A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues

Liou, Sofia; Memarzadeh, Sanaz; Dry, Sarah M.; Graham, Rondell P.; Moatamed, Neda A.

doi:10.4322/acr.2021.322

Cited by 1 publication

(1 citation statement)

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“…EMC is an aggressive sarcoma with a high rate of local recurrence (30-50%) and metastasis (30-50%), although long-term survival is high. Patients with this diagnosis are managed with surgery for the primary tumor, and chemotherapy and radiation therapy have been reported to be less effective [1]. According to previous reports, EMC does not have specific clinical features.…”

Section: Introductionmentioning

confidence: 99%

Extraskeletal Myxoid Chondrosarcoma of the Vulva: A Case Report

Omote¹,

Taniguchi²,

Ide³

et al. 2023

Cureus

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Extraskeletal myxoid chondrosarcoma (EMC) of the vulva is extremely rare. We report our experience with a case of disease control by radiation therapy to a localized lesion of EMC. A 41-year-old woman presented to our clinic with a vulvar mass. Magnetic resonance imaging showed a 15 cm mass between the perineum and the medial thigh muscle. It was the "adductor magnus muscle." After the needle biopsy, a histopathological diagnosis of EMC was made. Tissue genomic analysis detected the EWSR1-NR4A3 fusion gene. A joint operation by the Department of Orthopedics, Gynecology, and Plastic Surgery was performed, which included a wide excision of the perineum, partial excision of the medial thigh muscle, and rectus abdominis valvuloplasty. Intraoperatively, pubic infiltration was detected. Postoperative pelvic radiotherapy was administered as adjuvant therapy. Recurrent common iliac lymph node metastases outside the irradiation field and multiple lung metastases were observed. Pazopanib was administered as adjuvant therapy. Pulmonary metastases were controlled, but the pelvic tumor had spread, so the patient underwent radiation therapy. After second-line chemotherapy with doxorubicin, left pleural effusion and mediastinal lymph node metastasis appeared, and third-line chemotherapy with eribulin mesylate was administered. The pleural effusion improved, but the patient developed cough again, and trabectedin was administered as the fourth chemotherapy. In this case, there was no local recurrence for three years after radiotherapy, suggesting the effectiveness of radiotherapy in local control.

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Section: Introductionmentioning

confidence: 99%