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PubMed1992

Adult extrarenal rhabdoid tumor of the lacrimal gland.

A 50-year-old man presented with a rapidly growing mass in the area of the right lacrimal gland. An initial erroneous histopathologic diagnosis of a pleomorphic adenoma made on a small-incisional biopsy was later corrected to a malignant rhabdoid tumor when a wide local excision of the tumor was performed. The tumor was composed predominantly of dyscohesive, globoid, and eosinophilic cells, which frequently contained cytoplasmic inclusions. These were demonstrated to be composed of whorls of intermediate vimentin filaments. The tumor cells expressed epithelial membrane antigen as well as cytokeratin. Ultrastructurally, they displayed intercellular junctions and interrupted segments of linear basement membrane material. These findings, together with the development of the lesion within the parenchyma of the lacrimal gland, are suggestive of an epithelial origin. The patient was treated with radical surgery and adjunctive radiotherapy and chemotherapy, which are the recommended treatment modalities for this highly malignant tumor.

Antigens, Neoplasm↗
PubMed1998

A tumorous type of pulmonary rhabdomyomatous dysplasia.

We report an extraordinary case of tumorous type of rhabdomyomatous dysplasia without any other malformation in a fetus of 23 weeks gestation. Histologically, autopsy showed distinctive striated muscle tissue in the upper and middle lobes of the right lung. Unusual in this case, compared with previously published reports, is the absence of further malformations, the occurrence in the apical parts of the lung and the marked degree of rhabdomyomatous dysplasia. The case report is followed by a differential diagnosis, review of the literature and discussion of the pathogenesis.

Adult↗
PubMed1996

[Aniridia and Wilms tumor: 2 cases of fetal rhabdomyomatous nephroblastoma].

BACKGROUND: Wilms tumor is associated in 7 to 10% of patients with congenital abnormalities. Among those, aniridia is the most constant feature of the WAGR syndrome that includes, in one third of cases. Wilms tumor. We report two cases of aniridia associated with fetal rhabdomyomatous nephroblastoma. CASE REPORTS: Case 1. A one-year old girl with congenital aniridia was admitted for macroscopic hematuria. Abnormal ultrasonography and tomodensitometry revealed a large, bilateral, kidney tumor. The patient was given actinomycin and vincristine, without efficacy. Bilateral tumorectomy was performed 6 months later and the histological study showed a fetal rhabdomyomatous nephroblastoma. This patient is in remission at the age of 5. Case 2. A boy, also with congenital aniridia, presented with macroscopic hematuria at the age of 2 years revealing a nephroblastoma located on his right kidney. Preoperative chemotherapy remained uneffective and the nephrectomy performed 1 month later permitted the diagnosis of fetal rhabdomyomatous nephroblastoma. The patient is well 4 years later. CONCLUSION: Both cases of fetal rhabdomyomatous nephroblastoma, a histological variant of Wilms tumor, seem to be the first reported in the WAGR syndrome.

Aniridia↗
PubMed2005

Rhabdomyomatous mesenchymal hamartoma: a plaque-type variant in an adult.

We present the case of a 40-year-old woman who had a flesh-colored asymptomatic plaque-like lesion above her chin since she was 30 years old. She was generally healthy and physical examination revealed no congenital abnormalities. Histopathology revealed a normal epidermal surface, mature striated muscle fibers arranged randomly within the dermis, and subcutaneous tissue associated with normal-appearing mesenchymal elements such as adipose tissue, collagen, blood vessels, and mature hair follicles. This fits the diagnosis of rhabdomyomatous mesenchymal hamartoma. Our case was different from previously reported cases with regards to the age of onset and clinical presentation. We report this rare adult case and discuss its differential diagnosis.

Adult↗
PubMed1976

Pleomorphic rhabdomyosarcoma of the larynx. A case report and review of the literature.

A case of pleomorphic rhabdomyosarcoma of the larynx occurring in a 33-year-old male is reported. The clinical and histopathological aspects of this undoubtedly rare neoplasm are described. After a quick review of the most important classifications of rhabdomyosarcoma so far made, the authors suggest a division into two basic groups, i.e. pleomorphic and fetal rhabdomyosarcoma, while the botryoid sarcoma and the alveolar rhabdomyosarcoma should be considered only as two subgroups of fetal rhabdomyosarcoma. From a comprehensive review of world literature on the subject, the well-documented cases up to now described are only seven, including the authors. The present case is the third one after the instances of pleomorphic rhabdomyosarcoma reported by Filipo and Crifo (1964 and by Rodriquez and Ziskind (1970).

Adult↗
PubMed1998

Maximal preservation of renal function in patients with bilateral Wilms' tumor: therapeutic strategy of late kidney-sparing surgery and replacement of radiotherapy by high-dose melphalan and stem cell rescue.

In children with bilateral Wilms' tumor, the therapy should aim at maximal preservation of renal parenchyma and function. Local radiotherapy may give rise to second malignant neoplasms and may impair renal function. We present a therapeutic strategy without any irradiation. Three children were diagnosed with bilateral Wilms' tumor at ages from 6 months to 5 years. Each patient had a massive tumor with local stage III on one side; one had pulmonary metastases. The therapeutic strategy was first to obtain tissue for histology by percutaneous needle biopsy, to administer pre-operative chemotherapy until desired tumor shrinkage, and then to perform kidney-sparing resective surgery. After a period of conventional chemotherapy, the patients were consolidated with high-dose (HD) melphalan and ABMT. Renal parenchyma spared post-surgery (right/left) was 0%/70%, 60%/40% and 40%/60% of the original kidney volumes. The toxicity of the ABMT procedure was mild, the patients engrafted promptly, and were discharged on days +14 to +27. All patients survive disease-free, 3 years 4 months to 4 years 5 months post-transplant. Our program resulted in good preservation of renal parenchyma and normal function, and we consider the risk of this ABMT program smaller than the late consequences of local radiotherapy for children with bilateral Wilms' tumor. The therapeutic strategy described merits further evaluation.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1997

Rhabdomyomatosis of the newborn lung unassociated with other malformations.

AIMS: To describe a case of rhabdomyomatosis of the lung unassociated with other external or visceral malformations in a newborn infant. METHODS AND RESULTS: A 26 weeks gestation newborn male with no relevant medical or family history presented a well-circumscribed solid area in the posterior mediastinum occupying the upper lobe of the right lung. The possibility of neuroblastoma or an extralobar pulmonary sequestration were excluded after laboratory and arteriographic studies. No visceral anomalies were found. At the age of 9 months the patient underwent a partial lobectomy, and he is free of disease 39 months after surgery. Histological examination demonstrated the presence of numerous bundles of striated fibres arranged haphazardly in the pulmonary interstitium in a background of a type-II congenital cystic adenomatoid malformation-like morphology of the resected lung. CONCLUSION: The presence of striated muscle fibres in the lung not necessarily represents a lethal congenital malformation. As this case shows, rhabdomyomatosis of the lung can affect a single pulmonary lobe, and resection of the affected lung parenchyma may be curative. It is important for pathologists to be aware of this entity, although it is exceptional, and to include it in the differential diagnosis of pulmonary masses in the newborn lung.

Humans↗
PubMed2001

Implantable cardioverter defibrillator in a 4-month-old infant with cardiac arrest associated with a vascular heart tumor.

A previously healthy male infant was resuscitated after spontaneous ventricular fibrillation at 9 weeks of age. Echocardiography revealed three tumors in the left ventricle not amenable to complete resection. Despite treatment with antiarrhythmic agents the ventricular arrhythmias continued. When the child was 4 months old and weighed 7 kg an ICD system was implanted using epicardial sense-pacing leads and a superior vena caval lead as a subcutaneous defibrillator coil placed posterior on the left thorax. Shocks were delivered between the subcutaneous coil lead and the intraabdominally placed ICD can. This ICD array system has not been reported previously.

Defibrillators, Implantable↗