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Sarcoma 101

Risk factors

• Genetic syndromes: Li-Fraumeni (p53), familial adenomatous polyposis, Carney-Strakis, hereditary retinoblastoma (Rb), Neurofibromatosis (NF1)
• Germline mutations: succinate dehydrogenase
• Exposures: radiation, cyclophosphamide

Sarcoma types

• Liposarcoma: originate from adipose, can be well differentiated, de-differentiated, or pleiomorphic
• Leiomyosarcoma: originate from smooth muscle, can occasionally occur with EBV infection in immunocompromised patients
• Rhabdomyosarcoma: originate from skeletal muscle
• Malignant fibrous histiocytoma: originate from fibroblasts or histiocytes
• Solitary fibrous (hemangiopericytoma): can cause Doege-Potter syndrome (hypoglycemia from producing IGF), usually radiosensitive
• Ewing: more common in bone
• Perivascular epithelioid cell (PEComa): group of tumors including AML, may respond to mTOR inhibitors

Generalized management

• Goal: surgical R0 resection via wide local excision
• Adjuvants: questionable role for XRT and chemotherapy
• Recurrence: ideally managed with further resection

Sarcoma locations

Retroperitoneal

• Presentation: nonspecific, changes in weight/appetite
• Imaging: CT C/A/P with IV+PO contrast, may warrant MR A/P
• Biopsy: recommended, needed to differentiate from other sources of retroperitoneal masses and assess LN involvement
• Treatment: plan for R0 resection, consider neoadjuvant XRT (preferred over adjuvant)
• Incomplete resection: only consider for palliative benefit
• Follow-Up: chest/abdominal imaging with exam q3-6mo for 2-3yr, then q6mo for 2yr, then annually
• Recurrence: dependent on type of sarcoma, 5yr survival ranges 40-90%

Paratesticular + Spermatic Cord

• Presentation: unilateral firm mass next to testicle, usually painless but can be painful in certain subtypes
• Imaging: obtain scrotal US but cannot usually differentiate benign from malignant lesions
• Removal: ideally wide local excision via inguinal approach, if initially removed scrotally then recommend repeat surgery via inguinal approach with removal of all tissue including scrotal skin (hemiscrotectomy)
• Lymphadenectomy: not usually warranted, tumor spreads contiguously not via lymphatics, only rhabdomyosarcoma should warrant unilateral pelvic and retroperitoneal LND due to higher likelihood of lymphatic spread
• Adjuvant therapy: XRT may decrease local recurrence but not overall survival, chemo not recommended (except for RMS)

Prostate

• Presentation: retention, hematuria, dysuria, ejaculatory pain, constipation, weight loss, normal PSA
• Diagnosis: via prostate biopsy or transurethral resection
• Treatment: consider prostatectomy if no metastases, minimal benefit for chemotherapy if metastatic disease
• Prognosis: overall poor, negative margins and no metastases at diagnosis increase chances

Renal + Bladder

• Presentation: hematuria, obstruction, pain
• Workup: CT C/A/P, biopsy for diagnosis
• Treatment: surgical resection, consider neoadjuvant or intraop XRT