[Recurrent and metastatic infantile fibrosarcoma: a case report]. / Fibrosarcome infantile récidivant et métastatique: à propos d'un cas.

Infantile fibrosarcoma is a rare malignant tumor that usually occurs during the 1st year of life. It accounts for approximately 5-10% of all sarcomas in infants younger than 1 year of age. It usually has indolent progression and metastatic spread is rare. We report the case of a patient who had infantile fibrosarcoma of the trunk. At birth, the baby presented a soft tissue mass of the scapulothoracic region. Histopathological examination after complete surgical resection at first suggested an angioma. Reanalysis of the histology after a metastatic relapse resulted in the diagnosis of infantile fibrosarcoma, which was confirmed by the presence of the specific translocation seen in infantile fibrosarcoma (ETV6/NTRK3). This patient's progression was uncommon because he developed 3 metastatic relapses. The treatment consisted of surgery, chemotherapy, and radiation therapy. The patient is alive with persistent complete remission. We discuss the diagnostic and therapeutic issues of infantile fibrosarcoma. There is a risk of erroneous diagnosis in newborn infants between benign angiomatous tumor and infantile fibrosarcoma. The fusion transcript ETV6-NTRK3 resulting from the specific chromosomal translocation t(12;15)(p13;q25) is now a useful diagnostic tool for infantile fibrosarcoma. Surgery with wide resection is the mainstay of treatment. However, infantile fibrosarcoma is a chemosensitive tumor. If initial surgery cannot be done without mutilation or is impossible, preoperative chemotherapy should be given. The role of radiation therapy is still debated.

[Use of interferon in a case of hepatic hemangioma]. / Utilisation de l'interféron dans un cas d'hémangiome hépatique.

UNLABELLED: Hepatic hemangioendothelioma can have a fatal outcome. After failure of classic therapies, and when surgery or arterial embolization are not possible, those benign tumours may benefit from interferon alpha treatment. CASE REPORT: We report a case of a 14-month-old infant who presented with hepatic hemangiomatosis and cardiac failure. The disease can neither be controlled by steroids nor by radiotherapy associated with digitalo-diuretic treatment. Due to the important vascular volume of the tumour, neither surgical care nor arterial embolization were possible. Thus this infant was treated for ten months by interferon alpha. The evolution was favourable: cardiac failure, calcifications and volume of the angiomatosis were regressive. CONCLUSION: Interferon alpha treatment helps to control cardiac failure and the course of hepatic hemangioma in childhood.

[Agenesis of the right lobe of the liver. Apropos of a case]. / Agénésie du lobe hépatique droit. A propos d'une observation.

The authors describe a recent case of agenesis of the right lobe of the liver. Such cases are rare and can be diagnosed with state of the art imaging modalities, ultrasound and computed tomography, which permit early diagnosis and help avoid liver complications of cirrhosis, cholangiocarcinoma and hepatoma. This type of congenital anomaly can be associated with biliary tract disease, portal hypertension or other congenital anomalies.

[Digestive cystic duplications: contribution of ultrasonography in the neonatal period. Apropos of 3 cases]. / Duplications kystiques digestives: apport de l'échographie en période néonatale. A propos de 3 cas.

Three cases of enteric duplication cysts in newborns are reported. The importance of the ultrasonographic exploration for the diagnosis is outlined.

[Tumoral calcinosis in a white woman. Apropos of a case]. / Calcinose tumorale chez une femme blanche. A propos d'une observation.

A new of tumoral calcinosis is described in a 91 years old woman. It is a rare condition that affect almost always black subjects. This benign disease is characterised by progressive increase of subcutaneous calcified masses near larger joints. Only four cases were yet reported in a white female.

[Renal staghorn calculi in adults. A study of eighty patients with ninety two calculi (author's transl)]. / La lithiase coralliforme du rein de l'adulte. A propos de quatre-vingt-douze calculs chez quatre-vingts malades.

A homogeneous sery dealing with 80 cases of staghorn calculi in adult patients is examined from clinical and therapeutic points of view. Among 75 operated patients, 8 bilaterally, 67 conservative operations were performed. Enlarged intra-sinusal pyelotomy gives the best short and long-term results and is the surgical procedure of choice.

[Bilateral nephrocarcinomas in the adult. Bibliographical and statistical analysis. On the basis of 2 personal cases and 106 cases in the literature (author's transl)]. / Les néphroépithéliomes bilatéraux de l'adulte. Approche bibliographique et statistique. A propos de 2 cas personnels et 106 cas de la littérature.

On the basis of two personal cases, the authors undertook an overall study of 108 cases published between 1909 and 1978. First personal case: 2 successive tumours at an interval of 5 years. Total nephrectomy of the 2nd carcinoma resulted in survival for 6 years with hemodialysis. The patient is living, with metastases. Second case: 2 simultaneous carcinomas with adrenal metastases with death following unilateral partial nephrectomy. An exhaustive review of the literature revealed the following interesting statistical factors: 1.8 p. 100 of carcinomas of the kidney are bilateral. Amongst the 108 cases studied, 51 were simultaneous and 57 successive. Treatment becomes appropriately more and more audacious: total nephrectomy on one side and partial on another, double partial nephrectomy, performance "ex vivo" of partial excisions, bilateral total nephrectomy followed by hemodialysis or transplantation. These tumours affect young individuals: mean age 51 years and male in 7 cases out of 10. Mean survival of all cases combined was 18 months, but subtracting operative mortality this increased to 22 months. Mean survival was greater in successive carcinomas, in individuals aged over 55 years (26 months) than in simultaneous carcinomas of the younger age group (13 months). Overall prognosis has improved for simultaneous carcinomas (mean survival 2.5 months for the period 1909--1954 increasing to 25 months for the period 1972--1978); whilst it has remained stable (mean survival 21 months) for successive carcinomas. The mean interval between 2 successive carcinomas was 6 years and 6 months with a range of 5 months for 20 years. Prognosis was based on numerous factors, but in particular treatment. When surgery was incomplete or absent, mean survival was 5 1/2 months (40 cases), the figure reaching 26 months for the 52 cases in whom an adequate excision was performed (of the order of 30 months for the period 1972--1978). No published case should be reported before a minimum survival of 2 years.

[Isotopic kidney investigations in staghorn lithiasis in the adult (author's transl)]. / Les explorations rènales isotopiques dans la lithiase coralliforme de l'adulte.

The authors report a study of 37 kidneys in 30 patients with staghorn calculi undergoing isotopic investigation of renal function by sequential scintigraphy and isotopic nephrogram by gamma-camera. 7 of the patients had bilateral staghorn calculi. Tracers used: Hippuran labelled with I131 and DPTA labelled with Technetium 99 m. 62% of kidneys poorly tolerated their staghorn calculus as shown by severe impairment of the isotopic nephrogram involving the uptake phase. Amongst the 20 cases of unilateral lithiasis, a nephrectomy was performed whenever the functional value was less than 20% normal (7 kidneys). Twelve stones were removed with conservation of the kidney. It should be noted that with renal function greater than 70% normal, the thickness of the renal parenchyma as assessed by IVU is never less by more than 1/2 cm that of the contralateral kidney. In the group, 11 patients underwent postoperative urography and scintigraphy with a mean follow up of 4 years. All extractions of calculi by pyelotomy with or without a small nephrotomy benefited initially impaired kidneys. By contrast, in 5 kidneys where a large nephrotomy was made, there was a gain in function in only one. 1 remained stationary and the others lost respectively 20, 30 and 100% of their function. Correlation was good with other methods of split renal function studies (IVU and renal arteriography). Of essential importance is the fact that the irradiation associated with these isotopic investigations is 20 to 50 times less than that of IVU. Isotopic investigation is thus a good method for the assessment of changes in renal function after surgery for staghorn calculus.

[Arteriography of renal metastases of bronchial origin : use of angiotensin in one case (author's transl)]. / Artériographie des métastases rénales d'origine bronchique. Intérêt de l'angiotensine. A propos d'un cas.

A case of renal metastases from epidermoid bronchial cancer, discovered during an attack of hematuria, is reported. The patient had been operated upon for bronchial cancer two years ago and was in good general condition. Au I.V.U. showed the presence of an ill-defined renal mass. Selective renal arteriography demonstrated that the mass was hypervascularized and that a previous injection of angiotensin produced a malignant type of hypervascularization in the mass.

[A rare case of double biliary fistula (author's transl)]. / Un cas rare de double fistule bilaire.

The authors report a rare case of a double cholecysto-duodenal and cholecysto-colic fistula.