Nodular myofibroblastic mesenchymal dysplasia of the placenta in association with an unusual bone dysplasia.

This case report describes the recognition of nodular and asymmetric perivascular clusters of myofibrolasts-leiomuscular cells in the stroma of immature chorionic villi associated with a fetus delivered at 26 weeks gestation with a peculiar variation of bone dysplasia syndrome and oligohydramnios. Although myofibroblasts are known to exist at these sites, the finding may represent an unusual mesenchymal dysplasia of the placenta perhaps related to the syndrome.

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.

Papillary carcinoma of the thyroid developed in congenital dyshormonogenetic hypothyroidism without goiter: Diagnosis by FNAB.

A 5-year-old girl known to have congenital dyshormonogenetic hypothyroidism since birth and under appropriate treatment (without goiter) developed a thyroid nodule which was found in the clinical follow-up. Fine-needle aspiration biopsy of the nodule allowed to diagnose a papillary thyroid carcinoma, a diagnosis which was certified by the surgical specimen exam. Dyshormonogenetic goiter may rarely associate with thyroid carcinoma.

Koilocytes in urinary cytology in a patient with kidney transplant.

We are reporting the case of a 17-year-old girl with kidney transplant under immunosuppressive treatment. Evidences of transplant malfunction led to urinary cytology to rule out BKV infection. The smears showed the presence of koilocytes.

Acinic cell carcinoma of salivary gland with massive deposits of globular amyloid.

We present the case of a 23-year-old woman with a parotid gland tumor, the fine-needle aspiration biopsy smears of which showed epithelial cells with wide cytoplasm, isolated or arranged in micropapillary groups together with psammoma bodies. The surgical specimen contained a 5-cm tumor with the histologic features of an acinic cell carcinoma (ACC) with papillary areas. Notably, the cells of the tumor seemed to follow a sequence from large cells with rounded nuclei with open chromatin and prominent nucleoli to vacuolated cells with granular material, and finally to cells undergoing apoptosis.

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.

Omeprazole-associated changes in the gastric mucosa of children.

Aims: To describe histological findings in gastric mucosa biopsy specimens of children treated with proton-pump inhibitors (PPIs) for different periods of time.

Methods: Biopsy specimens from 12 children (aged 8 months to 15 years) treated with PPIs and 8 controls were processed for paraffin embedding and stained with H&E as well as inmmunohistochemically for sialyl-Tn antigen.

Results: The main changes were related to parietal cells which showed brisk cytoplasmic eosinophilia, apical cytoplasmic protrusions to dilated glands, cytoplasmic and nuclear hypertrophy, dilated intracytoplasmic canaliculi, binucleation and multinucleation.

Histopathologic diagnosis of celiac disease in children without clinical evidence of malabsorption.

Diverse clinical and pathologic experiences seem to have led to the idea that celiac disease is a spectrum in both categories. Conflicting results emerging from different reports have produced a large amount of confusion on the subject. This article discussed histopathology findings in 10 children with positive autoantibodies for celiac disease but without clinical evidence of malabsorption.

Small bowel gastric metaplasia: a report of three cases in children.

Aims: To report three children displaying gastric metaplasia antral pyloric type of the small bowel mucosa.

Methods: Analysis of clinical, histopathological and immunohistochemical data.

Results: The first patient was a 14-year-old girl with history of chronic intestinal pseudo-obstruction and chronic jejunitis; the second patient was a 6-year-old girl with a long-lasting jejunostomy; and the third patient was a 5-year-old girl with ileal-rectal anastomosis.

Colonic aberrant crypts in children with familial adenomatous polyposis.

Aberrant crypt foci of the colonic mucosa have been reported in adults. The alteration may be defined macroscopically or histologically and may or may not be combined with adenomatous changes. Aberrant crypt foci have been regarded as precancerous lesions and are more common in patients with familial adenomatous polyposis.

Extrahepatic biliary atresia with cartilaginous metaplasia in the gallbladder remnants.

Extrahepatic biliary atresia (EBA) is a chronic inflammatory process leading to fibrous obliteration of the biliary tree. Cartilaginous metaplasia within the remaining walls of the biliary main ducts in EBA has been reported once, although as cartilage heterotopia. A similar finding is presented here, but in the gallbladder of an infant with the full-blown manifestations of EBA; it is proposed that the alteration results from metaplasia of local connective tissue.

Pluripotential melanoblastoma, a unifying concept on malignancies arising in congenital melanocytic nevi: report of two cases.

Congenital melanocytic nevi are benign lesions present at birth and considered to be caused by a maldevelopment of the neural crest. The malignant potential of the congenital melanocytic nevi have been extensively addressed by several authors, and malignant melanoma is the most frequent neoplasm arising in these lesions. The present report describes two patients with congenital melanocytic nevi in which malignant melanoma with undifferentiated areas showing rhabdomyoblastic differentiation developed.