Difficult situations in sarcoma. Issue 2.

BACKGROUND: Patients with malignant endocrine pancreatic tumors (EPTs) are responsive to combinations of chemotherapy with streptozotocin and 5-fluorouracil/doxorubicin, whereas patients with malignant carcinoids are not. For both categories of patients, alpha-interferon and/or somatostatin analogs can produce long-lasting responses. Cisplatin in combination with etoposide has been suggested to be effective in patients with malignant neuroendocrine carcinomas. The authors used this therapy as second-line or third-line treatment in patients with poorly differentiated and/or rapidly progressing disease. METHODS: Thirty-six patients with histopathologically verified malignant neuroendocrine tumors were included: Eighteen tumors were of foregut origin, of which 5 were atypical, and 15 tumors were EPTs, of which 4 were poorly differentiated endocrine carcinomas. Three tumors were of midgut origin. The median patient age was 47.5 years. The median duration of disease from the time of diagnosis was 12 months. All patients had metastatic disease. Thirty of 36 patients had received previous treatment. Etoposide was given at a dose of 100 mg/m(2) per day for 3 days, and cisplatin was given at a dose of 45 mg/m(2) on Days 2 and 3 as a continuous intravenous infusion that was repeated every 4 weeks. RESULTS: Ten of 18 patients with foregut carcinoids (56%) responded radiologically and/or biochemically, with a median duration of 9 months; and 7 of 14 patients with EPTs (50%) responded radiologically and/or biochemically, with a median duration of 9 months. No difference in response was seen between patients with atypical or typical foregut carcinoids or between patients with well differentiated or poorly differentiated endocrine pancreatic carcinoma. Nineteen of 36 patients (53%) experienced World Health Organization (WHO) Grade 1-2 nephrotoxicity, and 23 patients (64%) suffered from WHO Grade 3-4 neutropenia. CONCLUSIONS: The combination of cisplatin and etoposide can produce significant responses in patients with heavily pretreated and poorly differentiated/rapidly progressing neuroendocrine tumors. The toxicity is considerable, and nephrotoxicity is the dose limiting factor.

Abdominal Neoplasms↗

PubMed2001

Cytologic diagnosis of gastrointestinal stromal tumor with emphasis on the differential diagnosis with leiomyosarcoma.

BACKGROUND: Gastrointestinal stromal tumor (GIST) has only recently been distinguished histologically and immunochemically from morphologically similar neoplasms of the abdomen. METHODS: The authors reviewed 15 cytologic cases of GIST (14 fine-needle aspiration [FNA] specimens and 1 peritoneal fluid specimen) and compared them with 23 cases of leiomyosarcoma (LMS) arising in the abdomen or pelvis (all FNAs). Immunochemistry (IC) was performed on both the cytologic and subsequent tissue specimens if sufficient specimen was available. RESULTS: Cytologic samples of GISTs typically showed irregularly outlined clusters of uniform spindle cells that were spread easily without crush artifact. The cells had wispy cytoplasm with long, delicate, filamentous extensions (13 cases; 87%). A prominent vascular pattern was common (9 cases; 60%); pleomorphism (1 case; 7%) was uncommon. The LMSs showed three-dimensional, tightly cohesive, sharply marginated syncytia of spindle cells, often with nuclear crush artifact. The cytoplasm/stroma had a distinct wiry, refractile appearance (21 cases; 91%); delicate filamentous cytoplasmic extensions (5 cases; 22%) and prominent vessels (3 cases; 13%) were less common. LMSs more commonly exhibited pleomorphism (14 cases; 61%). Epithelioid cytomorphology, mitoses, and necrosis occasionally were observed in both tumor types. IC for c-kit (on cytologic material) was positive in 10 of 10 cases of GIST (usually diffuse and strong) and 2 of 19 cases of LMS (focal). CD34 positivity favored GIST (4 of 9 cases) over LMS (1 of 19 cases). Smooth muscle actin was positive in 20 of 20 LMSs (strong and diffuse) and 6 of 10 GISTs (usually focal). Desmin was positive in 12 of 20 LMSs and was only focally positive in 1 of 11 GISTs. Correlation of IC results was excellent between cytologic and tissue specimens. CONCLUSIONS: Delicate cytoplasmic processes; a prominent vascular pattern; a lack of nuclear pleomorphism; and a c-kit-positive, desmin-negative immunoprofile are characteristic features of GIST and help distinguish these tumors from LMS in cytologic specimens.

Abdominal Neoplasms↗