Esophageal pleomorphic rhabdomyosarcoma: a rare high-grade sarcoma managed with upfront resection

TL;DR

A rare case of esophageal pleomorphic rhabdomyosarcoma was successfully treated with early surgical removal.

Contribution

This case highlights the effectiveness of upfront resection in managing localized pleomorphic rhabdomyosarcoma of the esophagus.

Findings

The patient underwent immediate esophagectomy for a large localized PRMS tumor.

Final pathology confirmed a high-grade PRMS without lymph node involvement, classified as Stage IIIA.

The case emphasizes the need for early surgery due to limited response to chemoradiation in PRMS.

Abstract

Primary esophageal sarcomas are exceedingly rare, with a subtype of pleomorphic rhabdomyosarcoma (PRMS) as exceptionally uncommon. We present the case of a healthy 58-year-old male with significant smoking history with 3 months of progressive dysphagia and 30 lb weight loss. Endoscopy revealed a large esophageal mass with biopsy demonstrating a high-grade malignant neoplasm with spindle cell features, favoring sarcoma. Staging workup confirmed a localized intraluminal tumor without distant metastasis. Given the progressive nature of the mass and near-complete obstruction, the patient underwent immediate esophagectomy. Final pathology demonstrated an 8.7 cm high grade PRMS involving the mucosa and submucosa without lymph node involvement, Stage IIIA (pT2N0M0), based on the American Joint Commission on Cancer (AJCC) criteria. This case underscores the importance of distinguishing sarcomas from esophageal carcinomas and recognizing early surgery as the cornerstone of management in localized PRMS given its limited responsiveness to chemoradiation.

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