix or vulva
    2. Mixed histological appearance of (a) squamous or glandular and (b) spindle cell elements, showing mitoses and atypia
    3. Invasion and possible metastasis
    4. Lack of evidence of other primary malignancy that may have given rise to vaginal metastasis (Gynecol Oncol 1998;70:303, Arch Gynecol Obstet 2005;271:264, South Med J 1975;68:1239)
Radiology description
  • Chest Xray, CT scan of the abdomen and pelvis to look for metastases and stage the tumor
Prognostic factors
  • Poor prognosis
  • According to one study, 57% of the patients died within 23 months and one patient developed a neck node metastasis within 6 months
  • Can metastasize to distant sites like supraclavicular nodes (Gynecol Oncol 1998;70:303)
Case reports
Treatment
Gross description
Microscopic (histologic) description
  • Tumor is composed of intimately associated epithelial and mesenchymal components
  • Epithelial component could be a variety of histological subtypes, alone or in combination, including squamous cell carcinoma, basaloid squamous carcinoma, adenocarcinoma, adenosquamous carcinoma, adenoid basal carcinoma, adenoid cystic carcinoma, undifferentiated carcinoma
  • Sarcomatous component may be homologous (fibroblasts and smooth muscle) or heterologous (cartilage, striated muscle, bone, etc.)
  • Predominant epithelial component is squamous cell carcinoma, while heterologous elements are rare (Pathol Res Pract 2011;207:253)
  • Necrosis can also be present (Gynecol Oncol 1998;70:303)
Microscopic (histologic) images

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Carcinosarcoma

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Various images

Cytology description
  • Both epithelial and spindle cells elements can be seen on Pap smear
  • Carcinomatous component is poorly differentiated with a high nuclear/cytoplasmic ratio and can be confused with squamous carcinoma in situ
  • Atypical spindle cells may represent sarcomatous component
  • Necrotic tumor diathesis can be present in the background (Am J Clin Pathol 2004;122:434)
Cytology images

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Pap stain

Positive stains
Negative stains
Differential diagnosis