Hemipelvectomy with laparoscopic abdominoperineal excision for epithelioid sarcoma treatment

Abstract

The epithelioid sarcoma (ES) is an unusual variant of the sarcoma, not reaching 1% of all soft tissue sarcomas. Initially reported in 1970, it has a more aggressive variant, which was classified as the “proximal-type” in 1997. These are aggressive tumors with high rates of distant metastasis and local relapses. Isolated radio and chemotherapy responses are poor and free-margin surgical resection is the treatment of choice.This is the case report of a 25-year-old male patient diagnosed with “proximal-type” ES in the perineal region. He underwent surgical resection in another institution and was later admitted to our institution with local recurrence with rectal and left thigh muscle invasion. Neoadjuvant radio-chemotherapy was performed, followed by laparoscopic abdominoperineal rectal resection and partial left hemipelvectomy associated with left lower-limb amputation. The patient had no postoperative complications and is currently undergoing outpatient follow-up. The anatomopathological analysis showed tumor-free margins.The “proximal-type” ES is a rare soft-tissue sarcoma subtype. High local recurrence, as well as the metastasis rates, makes its treatment a challenging task.

Anal canal epithelioid sarcoma: Case report and literature review

“…Epithelioid sarcoma is an histological subtype part of this heterogeneous group of soft tissue tumors and constitute 1% of it, in addition to being classified as malignant [4,6]. Just like any other sarcoma its usual location is the extremities; however, there is a proximal variant, which has a worse prognosis with higher recurrence rate and it can be distinguished by its location: in the trunk, pelvic region, perineum and genitals, in addition to having a rhabdoid cell morphology compared to the classic, with appearance of granulomatous tissue and spindleshaped cells among other histopathological characteristics [1,4].…”

“…They are prone to spread via the lymphatic and show little response to chemotherapy and radiotherapy, being the only treatment radical surgical resection. They have a survival rate of 50-65% in five years [4,6].…”

“…The latter, combined with its low incidence, makes this a diagnostic challenge. Therefore, imaging studies play a crucial role in the evaluation of this group of tumors [1,3,4].…”

“…The standard curative treatment for soft tissue tumors is surgical excision, being the local wide excision the most frequently performed [1,4,5]. Transanal excision should be reserved for tumors with benign characteristics, such as: smaller than 3 cm and located more than 8 cm from the anal margin.…”

“…Within this rare heterogeneous tumors, the epithelioid sarcoma (ES) constitutes less than 1% of all soft-tissue sarcomas; ES is characterized by its slow growth, predominance in young male adults, the tendency to originate in extremities and its resistance to chemo and radiotherapy. There is a poorly described proximal variant, that affects the trunk, pelvis, perineum and genitalia, which comes with a worse prognosis for its lack of histological differentiation, aggressive behavior, high re-currence rate and lymphatic spread causing metastasis to the lungs [4,5]. We present the case report of a 71-year-old male with an proximal epithelioid sarcoma of the anal canal which was successfully removed.…”