[Primary thymic carcinomas. Three cases and a review of the literature]. / Carcinomes thymiques primitifs : à propos de trois cas avec revue de la littérature.

Thymic carcinoma is a very rare malignancy. In 1999, a World Health Organization committee published histologic criteria for distinct thymoma entities (labelled as type A, AB, B1, B2, B3 thymomas) and for the heterogeneous group of thymic carcinomas, collectively called type C tumour. Thymic carcinoma differs from thymoma in that it displays cytologically malignant features, extensive local invasion, and a substantial potential for metastasis. It constitutes a heterogeneous group of tumours that display different biological behaviours and prognoses. The majority of thymic carcinomas are either squamous carcinomas or lymphoepithelioma-like carcinomas. This study included three male patients aged 20, 46 and 19years respectively with histologically proven thymic carcinoma diagnosed at the author's institution. All of the patients presented a large mass of the anterior mediastinum. Histological examination of the different tumours revealed three distinct variants of thymic carcinoma, namely: epidermoid carcinoma, clear cell carcinoma and lymphoepithelioma-like carcinoma.

Costal chondrosarcoma. Report of five cases.

We report five cases of coastal chondrosarcoma (CS) in four women and one man between 28 and 49 years of age. In four cases, the tumor had spread and infiltrated the adjacent structures (soft tissues, thoracic vertebrae, mediastinum, etc.). CS was diagnosed based either on wide surgical resection specimens in three patients, or on tumor biopsies in two cases. The CS was grade I in one patient, grade II in three cases, and grade III in one case. Wide surgical resection which was performed in three patients was associated in one case with adjuvant radiotherapy and chemotherapy. In the three cases, the disease natural history appeared favorable after a follow-up duration ranging from 1 month to 4 years. Two patients died, one after radiotherapy and chemotherapy done immediately after diagnosis as a result of the large size and invasive nature of the tumor. Coastal CS is characterized by a distinct potential for locoregional and distant metastasis. Diagnosis remains anatomopathological. Wide surgical resection is the only demonstrated curative treatment, even for high-grade CS.

A pulmonary mucinous cystic tumour of borderline malignancy.

We report a well-documented case of pulmonary mucinous cystic tumour of borderline malignancy involving the left lower lobe. The lesion was found incidentally by chest radiograph and CT scan with a provisional diagnosis of bronchioloalveolar carcinoma. The tumour was 4 cm in its greatest dimension, cystic and filled with gelatinous mucus. Microscopically, the neoplastic mucinous epithelium was composed of cuboidal cells with focally nuclear stratification and mild to moderate nuclear atypia. The patient has remained free from recurrence or metastases for 6 years. Pulmonary mucinous cystic tumour of borderline malignancy is a rare, recently described neoplasm, which spans a spectrum of tumours with malignant potential. The recent World Health Organization classification of lung tumours does not recognize this entity, which has a very good prognosis, and as such should be distinguished from classic pulmonary adenocarcinoma. Histological diagnosis can be difficult to distinguish from cystic bronchioloalveolar carcinoma or metastatic mucinous adenocarcinoma.

[Intracerebral schwannoma: case report]. / Schwannome intracérébral: étude anatomoclinique d'une observation.

BACKGROUND AND PURPOSE: We report a well-documented case of intracerebral schwannoma, presenting as a cystic and solid parietal mass, arising in a 20-year-old woman. Possible mechanisms underlying the histogenesis of this rare lesion are discussed. METHODS: The clinical, radiological and pathological features are described. The relevant literature is reviewed. RESULTS: The patient presented seizures and elevated intracranial pressure. Neuroradiologic findings showed a right parietal lesion with cystic and tissular components, intensely enhanced after injecting intravenous gadolinium. The tumor was removed through a right parietal craniotomy. Histological and immunochemical features confirmed the diagnostic of intracerebral schwannoma. The patient is alive without progressive local disease or metastasis. The origin of intracerebral schwannomas has been the source of much controversy. The most popular hypothesis argues that these tumors arise from the proliferation of Schwann cells in the perivascular nerve plexii. CONCLUSIONS: Intracerebral schwannoma is an extremely rare benign tumor. About fifty cases have been reported. The importance of recognizing this tumor is stressed, particularly in younger patients, given its benign nature, radiological resemblance to other tumors and favorable response to resection without toxic treatment.

Paraganglioma: report of a rare case with ovarian involvement.

We report a well-documented case of paraganglioma involving right ovary, which was initially misdiagnosed as a Sertoli-Leydig cell tumor and recurred one year later. The right ovarian tumor measured 105x90x60 mm and was associated to a subdiaphragmatic tumor measuring 80x60x35 mm, a peritoneal and a preureteral nodules measuring 10 mm either. Microscopically, tumor cells were arranged in trabeculae and cords separated by a delicate stroma. Their cytoplasm was abundant granular and eosinophilic. Their nuclei were enlarged and regular in size with coarse chromatine and a large nucleolus. The tumor expressed neuroendocrine markers (chromogranin, synaptophysin) epithelial membrane antigen and focally cytokeratin 7 and E-cadherin. Pathological ovarian paraganglioma diagnosis could be difficult but one should be aware of its bona fide existence. The clinical course is favourable in most of the cases.

[Darrier and Ferrand dermatofibrosarcomas--retrospective analysis of 81 cases over ten years (1983-1994)]. / Les dermatofibrosarcomes de Darier et Ferrand--analyse rétrospective de 81 cas sur dix ans (1983-1994).

Progressive and recurrent dermatofibrosarcoma, described by Darier and Ferrand in 1924, is a fibrous skin tumor with essentially local malignancy. This tumor is often only diagnosed after several recurrences. The authors present a retrospective study of eighty-one cases treated over a ten years period (1983-1994) and a review of the literature. The treatment is surgical: wide and deep excision with respection of the safe subjacent aponeurotic plane. Therapeutic modalities are defined, with particular emphasis of the need for primary surgical treatment by excision, the possibility of in this case being significantly greater than after salvage operations. The prognostical factors are clinical factors such as size of the tumor, the risk of malignant evolution or the need for repeated excisions.

Phase I trial of homoharringtonine in children with refractory leukemia.

Continuous infusion of homoharringtonine was administered to 17 children with refractory leukemia. Ten children with acute lymphoblastic leukemia received a total of 18 courses and seven children with acute nonlymphoblastic leukemia had a total of 13 courses. Doses were escalated from 1.65 to 8.5 mg/m2 for 5-10 consecutive days. Side effects included mild nausea and vomiting and transient changes in liver enzymes. Mucositis and diarrhea were more frequently seen at higher dose levels. Grade 3 hypotension and pain were seen at doses of 7 mg/m2 for 10 days. This is considered to be the maximum tolerated dose in this limited phase I trial. None of these previously heavily treated patients achieved a marrow remission.

Phase I and clinical pharmacological study of 4-demethoxydaunorubicin (idarubicin) in children with advanced cancer.

We conducted a phase I and pharmacokinetic study of i.v. idarubicin, a new anthracycline analogue, in 42 evaluable children 1-19 years old. Twenty-seven had leukemia and 15 had various solid tumors. The drug was administered in escalating doses of 10 to 40 mg/m2/course in 3 equal fractions over 3 consecutive days at 14- to 21-day intervals. Myelosuppression and mucositis were the limiting toxicities for short-term administration. Nausea, vomiting, and elevation of liver enzymes and bilirubin were the other toxicities encountered. Peak toxicity occurred 2 weeks after drug administration with median recovery by day 24. All but 4 patients with solid tumors had prior anthracyclines. Mild cardiac function changes without clinical symptoms were observed in 17 of 35 patients measured by serial cardiac evaluations. In addition, there were 4 patients with congestive heart failure. On postmortem examination, 4 patients had changes consistent with anthracycline cardiomyopathy at a prior median total anthracycline dose of 175 mg/m2. The maximum tolerated dose for patients with solid tumors was 15 mg/m2 course in 3 divided doses. Patients with leukemia tolerated 30 mg/m2/course. Six of 15 evaluable patients with acute lymphoblastic leukemia who received greater than or equal to 30 mg/m2 idarubicin achieved a remission (M1 marrow status). The plasma clearance of idarubicin fits a 3-compartment model with a harmonic mean half-life of 2.4 min, 0.6 h, and 11.3 h for alpha, beta, and gamma phases, respectively. Idarubicinol was the only metabolite detected in the plasma and it accumulated during the 3 days of therapy. Idarubicin is similar to daunorubicin in pharmacology and toxicity. While the cardiotoxic dose still must be delineated, the complete remission achieved in multiple relapsed patients with acute lymphoblastic leukemia indicate promising activity in at least that disease.

Phase I study of carboplatin (CBDCA) in children with cancer.

A phase I study of carboplatin (CBDCA) was performed in 40 children with advanced cancer. A single course of CBDCA consisted of 4 weekly 1-hour infusions followed by a 2-week rest. The starting dose of 100 mg/m2/week was 66% of the maximum tolerated dose in adults. Escalated dose levels given were: 125, 150, 175, and 210 mg/m2. Myelosuppression was dose limiting, with thrombocytopenia more pronounced than leukopenia. There was no evidence of cumulative toxicity. The maximum tolerated dose for children with solid tumors was 210 mg/m2/week X 4. Other side effects included transient nausea and vomiting at the higher dose levels and non-dose-related, reversible changes in creatinine clearance. One patient developed hives. No hepatic toxicity was seen. Among the 28 evaluable patients with solid tumors, one of ten with osteogenic sarcoma had complete disappearance of a lung nodule for 15+ months. Two of four patients with medulloblastoma had partial responses by clinical and computerized tomographic scan for 4 and 10 months. All three responders had received prior cisplatin therapy. CBDCA has major advantages over cisplatin in terms of reduced toxicity. Responses observed in patients previously treated with cisplatin are encouraging. The recommended phase II dose for children with solid tumors is 175 mg/m2/week X 4 with a 2-week rest.