CLINICAL PRESENTATION AND LONG-TERM OUTCOME OF PATIENTS WITH ECTOPIC ACTH SYNDROME DUE TO BRONCHIAL CARCINOID TUMORS: A ONE-CENTER EXPERIENCE.

OBJECTIVE: To describe the diagnostic features and long-term outcome of patients with bronchial carcinoid tumors with ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS), treated in our department. METHODS: We studied 10 cases with EAS and histologically confirmed bronchial carcinoid tumors, diagnosed from 1992 until 2006. Diagnosis was based upon blood, urine, radiologic, and interventional tests. Disease status at the time of the last follow-up was the primary outcome measure. RESULTS: Clinical manifestations included Cushingoid features (100%), psychiatric symptoms (90%), hypertension (70%), diabetes/impaired glucose tolerance (40%), osteoporosis (10%), and hypokalemia (10%). The average time from the onset of symptoms until diagnosis was 14.2 ± 17.0 months. None of the patients exhibited a positive cortisol or ACTH response to corticotropin-releasing hormone (CRH) test, and none showed a positive gradient on bilateral inferior petrosal sinus sampling (BIPSS). All tumors were identified by computed tomography and by octreotide scintigraphy in 8 patients. All patients underwent surgical resection of the tumor, and 2 patients had adjuvant radiation therapy. The mean follow-up was 126.6 ± 63.3 months. At latest follow-up, 8 patients were in remission and 2 had recurrence of the EAS; both had a multifocal tumor. The 2 patients submitted to adjuvant radiation therapy were in remission at their latest follow-up, despite local invasion and lymph node metastases. CONCLUSION: CRH test and BIPSS are the most useful methods in diagnosing EAS. For localization, repeated imaging studies are necessary. Surgical treatment is effective in most cases. Adjunctive radiotherapy may be useful in patients with lymph node metastases. Patients with multifocal disease should be monitored for potential recurrence.

Solid variant of primary effusion lymphoma in successfully treated HIV infection: a case report.

Primary effusion lymphoma (PEL) is a unique form of non-Hodgkin lymphoma, mainly met in severely immunocompromised, HIV-positive patients. PEL is aetiologically related to human herpes virus-8 (HHV-8) and it usually presents as a lymphomatous body cavity effusion in the absence of a solid tumour mass. Recently, cases of HIV-positive patients with HHV-8-positive solid tissue lymphomas, not associated with an effusion, have been reported (solid variant of PEL). The prognosis of PEL is reported to be poor. We report a case of an HIV-positive patient with a typical solid variant of PEL without effusion. Interestingly, his disease developed while being on stable antiretroviral therapy (ART) with high CD4 counts. He had a relatively long survival with chemotherapy and ART.

Primary chest wall tumors: early and long-term results of surgical treatment.

OBJECTIVES: A retrospective study of primary chest wall tumors (PCWTs/CWTs) was conducted to review their clinical, radiological and pathological features, as well as the early and long-term results of surgical management. MATERIALS AND METHODS: From 1986 through 1996, 41 patients (18/44% male, 23/56% female, aged 15-78 years) with PCWTs were treated in our department. RESULTS: Twenty-three patients (nine male, 14 female, mean age 36 years) had a benign CWT: enchondroma, five patients; fibrous dysplasia, four patients; neurilemmoma, three patients; osteochondroma, two patients; granular cell tumor, two patients; fibroma, two patients; lipoma, two patients; fibrolipoma, one patient; eosinophilic granuloma, one patient; aneurysmal bone cyst, one patient. Eighteen patients (nine male, nine female, mean age 59 years) had a malignant CWT: plasmacytoma, five patients; chondrosarcoma, two patients; osteosarcoma, two patients; fibrosarcoma, two patients; desmoid tumor, two patients; leiomyosarcoma, one patient; malignant fibrous histiocytoma, one patient; tendon sheath sarcoma, one patient; hemangiosarcoma, one patient; neurinosarcoma, one patient. The ribs were involved in 21 patients, the sternum in five patients, and the soft tissue in 17 patients. Distinction between benign and malignant CWT was not possible using radiographic criteria alone, and diagnosis was always confirmed histologically. Surgical treatment consisted of wide resection in 29 patients (15 benign/14 malignant CWTs), with the use of synthetic mesh in five cases, and excisional or incisional biopsy in 12 patients. There was no perioperative mortality. Two patients with a benign CWT (fibroma, one patient; neurilemmoma, one patient) had a local recurrence at 13 and 26 months after resection, respectively, and underwent wide resection (recurrence rate 8.7%). Follow-up at 3-13 years revealed one non-tumor-related death in patients with benign CWT (overall mortality rate 4.3%) and no other local recurrence. The overall 5- and 10-year survival in patients with malignant CWT was 33.3%. CONCLUSIONS: We believe that all CWTs should be considered malignant until proven otherwise. Wide resection with tumor-free margins is required in order to provide the best chance for cure in both benign and malignant lesions.