Lipoblastoma in adolescents and young adults: report of six cases with FISH analysis.

AIMS: Lipoblastoma is a rare benign adipocytic neoplasm that occurs primarily in infancy and early childhood. Histologically, there is some morphological overlap with atypical lipomatous tumour and myxoid liposarcoma and the age at presentation is often regarded as a major diagnostic criterion. However, we recently encountered several cases of lipoblastoma occurring in adolescents and young adults. The aim was to document the occurrence of lipoblastoma in older patients, with cytogenetic confirmation. METHODS AND RESULTS: Six cases of lipoblastoma in patients >12 years old were identified. The tumours occurred in four male and two female patients ranging from 14 to 24 years old. Our cases showed the classical histological features of lipoblastoma. Three tumours were composed predominantly of mature adipocytes and the three other cases showed an immature appearance, with a prominent myxoid matrix. Fluorescence in situ hybridization (FISH) demonstrated rearrangements of the PLAG1 region in two cases and polysomy for chromosome 8 in three other cases. None of the tumours had amplification of MDM2 or CDK4. CONCLUSIONS: Lipoblastoma occurs rarely in young adults and should enter into the differential diagnosis of 'atypical' fatty tumours in adults. Our report underscores the diagnostic value of FISH analysis.

Development of a calcifying fibrous pseudotumour within a lesion of Castleman disease, hyaline-vascular subtype.

A nine year old boy with localised Castleman disease of the hyaline-vascular subtype developed a calcifying fibrous pseudotumour. This pathological association does not appear to have been described before. In this case, the development of this very unusual soft tissue tumour-like process was thought to be related to a previous fine needle aspiration biopsy, which was performed because of lymphadenopathy localised to the right inguinal area. This case provides further evidence of the reactive nature of calcifying fibrous pseudotumour and also broadens the pathological spectrum of the stromal cell proliferation that occasionally supervenes within lesions of Castleman disease, hyaline-vascular type.

Subcutaneous panniculitis-like T-cell lymphoma: further evidence for a distinct neoplasm originating from large granular lymphocytes of T/NK phenotype.

We report the case of a 20 year-old caucasian woman who presented a primary subcutaneous panniculitis-like T-cell lymphoma (SPTCL) as an invasive tumor of the chest wall. Herein, the neoplastic cells were found to express a CD3+CD8+ phenotype but also displayed variably the natural killer (NK)-associated antigens CD56 and CD57 as well as granzyme B. On cytological examination, these cells showed a large granular lymphocyte (LGL)-like morphology with presence of azurophilic granules in their cytoplasm. Electron dense and membrane bound granules like those found in cytotoxic T lymphocytes (CTL) were also demonstrated by electron microscopy. Neither rearrangement of the T-cell receptor subunits nor Epstein-Barr virus (EBV) genome was observed at the molecular level. The LGL-like features of the neoplastic cells found in this case and the presence of NK-associated antigens provide additional support to the cytotoxic derivation of most SPTCL.

[A bizarre excrescence of the umbilicus in a 1-month-old child]. / Une excroissance bizarre de l'ombilic chez un enfant de un mois.

We report the case of an umbilical polyp, derived from omphalo-mesenteric remnants in an one-month-old female child. This rare abnormality results from a closure defect of the vitelline duct. The vitelline duct normally closes between the 5th and the 7th weeks of intra embryonic development but can lead to several pathologies in case of closure defects, giving rise to abdominal (Meckel diverticulum, vitelline cyst) or umbilical symptoms (umbilical fistula, umbilical sinus and umbilical polyp). These disorders have a 2% incidence, and may induce clinical symptoms of varied gravity ranging from clinical silence to acute abdomen. We seized the opportunity of this rare clinical observation to review the nosology of vitelline duct defects at the light of embryologic data.

[Importance of bone grafting in open tibial fractures (Gustilo III)]. / Importance de l'apport osseux dans les fractures ouvertes (Gustilo III) de jambe.

The authors report a retrospective study of 6 cases treated over the period 1988-1993.The closure of the wound was achieved by means of 3 free flaps (2 provided bone and skin in a single flap and the third was a latissimus dorsi muscle flap) and 3 pedicular flaps.The fixation of the fracture was performed by an external fixator in all cases. In one third of the cases a secondary nailing was performed to obtain consolidation. A bone graft was needed in 2/3 of the cases (2 bone free flaps, 1 bone allograft, 1 cancellous autograft).

A case of pleomorphic T-cell lymphoma with a high content of reactive histiocytes presented with hypereosinophilia.

A case of peripheral T-cell lymphoma classified, according to the updated Kiel classification, as a large pleomorphic T-cell lymphoma with a high content of reactive histiocytes and blood hypereosinophilia is reported. Light microscopic examination revealed a diffuse effacement of the lymph node structure by large pleomorphic lymphoma cells mixed with eosinophils and many histiocytes, some of them presenting discrete features of hemophagocytosis. The neoplastic cells were CD3, CD5, CD8 and HLA-DR positive but failed to show CD30 antigen. DNA molecular analysis displayed simultaneous rearrangements of the genes coding for the delta chain of the T-cell receptor and for the Ig heavy chain. Increased serum levels of angiotensin converting enzyme and ferritin were found and probably induced by the reactive histiocytes. Immunoassays (ELISA) with antibodies directed against some cytokines and against the Tac peptide (sIL-2R) were performed. They demonstrated high serum levels of sIL-2R and a slight increase in GM-CSF, but neither IL-5 nor IL-3. The association of blood hypereosinophilia and histiocytic hyperplasia with a peripheral T-cell lymphoma is discussed.

[Sebaceous cutaneous carcinoma. Value of early cytological test]. / Carcinome sébacé cutané. Intérêt de l'examen cytologique précoce.

Sebaceous carcinoma is a skin tumour which frequently metastases to the visceral organs. Needle biopsy is needed for rapid diagnosis. We observed a case in a 65-year-old patient who had a tumour formation below the right maxillary angle and homolateral justamandibular lymph node enlargement. Cytology of the needle biopsy showed a double cell population: small anaplastic cells and an agglutinated cell mass with peripheral maturation and sebaceous differenciation. Treatment was surgical with homolateral node dissection and secondary plasty. The tumour was large, non-encapsulated with local infiltration. On light microscopy, two cell populations were seen. Evident sebaceous differentiation was confirmed by histochemical staining. Immunolabelling confirmed the epithelial nature of the tumour. The clinical diagnosis of sebaceous carcinoma is difficult. Early cytology is needed to identify the two cell components when other cytological signs do not allow a positive diagnosis.

[Leiomyosarcoma of the small intestine presenting as an isolated lower digestive tract hemorrhage. Report of 2 cases]. / Léiomyosarcome du grêle révélé par une hémorragie digestive basse isolée. A propos de deux cas.

The authors report two cases of leiomyosarcoma of the small intestine revealed by lower and isolated digestive bleeding. In both cases, only the pelvic examinations by means of ultrasonography and/or CT scan have shown a mass which corresponded to the small intestine tumor. The authors underlined the necessity of US and/or CT scan pelvic when faced with a lower digestive bleeding whose etiology cannot be determined by the endoscopic or radiologic intestinal examinations.

[Synovial cyst of the hip. An unusual pathology for the orthopedic surgeon. Apropos of 2 cases]. / Le kyste synovial de hanche. Une pathologie inhabituelle de chirurgien orthopédiste. A propos de deux observations.

A synovial cyst of the hip was identified by CT scan in two cases of iliac and/or femoral vein compression. In one of these cases, incomplete resection required radical treatment of the underlying condition, osteoarthritis of the hip. The authors discuss the clinical signs, aetiopathogenesis and treatment of this disease which, although rare, has been frequently reported in the literature over the last decade.

[Treatment of bone infections with gentamicin-containing acrylic cement beads. Prognostic value of the study of the suction drainage fluid]. / Le traitement des infections osseuses par les billes de ciment acrylique à la gentamicine. Valeur pronostique de l'étude des liquides des drains de redon.

Treatment of bone infections with gentamicin-PMMA beads is an adjunct to conventional surgery. Early failures occurred in a number of cases, due to technical problems, such as incomplete debridement, residual alloplastic material, or gentamicin-resistant pathogens. To evaluate short term results of the method, clinical and bacteriological pre-therapy data were compared with results obtained two and six weeks after surgery, in 25 patients. Gentamicin concentrations, gentamicin levels/MBC for gentamicin, and bactericidal activity in secretions collected from suction drainage were not correlated with outcome. Cultures from the drains were the best parameters: cultures remained positive throughout follow-up in patient with poor clinical results.