Le rétinoblastome

Résumé: Le rétinoblastome est la tumeur maligne intraoculaire la plus fréquente de l’enfant. Son incidence est de 1/15000 naissances. Soixante pour cent sont unilatéraux avec un age médian au diagnostic de deux ans, la plupart étant des formes non héréditaires. Le rétinoblastome est bilate ral dans 40 % des cas, l’age médian de survenue au diagnostic est alors d’un an. Toutes les formes bilatérales ou unilatérales multifocales sont héréditaires. Le rétinoblastome héréditaire constitue un syndrome de prédisposition génétique au cancer: un sujet porteur d’une mutation constitutionnelle du gène RB1 présente un risque supérieur à 90 % de développer un rétinoblastome et est par ailleurs exposé au risque de tumeur secondaire. Les deux sympt?mes les plus fréquemment rencontrés sont la leucocorie et le strabisme. L’examen du fond d’oeil permet le diagnostic, l’échographie, le scanner et l’IRM pouvant y contribuer. La prise en charge des patients doit prendre en compte divers facteurs: le potentiel visuel, la possible nature héréditaire de l’affection et le risque vital. Une énucléation est souvent nécessaire en cas de forme unilatérale, un traitement adjuvant étant indiqué en fonction des facteurs de risque histologiques. Un traitement conservateur pour au moins un oeil est possible dans la plupart des formes bilatérales: thermochimiothérapie, cryothérapie, laser (thermothérapie), curiethérapie par disque d’iode. On tente de limiter les indications de radiothérapie externe aux grosses tumeurs avec essaimage vitréen, en raison des effets tardifs dont les sarcomes secondaires. Un suivi à long terme et une information précoce des patients et de leur famille concernant les risques de transmission et de tumeurs secondaires sont nécessaires. Oncopédiatrie     

Malignant triton tumor of the sinonasal tract

INTRODUCTION: The combination of a malignant peripheral nerve sheath tumor with rhabdomyosarcomatous elements has been termed Malignant Triton Tumor (MTT). This tumor was first reported in 1932 and the prognosis was poor. Involvement of the sinonasal tract is rare with only 10 case reports. CASE: We report the case of a 80-year-old woman who presented an obstruction of the right nasal fossa which developed over several years without anosmia. She consulted because of recurrent epistaxis. The CT-scan revealed a tumor occupying the right ethmoid the right maxillary sinus and the homolateral nasal fossa. Resection was achieved via an extended mediofacial route. The histological diagnosis was low-grade schwannosarcoma with a discretely extended rhabdomyosarcomatous component, i.e. MTT. No complementary treatment was given. Eight months later, the tumor relapsed with cerebral involvement. Despite new surgery with two teams, a neurosurgical and a radiotherapy team, the tumor relapsed at the ethmoid-nasal level. After five years, the patient is alive with local progression without metastasis. DISCUSSION: MTT of the sinonasal tract is a rare disease that clinicians should be aware of and that must be included in the differential diagnosis of malignant lesions involving the sinonasal tract. Most of the reported cases involving localizations other than the ethmoidonasal cavities, are associated with von Recklinhausen neurofibromatosis and occurr in young subjects (mean age 35 years). There have however been sporadic cases, mainly in older women or after radiotherapy. The prognosis is somewhat better for ethmoidonasal localizations.     

Pindborg tumor: a poorly differentiated form without calcification

BACKGROUND: Pindborg tumor is a rare benign epithelial calcified odontogenic tumor. Radiological diagnosis is generally suspected because of the presence of calcifications. CASE REPORT: A 61-year-old man presented a polymorphous Pindborg tumor of the anterior maxillary. The diagnosis was hindered due to the nonspecific radiographic image and the lack of calcification. Pathology provided the positive diagnosis of poorly-differentiated young odontogenic epithelial tumor. DISCUSSION: Pindborg tumor is a rare lesions usually found in the posterior mandibular bone. Calcification is a characteristic feature. There are two historical forms, a squamous form with very favorable outcome and a clear-cell form with less favorable prognosis.     

Unilocular macrocystic serous cystadenoma of the pancreas: a morphologic variant to be considered

Serous cystadenoma of the pancreas is a benign cystic tumor, which radiological diagnosis is easy in its typical microcystic variant. The macrocystic variant is uncommon and raises diagnostic problems with other macrocystic lesions of the pancreas such as pseudocysts and mucinous cystadenomas. We report the case of a young woman with a unilocular macrocystic serous cystadenoma of the pancreas which was identified on pathologic examination of the surgical specimen, after unconclusive abdominal ultrasound and CT-scan. This case describes an unusual clinical presentation of this cystic tumor and emphasizes that the diagnosis of such an entity is still based on pathological examination after cyst removal.     

Solid-pseudopapillary tumor of the pancreas: a clinical study of five cases, and review of the literature

INTRODUCTION: Solid pseudopapillary tumors of the pancreas are rare and their origin is unknown. The aim of this work was to report five new cases. MATERIAL AND METHODS: Retrospective study of data from patients operated on from 1983 to 2002 in a university hospital specialized in pancreatic surgery. Patients were identified in a prospectively constituted database of pathologic examinations. RESULTS: Five patients (three men and two women, aged from 15 to 69 years) underwent pancreatectomy for a solid pseudopapillary tumor, which was discovered fortuitously by imaging in three cases. Tumor diameter ranged from 4 to 15 cm. Diagnosis was made preoperatively in only one patient. There were three pancreaticoduodenectomies and two left pancreatectomies, with extension to the transverse colon due to vascular reasons in two cases. Only one significant complication occurred (one colonic fistula). With a follow-up ranging from 6 months to 6 years, all patients are alive without recurrence. CONCLUSIONS: Solid pseudopapillary tumors are not exceptional in men. Complete resection can need extension to neighboring organs but allows good long-term survival.     

Laparoscopic pancreatic resections

The feasibility of laparoscopic pancreatic resection has been demonstrated. However, the real clinical benefit for the patients remains questioned. The best indication for a laparoscopic approach appears to be the resection of benign or neuro-endocrine tumors without a need for pancreato-enteric reconstruction (i.e enucleation or distal pancreatectomy). The use of the laparoscopic approach for malignant tumors still remains controversial. The benefits of minimally invasive surgery are clearly correlated with the successful management of the pancreatic stump. Pancreatic related complication rate (fistula and collection) is 15% when using pancreatic transection with a laparoscopic endostappler.     

Superficial pT1G3 bladder tumor: 24 case reports

The authors report 24 patients presenting a transitional tumor of bladder pT1G3 collected between January 1996 and December 2000. They represent 19% of the superficial tumors of bladder. Two patients had of straightaway a cystectomy after resection for an unverifiable tumor by endoscopy and another after a second resection discovering a real pT2. Only 5 patients received a BCG therapy is 24% of the cases. A recurrence without progression has been noted in 38% of the cases and a progression in 19% of the patients. These last patients had a cystectomy and with a follow-up to 28 months, no recurrence has been noted.     

Pseudosarcomatous fibromyxoid tumor of the prostate: report of a case

The authors report a case of pseudosarcomatous fibromyxoid tumor of the prostate. It is always a benign lesion, whose mains differentials diagnoses are sarcomas of the prostate. Diagnosis is based on histological examination. Treatment is surgical.