Translocation of BCL2 and BCL6 to the same immunoglobulin heavy chain locus in a case of follicular lymphoma.

Follicular Lymphoma is a low grade malignancy of mature B-cells. The hallmark chromosome abnormality is the translocation t(14;18) which is observed in 70 - 80% of cases with a translocation t(3;14) present in a further 10%. Rarely both of these translocations, or one of their variants, may be present. These co-incident translocations usually involve different Ig loci or different Ig alleles. We present here a case of Follicular Lymphoma with leukemic presentation and a complex translocation involving the IgH, BCL2 and BCL6 loci. Double oncogene translocations to a single immunoglobulin locus are extremely rare in lymphomas with few cases described to date. To our knowledge this is the first reported case with a complex translocation involving these loci.

Complicated Meckel's diverticulum diagnosed preoperatively in three cases by complementing CT with detailed us studies.

The clinical and imaging features of Meckel's diverticulum of the adult are reviewed through the report of three complicated cases, one presenting with perforation, the second with intestinal bleeding, and the third with intestinal occlusion due to phytobezoar impaction. Intradiverticular heterotopy of gastric mucosa was the responsible common denominator for the first two cases. Intradiverticular calcified entheroliths were also found in the second case. Obstruction due to a phytobezoar as observed in the third case is a very rare presentation. This report clearly emphasizes and encourages the approach of this difficult diagnosis by combined CT and US imaging.

Angioimmunoblastic lymphadenopathy following ciprofloxacin administration.

Angioimmunoblastic lymphadenopathy (AILD) is a rare disorder characterised by generalised lymphadenopathy, fever, hepatosplenomegaly, immune hemolytic anemia and polyclonal hypergammaglobulinemia. We report the occurrence of histology-proven AILD in a patient who had received ciprofloxacin. We suggest that this drug may be added to the list of agents susceptible to elicit AILD.

US and CT diagnosis of complete cecocolic intussusception caused by an appendiceal mucocele.

Appendiceal mucoceles are found in only 0.2-0.3% of all appendectomy materials. Colocolic intussusception of the appendix is also very uncommon. We report the very rare association of these two entities in a 40-year-old patient presenting with intermittent right abdominal pain accompanied by a palpable mass in the right flank. The full diagnosis was made preoperatively by ultrasound and confirmed by helical CT by means of unequivocal signs of intussusception associated with a very suggestive "cup-and-ball" aspect of the mucocele induced by a global mucinous cystadenoma of the appendix. A brief review of the available literature on mucocele is given.

Imaging demonstration of fistulous gas communication between joint and ganglion of medial malleolus.

We report an unusual demonstration of a fistulous gas communication between the ankle joint and ganglion of the medial malleolus. The imaging findings support the mechanical hypothesis for the genesis of intraosseous ganglion cysts.

Trisomy 6 in Merkel cell carcinoma: a recurrent chromosomal aberration.

UNLABELLED: We retrospectively investigated 17 cases of primary and metastasizing Merkel cell carcinomas (MCC) from 14 patients using chromosomal in-situ hybridization (CISH) to study the occurrence of trisomy 6 in these lesions. METHODS AND RESULTS: Histological diagnosis on all tumour samples was obtained on haematoxylin and eosin stained sections. Immunohistochemistry was performed with antibodies against pancytokeratin (CAM 5.2), cytokeratin 20 (CK20), MIC2 antigen (CD99), neuron-specific enolase (NSE), and chromogranin A (chrA). Sections (4 microm) of the paraffin-embedded tumours were analysed with alpha-satellite centromeric probes for chromosome 6 or 17 using CISH. The signal was amplified by the Tyramide Signal Amplification (TSA) assay. Immunohistochemically, the tumours showed the same general epithelial neuro-endocrine pattern: 11/13 expressed cytokeratin 20, and 47% exhibited trisomy 6, with no significant difference between primary and metastatic lesions. Incomplete follow-up data did not allow us to establish a prognostic value of trisomy 6, however, this aberration might be an additional diagnostic tool in distinguishing MCC from other small round blue cell tumours. CONCLUSIONS: CISH seems to be a promising adjunctive method to diagnose Merkel cell carcinoma. Trisomy 6 should be investigated more closely in these cases, as has been done for chromosomes 1 and 11. Of particular interest would be identification of modifications in proto-oncogene(s) located on chromosome 6.

Hazards of laparoscopic adrenalectomy for Conn's adenoma. When enthusiasm turns to tragedy.

A 74-year-old man with primary aldosteronism had a small tumor (27 x 23 mm) of his right adrenal gland successfully removed by a transperitoneal laparoscopy. Despite absence of malignancy in the resected tumor and complete relief of all symptoms in the immediate postoperative period, recurrence occurred 6 months later. The tumor behaved as a carcinoma spread in the peritoneal cavity, and the patient eventually died with peritoneal carcinomatosis. We suggest that the laparoscopic technique coupled with pneumoperitoneum may have favored this recurrence.

Peritoneal carcinomatosis following laparoscopic resection of an adrenocortical tumor causing primary hyperaldosteronism.

A clinical syndrome combining hypertension and hypokalemic alkalosis led to the diagnosis of primary hyperaldosteronism, caused by a right-sided, 2 cm large, apparently benign aldosterone-producing adenoma. The adrenal tumor was completely resected by laparoscopic adrenalectomy. Six months after surgery, the patient exhibited a severe relapse of hyperaldosteronism. Extensive peritoneal metastases of a mixed aldosterone- and cortisol-secreting adrenocortical carcinoma were found at abdominal laparotomy. In the light of this case report, we discuss the possibility that laparoscopic resection of adrenocortical tumors might contribute to their subsequent peritoneal dissemination.

[Triton's malignant intrapulmonary tumor]. / Tumeur triton maligne intrapulmonaire.

The authors report a case of an intrapulmonary tumour which on histological analysis was found to be a malignant Triton tumour after surgical excision. The tumours proved to be of nervous tissue origin and was a rare variant of malignant tumours of the surrounding of peripheral nerves with rhabdomyoblastic heterologous differentiation which is rarely described in the lungs. The characteristics of the tumour as well as therapeutic approaches are discussed followed by a review of the literature. A particular aspect of the case presented was to realise that there was a right to left shunt and this is also discussed.

[Wegener's granulomatosis: clinical, radiological and diagnostic characteristics. Apropos of a case]. / La granulomatose de Wegener: particularités cliniques, radiologiques et diagnostiques. A propos d'un cas.

The authors report an observation of Wegener's granulomatosis with pulmonary, bronchial, renal, cutaneous and sinusal involvement. Five years ago, the patient was referred to us because of bilateral diffuse pulmonary infiltrates of unknown origin. A corticotherapy was introduced at that time and a complete clearance of the pulmonary infiltrates was noted. Nineteen months after the treatment's withdrawal, the disease recurs with the reappearance of pulmonary infiltrates. Beyond these unusual clinical aspects, histological examination of the bronchial biopsies were of diagnostic value.

[Intra-osseous and intra-articular cyst of the knee. Apropos of a case associated with osteochondritis dissecans]. / Kyste synovial intra-osseux et intra-articulaire du genou. A propos d'un cas associé à une ostéochondrite disséquante.

Subchondral bone cyst, intraarticular synovial cyst, and osteochondritis dissecans arising together are analyzed: this association has so far not been reported. If we accept the theory of a mechanical origin for subchondral bone cysts (leakage of joint fluid or intrusion of the synovial membrane through a breach in the cartilage), then the osteochondritis certainly provided the port of entry that allowed formation of the intraosseous synovial cyst in this patient.

[Primary orbital lymphoma within the context of Sjögren's syndrome secondary to rheumatoid arthritis]. / Lymphome orbitaire primaire dans un contexte de syndrome de Sjögren secondaire à une polyarthrite rhumatoïde.

We are describing the case of a seventy-four year old woman presenting a voluminous hard and painless tumor of the right lids, which has appeared half a year ago. Past medical history consists in a Sjögren's syndrome secondary to rheumatoid arthritis. The right eye is not visible and the left eye presents an important xerophthalmia and an opaque and vascularized cornea. CT Scan and biopsy allow to diagnose a diffuse monocytoid B cell non hodgkin-lymphoma, starting from the lacrymal gland with an extension to the lids and the anterior orbit. The appearance of a non hodgkin lymphoma in the course of the evolution of a rheumatoid arthritis and/or Sjögren's syndrome has been described well. The monocytoid feature of this lymphoma with the Sjögren's syndrome has recently been reported. However, the orbital localisation of this lymphoma is rare.

[Fulminant hepatic failure of neoplastic origin]. / Faillite hépatique fulminante d'origine néoplasique.

The case of a 59-year-old woman, hospitalised for a degradation of her health status which evolved within 10 days in fatal liver failure is reported. Although acute yellow atrophy was diagnosed, its origin remained elusive. Post-mortem examination of the liver disclosed massive hepatic infiltration by a breast adenocarcinoma. The causes of acute hepatic failure are reviewed and, particularly, the neoplastic ones.

Clonal rearrangement of the T-cell receptor beta-chain gene in the pleural fluid of a patient with thymoma.

Thymoma is generally considered as an epithelial malignancy surrounded by lymphocytes not belonging to the tumour. This report documents a case of malignant thymoma associated with a lymphocytic pleural effusion. The pleural lymphocytes were mature T-cells, a small proportion of which were shown to have a monoclonal rearrangement of the beta-chain of the T-cell receptor. On the contrary, the lymphocytes of peripheral blood exhibited a germline configuration. The nature of the monoclonal population found in the pleural fluid is discussed and if the cells are of thymic origin their clonal configuration may point to a neoplastic nature of lymphocytes infiltrating malignant thymoma. Alternatively, the clonal T-cell subpopulation may result from an aberrant immunological response to the thymoma.

Amylase-producing IgD-type multiple myeloma.

We describe the case of a 63-year-old woman with an IgD-type multiple myeloma and hyperamylasaemia. The evolution of the amylase concentration, the immunohistochemical data and the intracellular amylase contents of the plasma cell were consistent with secretion of amylase by the malignant clone. Moreover, cytogenetic analysis of the bone marrow revealed two structural rearrangements involving chromosome 1 near the amylase locus. Multiple myeloma should be added to the amylase-secreting tumours. This rare entity is not confined to Japan, where it was first recognized.

Antiglomerular basement membrane nephritis induced by IgA1 antibodies.

A 62-year-old man was admitted for acute renal failure due to crescentic rapidly progressive glomerulonephritis. Kidney biopsy showed intense staining for IgA, IgA1 and C3 in a linear pattern along the glomerular basement membrane (GBM). Serologic tests for detecting anti-GBM antibodies were positive for IgA class and IgA1 subclass and negative for IgG and IgA2. Serum IgA anti-GBM antibodies appeared essentially in the polymeric form, an observation in agreement with recent studies demonstrating polymeric IgA response after parenteral immunization. There was no sign of pulmonary involvement. This patient is the first to present with an isolated nephritis due to anti-GBM antibodies restricted to the IgA class (IgA1 subclass). Routine tests for circulating anti-GBM antibodies should also include the IgA class.