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Hemipelvectomy with laparoscopic abdominoperineal excision for epithelioid sarcoma treatment
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 loc...
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Published in: | Journal of Coloproctology 2017-09, Vol.37 (3), p.247-250 |
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Main Authors: | , , , , , , , , , , |
Format: | Article |
Language: | English |
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Citations: | Items that this one cites |
Online Access: | Get full text |
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Summary: | 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.
O sarcoma epitelióide (SE) é um subtipo incomum de sarcoma, não chegando a 1% dos sarcomas de partes moles. Foi inicialmente descrito em 1970, sendo que em 1997 uma variante mais agressiva foi classificada como “tipo proximal”. São tumores agressivos, com altas taxas de metástases e recidiva local, com resposta pobre à quimioterapia e radioterapia isoladas. A ressecção cirúrgica com margens livres é o tratamento padrão.
Apresentamos o caso de um paciente de 25 anos com SE tipo proximal avançado em região perineal, submetido a ressecção em outro serviço, evoluindo com recidiva local com invasão do canal anal e musculatura da coxa esquerda. Foi realizado tratamento radioquimioterápico neoadjuvante seguido de ressecção cirúrgica com amputação abdominoperineal do reto por videolaparoscopia associada a hemipelvectomia parcial esquerda incluindo o membro inferior esquerdo (MIE). O paciente apresentou boa evolução pós operatória e encontra-se em segmento ambulatorial. O anatomopatológico evidenciou margens cirúrgicas livres.
O SE proximal é um tipo raro de sarcoma de partes moles, cujo tratamento curativo é desafiador, em virtude dos altos índices de recidiva local e metástases à distância. |
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ISSN: | 2237-9363 2317-6423 2317-6423 |
DOI: | 10.1016/j.jcol.2017.03.005 |