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Background: Adenoid cystic carcinoma (ACC) of the Breast is a rare tumour (less than 1 % of all breast carcinomas). The aim of this study was to determine the clinical, histological and immunohistochemical characteristics of these tumours., Methods: From the database of the Bergonié Institute of Bordeaux, 30 cases of ACC were identified. The clinical and histological features of these carcinomas were characterized. An immunohistochemical study was performed with the following antibodies: ER, PR, HER-2-neu, Vimentin, EGFR, P63, SMA, CK5/6, CK8/18, CK14, cKIT, MIB1, CD44 and CD24., Results: Thirty patients were included (median age 60.7 years). The 10 axillary lymph node dissections and two sentinel lymph procedures were negative. The architecture was frequently of a mixed type (26/30) and less often solid (4/30). Among the 23 patients for whom follow up was available (median follow-up: 84 months [2-288]), there were three local recurrences and three metastatic events. The tumors with recurrence and metastasis showed more necrosis, a mitotic count greater than 4/10hpf, and in one case perineural infiltration. All the tumours were ER, PR and Her-2-neu negative. Morphological and immunophenotypical analysis disclosed in each tumor, a basaloid and a luminal cell population with divergent immunophenotypical patterns., Conclusions: The mammary ACC is made of two cell types and is of good prognosis despite its triple negative phenotype, similar to the basal-like infiltrating carcinoma NOS. Axillary lymph node dissection is not recommended. Good local control by at least large lumpectomy with long-term follow-up is necessary., (Copyright (c) 2010 Elsevier Masson SAS. All rights reserved.)

We report six cases of carcinomas arising within fibroadenomas. Fibroadenoma is a benign neoplasm occurring in young women. Its association with carcinomas is unfrequent and particularly reported in older women. Few data are available on the histologic features of fibroadenomas harboring malignant lesions. In this study, most cases of fibroadenomas showed cysts, sclerosing adenosis, epithelial calcifications or papillary apocrine changes. These fibroadenomas are classified as complex and are a long-term risk factor for breast cancer. The complex fibroadenoma may be specific of fibroadenoma associated with carcinoma.

The authors present three cases of paraganglioma located in the cauda equina. In all cases pre-operative diagnosis was neurinoma of the cauda equina and only the histology permitted correct identification. All were intradural extramedullary tumors and in two cases the presenting and dominant symptom was low back pain and sciatica. The remaining third case experimented a paraparesis with urinary and fecal incontinence. The follow-up (range of 4 months-4 years) after total excision was uneventful in two cases and showed in the third case with preoperative paraparesis, some persistent urinary incontinence (follow-up: 3 years) with good motor recovery. The light microscopic features were a Zellballen pattern of cells containing argyrophil granules. Electron microscopy was not performed. The most likely theory regarding the embryogenesis of cauda equina paragangliomas is that they arise from pre-existing paraganglia, possibly of the visceral-autonomic group. The prognosis after complete excision appears to be good. The literature is thoroughly reviewed.

Comple remission was obtained in two patients with hairy cell leukaemia and severe bone marrow insufficiency, using combined chemotherapy (rubidazone, arabinosyl cytosine, 6-mercaptopurine and cyclophosphamide). The remissions -- as demonstrated by normal blood cell counts and differentials and complete disappearance of hairy cells and fibrosis in bone marrow biopsies -- have now persisted for 6 and 3 months respectively. Intensive chemotherapy should be considered in patients who, after splenectomy, still have severe bone marrow depletion with persistent anaemia, neutropenia and/or life-threatening thrombocytopenia. Protected environment in a haematology intensive care unit is a prerequisite of intensive chemotherapy in such patients.

Invasion of the internal mammary lymph nodes is common in tumoral pathology of the breast, but an isolated internal mammary lymphadenopathy occurring long after treatment of the primary tumour is a rare event. The authors report one case and discuss the prognostic and therapeutic factors involved.

Of 91 acute leukaemia patients treated with m-Amsa, 19 received intermittent doses, 23 received daily doses and 49 underwent courses with combined m-Amsa and cytosine arabinoside (Ara-C). Intermittent doses had minimal therapeutic activity and toxicity. Among the 23 patients given daily doses, complete remission was observed in 5/12 relapses of ALL and in 2/11 relapses of AML. When Ara-C (200 mg/m2 x 5 days) was administered concomitantly with m-Amsa (200 mg/m2 x 5 days or 120 mg/m2 x 7 days), 17 out of 37 patients with advanced relapses of ALL (13/25 children and 4/12 adults) went into complete remission. While high doses of m-Amsa alone were well tolerated, the combined treatment with high doses of both drugs resulted in severe gastro-intestinal toxicity. Cardiac disorders were observed in patients who had previously received high doses of anthracyclins; there were 5 cases of dysrhythmia and 1 case each of sudden death, ECG alterations and heart failure. In view of its indisputable activity, m-Amsa should be used at an earlier stage in the treatment of acute leukaemias.

Treatment of a panmedullary ependymoma involved a three-stage operation with total excision under microscopic control and the use of the Cavitron. The patient, a 22 year old woman, presented with a three-year history, with clinical onset of staged spinal pain and cervicobrachial neuralgia, of spasmodic paraparesis with sensory and sphincter disturbances. The extent of the lesion from C3 to L2 was determined from data from conventional myelography with Iopamiron, a CT scan with intrathecal contrast and nuclear magnetic resonance imaging of sagittal and frontal sections. The tumor, a grade I ependymoma, was treated by three-stage laminectomies (L2-T12, T12-T3, T3-C3), total excision being obtained by ultrasound fragmentation (Cavitron). Gross pathology showed a heterogeneous appearance with two cysts, one capping the tumor from the bulbospinal junction to C3, the other attached to the medullary cone. Hemorrhagic cavities were noted at cervicothoracic region and multiple microcysts in the dorsal expansion. The postoperative course was uneventful with recovery of walking wearing a bivalve acrylic corset, the most disturbing functional complication being the posterior cord syndrome responsible for an ataxia.

A case of aneurysmal bone cyst of the fifth cervical vertebra was unusual in that it occurred in a 35 year old man treated initially by radiotherapy (3,800 rads), within 3 years, worsening of clinical and radiologic signs led to a complete two-stage exeresis because of extension of lesion to body and posterior arch of C5. This male patient was 35 years old at diagnosis and 38 at time of surgery (respectively 1.2 and 2.5% of cases in the Hay series and 1.9% in the Ruiter series), this lesion affecting mainly age groups under 20 years. Aneurysmal bone cyst (ABC) constitutes 1.4% of primary bone tumors (Dahlin), and spinal localizations 3% (Biesecker), 14% (Reiter) or 20% (Tillman) of total ABC. The cervical lesion represents 13% (Reiter) or 22% (Hay) of spinal localizations, C5 being affected twice in the 17 cases reported by Ameli, and fills, according to Hay, 3.3% of all spinal column lesions and 15% of cervical lesions. Initial treatment applied in another center was by radiotherapy alone at the dose of 3,800 rads (the recommended dose-level in the literature being 20 to 30 Grays). Neither clinical nor radiologic improvement was reported. The recurrence rate after all types of treatment for ABC was 12.6%, and after radiotherapy alone was 11% in the Hay series, MacCarty reporting only one recurrence among 9 patients treated with irradiation alone.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute basophilic leukaemia (ABL) is a rare disease characterized by high fever, anaemia and haemorrhagic diathesis. Its prognosis is somber and its response to therapy mediocre. Death is mainly due to cerebral or digestive hemorrhage and coronary disease. In a very small number of cases ABL, like all diseases accompanied by an increase in basophils, may be associated with hyperhistaminemia responsible for cutaneous and gastric symptoms.

Twenty-one patients with advanced, inoperable epidermoid carcinoma of the oesophagus were treated with combined 5-fluorouracil (F, 600 mg/m2, days 1 and 8), adriamycin (A, 30 mg/m2, day 1) and cis-platinum (C, 75 mg/m2, day 1), together with hydratation and mannitol-induced diuresis. Each course was repeated after 4 weeks. Response could be assessed in all 21 patients and was objective in seven (33%), who had metastasis prior to treatment. Two of these 7 patients went into complete remission confirmed by negative endoscopy and pathology; 5 showed partial response. Median survival of the 21 patients was 8 months; it fell to 4.5 months in non-responders and rose over 9 months in responders. Three patients survived for more than 16 months. No severe bone marrow depression or nephrotoxicity was observed. The 3-drug combination appeared to be more effective than the additive effects of each drug given separately. It is concluded that the FAP regimen is useful in the treatment of advanced oesophageal tumours.

Chemotherapy-induced alopecia observed in cancer patients can now be prevented by a simple, effective, inexpensive and well tolerated procedure: scalp hypothermia. Refrigeration is obtained by placing on the scalp two bags filled with crushed ice 15 minutes before, and removing them 15 minutes after intravenous injection of antineoplastic drugs. Only patients treated with drug combinations that are rapidly administered (into the giving-set tube or by i.v. infusion lasting less than 60 minutes) seem to benefit from scalp hypothermia. The fact that good results were obtained with those drugs (adriamycin, cyclophosphamide, 5-fluorouracil, methotrexate, vincristine) and modes of administration that are most commonly used in women with breast cancer or ovarian cancer makes this procedure extremely interesting.