Müllerian adenosarcorna of the cervix: Differentr'al diagnosis, histogenesis and review of the literature

Phyllodes tumor is an uncommon breast lesion with characteristic histologic appearance when examined by hematoxylin and eosin staining: leaf-like fronds projecting into cystic spaces on low-power microscopy, and biphasic (epithelial and stromal) components on high-power microscopy. We report a rare primary case of this tumor arising within the vulva. A 34-year old African American female presented with a 3 cm slow-growing vulvar mass initially thought to be an inclusion cyst. The lesion was excised and histologic examination demonstrated this lesion to be a rare case of benign phyllodes tumor with morphologic features similar to those arising from breast tissue. Patient received no further treatment and did not exhibit any recurrence or metastasis. Nearly two years after excision, the patient died due to an unrelated medical cause. This rare tumor should be considered in the differential diagnosis for women presenting with a slow-growing vulvar mass.

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“…Mullerian adenosarcoma also display periglandular condensation that distinguishes them from phyllodes tumor. 16 …”

Section: Discussionmentioning

confidence: 99%

Benign Phyllodes Tumor of the Vulva: A Case Report and Literature Review

2015

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Gutartige Tumoren des Uterus

Osmers

2005

Gynäkologe

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Uterine Adenosarcoma: a Review

et al. 2016

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Adenosarcomas are rare malignancies of the female genital tract, accounting for approximately 5 % of uterine sarcomas. Occasionally, adenosarcoma occurs in the ovaries or in extra-uterine tissue, which may be related to endometriosis. These tumors are characterized by benign epithelial elements and a malignant mesenchymal component. Pathologic diagnosis is dependent on the identification of the characteristic morphologic features. The most common immunohistochemical markers for adenosarcoma are CD10 and WT1, but these are not specific. The most frequent presenting symptom is abnormal uterine bleeding. The majority of patients present with stage I disease, with a 5-year overall survival of 60 to 80 %. Survival is influenced by the presence of myometrial invasion, sarcomatous overgrowth, lymphovascular invasion, necrosis, and the presence of heterologous elements including rhabdomyoblastic differentiation. Patients with sarcomatous overgrowth have significantly increased risk of recurrence 23 versus 77 % and decreased 5-year overall survival 50 to 60 %. Standard of care treatment is total hysterectomy with bilateral salpingo-oophorectomy without lymphadenectomy, as the incidence of lymph node metastasis is rare. Retrospective data does not support the use of adjuvant pelvic radiotherapy in uterine adenosarcomas as no survival benefit is seen. Insufficient data exists to recommend routinely neoadjuvant or adjuvant chemotherapy for uterine adenosarcomas. Limited evidence exists for the role of hormonal therapy in uterine adenosarcomas. The PIK3/AKT/PTEN pathway is mutated in ∼70 % of adenosarcomas, and this may represent a possible therapeutic target. This article reviews the current state of knowledge concerning uterine adenosarcoma and discusses the management of this rare tumor.

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Müllerian adenosarcorna of the cervix: Differentr'al diagnosis, histogenesis and review of the literature

Cited by 14 publications

References 21 publications

Benign Phyllodes Tumor of the Vulva: A Case Report and Literature Review

Benign Phyllodes Tumor of the Vulva: A Case Report and Literature Review

Gutartige Tumoren des Uterus

Uterine Adenosarcoma: a Review

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