Granular cell tumors of the head and neck in children: the experience at the Children's Hospital of Philadelphia.

Granular cell tumors are rare, usually benign tumors of controversial histogenesis. These tumors are uncommon in children. Granular cell tumors in adults have a predilection for the head and neck, but this was not noted in our experience with children. Of the 22 granular cell tumors diagnosed and managed at the Children's Hospital of Philadelphia from 1960 to 1988, 7 were from the head and neck. One child with a subglottic granular cell tumor is of particular interest and is presented in detail.

Beckwith-Wiedemann syndrome with unusual hepatic and pancreatic features: a case expanding the phenotype.

We describe an infant with Beckwith-Wiedemann syndrome (BWS) who had hepatic and pancreatic findings not previously described in BWS. These were biliary dysgenesis and enlargement and cystic dysplasia of the pancreas. The biliary dysgenesis was characterized by proliferation of abnormally shaped ducts in the portal tracts. Massive enlargement and cystic dysplasia of the pancreas was associated with ductular proliferation, virtual absence of normal exocrine tissue, and an increase in endocrine tissue.

Glycogen-rich clear cell carcinoma in the tongue. A cytochemical and ultrastructural study.

An unusual epithelial neoplasm arising in the tongue of a 17 month-old-boy was studied by light and electron microscopy. By routine light microscopic examination, the tumor cells exhibited clear cytoplasm. Histochemical studies revealed the presence of large amounts of periodic acid-Schiff (PAS)-positive, diastase-labile material in the cytoplasm. Ultrastructurally, this material was identified as electron dense particles (20-35 nm diameter) of beta-glycogen. The epithelial nature of the tumor cells was suggested by the presence of intercellular junctions, segments of basal lamina, and well-developed Golgi complexes. Furthermore, the cells were often clustered and formed abnormal lumina with small microvillus projections. Several histologic features, such as lack of capsule, infiltration of muscle and invasion of blood vessel, indicated that this neoplasm was malignant. However, there was no recurrence or distant metastases more than 2.5 years after surgical excision, suggesting a low-grade malignancy. The glycogen-rich clear cell variant of adenocarcinoma is histogenetically derived from minor salivary glands like most of the glandular neoplasms in the oral cavity. Investigation of the literature on adult and childhood minor salivary gland neoplasms revealed that the glycogen-rich, clear-cell variant of adenocarcinoma has not previously been reported in the tongue.

Electron microscopy of glandular Schwannoma.

This report is the first description of the ultrastructure of the glandular elements in a so-called glandular Schwannoma (malignant peripheral nerve sheath tumor with glandular elements). The appearance of this uncommon tumor was typical by light microscopy. Electron microscopy of the sarcomatous component revealed features consistent with Schwann cell origin. Ultrastructure of the glands revealed a well-differentiated epithelium with basement membrane, desmosomes and the presence of microvilli with core rootlets, glycocalyx, and intracytoplasmic R-bodies. No cilia or blepharoplasts were present, arguing against ependymal origin of these glands. The microvilli with core rootlets, the glycocalyx and the R-bodies indicate the glandular epithelium was of the "intestinal type." The basis for the presence of such epithelium in Schwann cell neoplasms is uncertain, but pathologic induction-interaction between neural crest and mesenchyme may be the most plausible explanation.