Elsevier

Gynecologic Oncology

Volume 107, Issue 1, October 2007, Pages 130-135
Gynecologic Oncology

Review
“Proximal-type” epithelioid sarcoma vs. malignant rhabdoid tumor of the vulva: A case report, review of the literature, and an argument for consolidation

https://doi.org/10.1016/j.ygyno.2007.05.045Get rights and content

Abstract

Background.

Vulvar sarcomas, which comprise less than 2% of vulvar malignancies, are a heterogeneous group of tumors. Sub-categorization of these tumors makes treatment strategies difficult to evaluate. “Proximal” type epithelioid sarcoma (PES) and malignant rhabdoid tumor (MRT) describe a rare subset of vulvar malignancies which share histologic features and an aggressive clinical course.

Case.

We describe a case of vulvar PES/MRT in a 35 year old female treated with radical excision, inguinal–femoral lymphadenectomy, and adjuvant radiation who remains without evidence of disease 40 months after surgery. We review all English language reports of vulvar PES or MRT, and compare treatments and outcomes.

Conclusion.

Vulvar PES/MRT is a rare disease attended by high rates of local and distant relapse. Radical excision remains the foundation of treatment. The role of adjuvant therapy, and particularly radiation, remains unclear but merits consideration.

Introduction

Proximal-type epithelioid sarcoma (PES) of the vulva is an exceedingly rare, aggressive mesenchymal tumor first described by Enzinger in 1970 [1]. Histologic similarity between PES and extrarenal malignant rhabdoid tumor (MRT) has prompted some authors to postulate that these entities may be a single disease [2]. These tumors are differentiated from the more common distal epithelioid sarcoma counterpart by an aggressive pattern of local recurrence despite negative margins and a propensity for early metastasis [3]. They occur in younger women, predictably in the 3rd to 5th decades of life, and the prognosis is generally poor. The role and choice of adjuvant therapy remain unclear owing to the rarity of disease.

We review the literature and report a case of proximal-type epithelioid sarcoma of the vulva successfully treated with radical vulvectomy, ipsilateral lymph node dissection, and postoperative radiation.

Section snippets

Case presentation

A 35 year old nulliparous Caucasian female presented with a right vulvar mass enlarging over a period of weeks. Vulvar biopsy revealed a poorly differentiated malignancy, composed of gland-like large alveolar clusters and focal spindle cell components (Fig. 1). The tumor stained positive for EMA, vimentin, and cytokeratin AE1/AE3. CD34 staining was negative, and the pathologists favored a diagnosis of PES.

At presentation the lesion measured 4 × 3 cm with a gross depth of 3 cm. It was mobile in

Discussion

Epithelioid sarcoma, a rare soft tissue malignancy of mesenchymal origin, was first described by Laskowski in 1961 [4]; however, Enzinger was the first to attempt to establish it as a distinct entity [1]. These rhabdoid appearing tumors can be distinguished from poorly differentiated carcinoma, rhabdomyosarcoma, synovial sarcoma, malignant mesothelioma, and melanoma using immunohistochemistry [5]. Differentiation from extrarenal rhabdoid sarcoma has been more difficult, however, because

Presentation

Patients with PES/MRT typically present between the 3rd and 5th decade of life with a visibly enlarging but painless nodule in the trunk, commonly in the axilla or genitals. Occurrences in the mons, clitoris, Bartholin’s gland, and vulva have all been reported. The growth rate and symptom pattern appear variable and considerable treatment delay is described by Ulutin et al. who observed a median time to diagnosis of 6 months [7]. Despite similar microscopic features, the “proximal” variant of

Macroscopic findings

Lesions are firm to fleshy, white with focal pigmentation, and can consist of either single or multiple nodules. Focal necrosis and hemorrhage can produce a yellow to brown appearance. The mass arises in the reticular dermis and typically remains superficial but can extend deeply [2]. Most authors have reported that surgical specimens have a negative margin implying that invasive projections are uncommon.

Microscopic findings

The distinguishing architectural features of PES/MRT include a nodular arrangement of

Management

Current treatment options for MRT/PES are modeled after the treatment of distal and extra-genital sarcomas. The cornerstone of management remains surgical extirpation with wide margins (> 2 cm) [18]. Inadequate margins have been associated with an increased risk of local recurrence [11], [12]. The presence of lymphatic metastases worsens the prognosis, but clinical benefit has not been consistently demonstrated for routine or contralateral lymph node dissection in the absence of clinically

Prognosis

The reported 5 year overall survival rates for PES/MRT, primarily “distal-type”, range from 50% to 80% [18], [19], [21]. The prognosis for centrally located “proximal” lesions, however, appears worse. Local recurrence despite negative surgical margins is common and distant metastasis eventually occurs in up to 60% of cases [19], [20], [21], [22]. Hasegawa et al. reviewed 20 cases of PES/MRT and noted a tumor-related mortality of 65%; however, the management of these patients varied extensively.

Conclusion

Given the similarity in histology, immunohistochemical staining patterns, and now clinical behavior between reported cases of PES and MRT we would advocate a “lumping” rather than “splitting” approach to this condition. The rarity of the disease and the varied therapeutic strategies employed to date preclude definitive statements regarding treatment. Nevertheless, since most lesions present as an isolated finding and recurrence of any sort in attended by a poor prognosis, we believe that

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