[Kaposiform haemangioendothelioma associated with B-cell acute lymphoblastic leukemia]. / Hémangioendothéliome kaposiforme associé à une leucémie aiguë lymphoblastique B.

BACKGROUND: Herein, we report the first case of kaposiform haemangioendothelioma (KHE) associated with acute B-lymphoblastic leukemia (B-ALL). PATIENTS AND METHODS: A five-month-old infant presented a plaque of angiomatous appearance on the forearm that had increased in volume since birth, as well as pallor and cutaneous haematomas. Kasabach-Merritt syndrome (KMS) was evoked despite hepatomegaly and considerable splenomegaly. Laboratory tests revealed severe anaemia and thrombocytopenia as well as major hyperleukocytosis with 90% blasts. Skin biopsy revealed vast vascular lobules containing cohesive fusiform endothelial cells not expressing Glut1, bound up in a dense infiltrate of B-lymphoblast cells. It was in fact KHE associated with B-ALL confirmed by the myelogram. The child was treated with the INTERFANT 2006 protocol followed by allograft of haematopoietic stem cells, which resulted in complete haematological remission. At the same time, almost total regression of KHE was noted. DISCUSSION: In this infant, KHE had an inflammatory appearance and was associated with thrombocytopenia, evocative of KMS. Analysis of blood and marrow samples resulted in a diagnosis of B-ALL. Histopathological examination of the angioma revealed a typical appearance of KHE associated with dense lymphoblastic proliferation. This appearance could have resulted either from passive contamination by circulating blast cells or from active recruitment of tumor cells at the KHE site. CONCLUSION: HK mimicking KMS may reveal B-ALL.

Cleft palate associated with cervico-facial teratoma: report of two cases and review of the literature.

We report two original cases of association of cleft palate and lateral cervico-facial teratoma. We discuss the embryological explanation. The first child presented a cleft palate associated with two cervico-facial localisations of teratoma. The other had Pierre Robin sequence associated with lateropharyngeal teratoma with an extra sub maxillary localisation. Most reported cases were of midline teratomas, leading different authors to advance a mechanical origin to the cleft. Our cases are different: we could hardly find reported cases of associate lateral tumours and cleft palate, which would suggest two different embryologic mechanisms, or at least a combination of more complicated phenomenons.

[Apropos of 1 case of paratesticular papillary serous cystadenocarcinoma]. / A propos d'un cas de cystadénocarcinome papillaire séreux paratesticulaire.

The occurrence within the testis or paratesticular tissue of serous tumors, similar to ovarian tumors, is rare. This article reports a primary serous paratesticular cystadenocarcinoma in a 39 year-old man. From data of the literature, we offer guidelines for diagnosis, histogenesis and treatment of this rare tumor.

[Presacral myelolipoma]. / Myélolipome présacré.

Presacral myelolipoma is a rare benign tumour of unknown aetiology, composed of mature adipose tissue with intermixed normal haematopoietic cells. Computed tomography is of help in the diagnosis but biopsy is mandatory in order to avoid unnecessary surgery. A case is reported.

[Splenic peliosis. Apropos of 2 cases]. / La péliose splénique. A propos de deux observations.

Two cases of splenic peliosis are reported. Clinical and radiographic signs and laboratory results do not contribute greatly to diagnosis which is based on histology findings. Splenic rupture is the major complication and requires immediate splenectomy.

[Villous tumors of the Wirsung's duct and pancreatic intraductal adenocarcinoma: interrelation or accidental association?]. / Tumeurs villeuses du canal de Wirsung et adénocarcinome pancréatique intra-canalaire: filiation ou association fortuite?

Villous neoplasms of the main pancreatic duct are uncommon. Two cases of neoplasm of the main cephalic pancreatic duct in 61- and 42-year-old patients presenting with long standing (10 and 12 years) history of abdominal pain are reported. In both cases, duodenal fistula was present and mucus was observed by endoscopy at the fistula and major papilla levels. Endoscopic retrograde pancreatography showed a stricture of the main pancreatic duct in the pancreatic head. In one case, with incomplete stricture, pancreatic ducts disclosed typical features of chronic obstructive pancreatitis and contained mucus casts. Histologic examination of total and proximal duodenopancreatectomy showed a villous neoplastic pattern with focal malignant changes within the main pancreatic duct. The adjacent pancreatic tissue showed signs of stromal invasion without lymph node or nervous infiltration. Glandular parenchyma was atrophic in the pancreatic body and tail, with extensive fibrosis, and the pancreatic duct depicted signs of nonpapillary hyperplasia. Histochemical study disclosed a predominant sialomucin secretion by villous adenoma and sulfomucin secretion by epithelial cells lining the accessory or main caudal pancreatic ducts. These results lead us to suggest a possible relationship between villous adenoma of ducts and pancreatic adenocarcinoma.

Expression of the human nephron differentiation molecules in renal cell carcinomas.

The authors tested frozen sections from 28 renal cell carcinomas (RCC)--21 clear, 1 eosinophilic, 4 basophilic, and 2 spindle-shaped cell type--with monoclonal antibodies (MAb) reacting against cytokeratin, vimentin, CD24, CALLA/CD10, villin, CD26, and HLA class I and class II molecules. These molecules are markers of specific segments of the mature kidney, and their loss or acquisition reflects the different steps of human nephrogenesis. KI67 MAb was used to evaluate cell-proliferating activity. All RCC cases expressed cytokeratin. Coexpression of vimentin was observed in 21 of 28 cases. Whether of clear or chromophilic type, all tumoral cells strongly expressed CD24 molecule, present on primitive blastema cells. All clear-type RCCs expressed CALLA/CD10 and 60% were also villin positive; some were faintly positive for CD26. CALLA, villin, and CD26 were not detected in basophilic cell type. HLA class I molecules were variably expressed in almost all cases, but HLA class II were never detected on tumoral cells. Except for the spindle-shaped population, cell-proliferating activity was low. These results favor the hypothesis that RCCs derive from cells that have 'recovered' the different options of metanephric differentiation. Clear cells show evidence of maturation toward proximal type, while basophilic cells do not. It would be of interest to evaluate the usefulness of serum measurements of villin and/or CALLA as markers in clear cell-type RCC.

[Primary pulmonary sarcoma with spindle-shaped cells. Apropos of a case]. / Sarcome pulmonaire primitif à cellules fusiformes. A propos d'un cas.

Primary pulmonary sarcoma is a very rare malignant tumour. The authors report a case of a fusiform cell sarcoma simulating a pulmonary metastasis in a woman who previously had a mammary carcinoma. Lymph node invasion is rare. The nature of the tumour was confirmed by histology and aided by immunochemistry or electron microscopy. The primary origin of the tumour can only be confirmed after negative results from careful examination of the marrow, the gastro-intestinal tract, the genito-urinary system and above all the uterus. The prognosis depends on the size of the tumour and the mitotic score. Surgery is the only curative treatment.

[Bronchial cancer of several histological components: an additional argument for operating on small-cell cancers]. / Cancer bronchique à plusieurs composantes histologiques: un argument supplémentaire pour opérer les cancers à petites cellules.

Analysis of the histological data of the primary lung cancer can now be refined by means of electron microscopy. A particular sub-group has been isolated in this way: composite cell anaplastic cancer. The authors report a case of this type of cancer and present the various histological hypotheses proposed to explain this composite nature; they define the current therapeutic approach based on these data.