[Rosai-Dorfman disease: Case report].

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare dis order of unknown etiology, generally associated with enlargement of superficial and/or deep lymph nodes. Most patients do not require treatment. We report the case of a 10 month old infant who was admitted with a left cervical tumor of 25 days duration.

Symptomatic bone langerhans cell histiocytosis treated at diagnosis or after reactivation with indomethacin alone.

This study evaluated the outcome of patients with symptomatic bone Langerhans cell histiocytosis (LCH) treated with indomethacin alone, either at diagnosis or after reactivation (after recurrence with previous therapies). We evaluated the nonrandomized use of oral indomethacin (2 mg/kg/d) in patients with symptomatic single-system bone LCH. From 1997 to 2012, 38 sequential patients were treated for a median of 4 months.

Juvenile xanthogranuloma with bilateral ocular involvement: complete response after treatment with vinblastine: case report.

Purpose: To present an infant with bilateral ocular and skin involvement by juvenile xanthogranuloma and the results of treatment with vinblastine.

Methods: Analysis of the clinical and histopathologic data and the results of the treatment.

Results: After 14 months' evolution with no response to other treatments, the lesions responded to a course of vinblastine (6 mg/m2/weekly, corrected for weight), which was followed by a maintenance treatment for 52 weeks.

Neuroblastoma in a patient with Coffin-Siris syndrome.

We report the case of an 8-year-old boy with the phenotypic features of Coffin-Siris syndrome diffuse hypertrichosis, flat occiput, scant scalp hair, flat supraorbital arch, triangular eyebrows, horizontal palpebral fissure, anteverted nares, triangular philtrum, coarse lips, high-arched palate, micrognathia, low set and dorsaly rotated ears, short neck, wide thorax, widely set nipples, transverse palmar crease, psychomotor delay, urinary malformations (paraurethral diverticulum, hypoplasia of left kidney associated with vesicoureteral reflux grade 3-4), bilateral inguinal hernia, and dorsolumbar kyphoscoliosis. In the follow-up he presented a retroperitoneal neuroblastoma. Although this type of tumor has been referred to develop in several genetic and mutimalformative syndromes, it seems that present association has not been previously reported.

Cardiac liposarcoma and bilateral ovarian mature teratoma in a patient with congenital heart disease and f-anisosplenia.

A 10-year-old female with atrial septal defect presented with bilateral ovarian mature cystic teratomas. Three spleniculi adjacent to the main spleen were noticed while the endoscopic surgery. Two years later a heart tumor was discovered which led to death.

Reactivation and risk of sequelae in Langerhans cell histiocytosis.

Objective: To evaluate disease reactivation in patients with Langerhans cell histiocytosis (LCH) and its impact on adverse sequelae.

Materials And Methods: A retrospective evaluation of 300 patients diagnosed with LCH between 1987 and 2002 with complete response to initial treatment was performed.

Results: Mean age at diagnosis was 5.

Fine-needle aspiration biopsy in pediatric oncology patients: a review of experience with 829 patients (899 biopsies).

The authors review their 20-year (1985-2004) experience with fine-needle aspiration biopsy (FNAB) in 829 children (all but 1 younger than 18 years), from whom 899 samples were obtained. All patients presented with clinical data suggesting malignancy. The procedure followed standard methods (22- to 24-gauge needle; mean of six alcohol-fixed and/or air-dried, H&E- or Giemsa-like-stained smears/procedure; leftover clots processed as a biopsy).

Spinal cord compression: a review of 70 pediatric patients.

The authors report their experience with 70 pediatric patients with spinal cord compression (SCC) due to malignancies identified among 898 patients with solid tumors. An extradural tumor was the most frequent cause of SCC (71%); 54% of these were soft tissue sarcomas and neuroblastoma. Most intradural tumors (70%) were outside the spinal cord, 9/12 being metastatic medulloblastomas.

Fetal rhabdomyomatous nephroblastoma: report of 14 cases confirming chemotherapy resistance.

The authors retrospectively reviewed their experience with 14 cases of fetal rhabdomyomatous nephroblastoma (15.6% of all renal tumors seen in the 1984-1998 period), analyzing the diagnostic and treatment approach as well outcome. Mean age at presentation was 27 months; 10 (71%) patients were younger than 36 months (5 of these were less than 12 months) at diagnosis.

Bilateral nephroblastoma in familial Hay-Wells syndrome associated with familial reticulate pigmentation of the skin.

We report on a girl with maxillary hypoplasia, prominent ears, dry sparse hair, palmar and plantar keratoderma, dystrophic nails, patchy pigmented skin lesions in hands and feet and bilateral Wilms tumor. She was born with bilateral ankyloblepharon. The mother and maternal grandmother presented similar ectodermal defects.

Fetal rhabdomyomatous nephroblastoma: diagnosis by fine-needle aspiration cytology--a case report.

The present report describes the case of a 21-mo-old boy with a fetal rhabdomyomatous nephroblastoma (FRN) diagnosed by fine-needle aspiration cytology (FNAC). The smears contained numerous isolated or clustered, deeply eosinophilic, thin, elongated rhabdomyoblasts with oval nuclei. Some of these cells presented cross-striations.

Retroperitoneal and deep-seated lipoblastoma: diagnosis by CT scan and fine-needle aspiration biopsy.

Lipoblastomas are most commonly superficially-located tumors. Our experience with three examples of deep-seated lipoblastomas is reported. The CT scans of all cases showed low-attenuation densities (less than 0 Hansfield units), strongly suggesting fatty composition.

Pleuropulmonary blastoma: diagnosis by fine-needle aspiration cytology: a case report.

Pleuropulmonary blastoma (PPB) is an unusual pleural-based blastoma presenting in childhood composed by undifferentiated sarcomatous tissue with divergent differentiation and occasional benign epithelial-lined structures. We are presenting the cytologic features of PPB as diagnosed by fine-needle aspiration cytology (FNAC) of the lower lobe of the right lung in a 4-year-old girl. The smears showed highly malignant cells with hyperchromatic oval or multilobulated pleomorphic nuclei strongly suggesting an aggressive sarcoma.

Undifferentiated (embryonal) sarcoma of the liver: fine-needle aspiration cytology and preoperative chemotherapy as an approach to diagnosis and initial treatment. A case report.

The present report describes the case of a 9-year-old girl with an undifferentiated (embryonal) sarcoma of the liver diagnosed by fine-needle aspiration cytology (FNAC). The smears revealed pleomorphic cells, some with cytoplasmic vacuoles and eosinophilic inclusions, as well as spindle cells and myxoid tissue, cytologic features which appear to be distinctive from any other malignant tumor of the liver. Preoperative chemotherapy was then given using a protocol for high-grade soft-tissue sarcomas (MMT95 953 branch B, SIOP).

Rhabdomyosarcoma of extrahepatic biliary tree: initial treatment with chemotherapy and conservative surgery.

Background: The very low frequency of rhabdomyosarcoma (RMS) of the extrahepatic biliary tree has impeded the development of a standardized form of treatment.

Procedure: Based on the good response of embryonal RMS to chemotherapy, we used a multi-drug protocol as the initial treatment of a 3-year-old girl after obtaining adequate transparietohepatic biliary drainage. The treatment achieved complete remission.

Cytologic characteristics of peripheral neuroectodermal tumors in fine-needle aspiration smears: a retrospective study of three pediatric cases.

Cytologic diagnosis of peripheral neuroectodermal tumors (PNT) on fine-needle aspiration (FNA) smears represents a challenge to the cytopathologist. Usually ancillary studies are used to achieve definitive diagnosis. We retrospectively examined FNA material from three cases of PNT with the aim of identifying their features.

Malignancy-associated membranoproliferative glomerulonephritis.

An 11-year-old girl with an abdominal desmoplastic round cell tumor, treated with chemotherapy, presented with gross hematuria and proteinuria. Renal biopsy revealed type I membranoproliferative glomerulonephritis (MPGN). The association of a malignant tumor and MPGN is extremely unusual in children, and the pathogenesis of the renal lesion under these circumstances is unknown.

Chondrosarcoma of bone as second tumor after diffuse anaplastic Wilms' tumor: diagnosis by fine-needle aspiration cytology.

A 6-yr-old boy already treated (surgery and chemotherapy) for a diffuse anaplastic Wilms' tumor discovered at the age of 10 mo, presented with a large tumor in the left femur. This proved to be a chondrosarcoma after fine-needle aspiration cytology, a diagnosis later confirmed by histology of the tumor. The patient was treated with marginal excision and prosthetic replacement but died 1 yr after this operation with lung metastasis.

Incidence of childhood cancer in La Plata, Argentina, 1977-1987.

Incidence data on malignant tumors in children aged 0-14 for the period 1977-87 were calculated in La Plata, Argentina. The number of children living in La Plata was 151,085. The overall incidence of malignant tumors was 106.

Anaplastic Wilms' tumor. Initial diagnosis by fine needle aspiration.

The diagnosis of a diffusely anaplastic Wilms' tumor in a ten-month-old child was first suggested by cytologic and histologic study of a fine needle aspirate. The mesenchymal component was predominant (75%) and exhibited some cells with hyaline, periodic acid-Schiff-positive, intracytoplasmic droplets. Foci of clear-cell carcinoma were also present.