glycogen and Mandibular-Neoplasms

Ewings' sarcoma involving the facial bones is rare although it is the second most frequent bone malignancy. For the diagnosis a biopsy is needed. Currently the treatment is the combination of chemotherapy and radiotherapy with surgery indicated in only a few instances. We report a case of Ewing's sarcoma of the mandible and describe its clinicopathologic features and the treatment of this disease.

Topics: 12E7 Antigen; Adolescent; Antigens, CD; Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Cell Adhesion Molecules; Combined Modality Therapy; Female; Glycogen; Humans; Mandibular Neoplasms; Sarcoma, Ewing; Vimentin

Clear cell squamous cell carcinoma (CCSCC) is a rare variant of squamous cell carcinoma, first reported by Kuo, who described 6 cases of squamous cell carcinoma of the skin of the head and neck. CCSCC is composed of cells with clear cytoplasm, which Kuo attributed to the accumulation of intracellular fluid and not the presence of glycogen, lipid, or mucin. This case describes a 59-year-old woman who presented with an exophytic, hemorrhagic lesion on the posterior mandibular gingiva of 2 months' duration. Histologic examination revealed dysplastic stratified squamous epithelium showing transition to an infiltrating tumor composed of islands of epithelial cells with clear cytoplasm. The cytoplasm stained positive with periodic acid Schiff but was diastase labile. Mucicarmine stains were negative for intracytoplasmic mucin. This is the first reported case describing primary glycogen-rich CCSCC of the mandibular gingiva.

Topics: Carcinoma, Squamous Cell; Female; Gingiva; Glycogen; Humans; Mandibular Neoplasms; Middle Aged

Ewing family of tumors (EFTs) comprise highly malignant, nearly undifferentiated neoplasms including Ewing's sarcoma (ES), primitive neuroectodermal tumor (PNET) and a spectrum of other unusual variants. In general, EFTs are included among small blue cell tumors. Establishing histological diagnosis can be difficult; CD99 and FLI1 immunohistochemical staining has improved diagnosis, but these markers are not specific for EFTs. The diagnosis of EFTs is confirmed by molecular diagnostic testing showing the presence of established rearrangements of the EWS gene. The use of this molecular signature in EFTs revealed rare variants in the histomorphologic spectrum of these tumors. The authors report an unusual variant of EFT in the mandible of a 17-year-old patient, which was confirmed by translocation rearrangement in EWR1 gene at 22q12 by fluorescence in situ hybridization. The unusual histologic features, with prominent spindling of tumor cells and deviation from the classic features of Ewing's sarcoma posed a diagnostic challenge for the medical centers involved in the diagnosis and treatment of this patient. This highlights the importance of the genetic study of undifferentiated sarcomas to identify rare morphologic variants of ES, in view of the chemosensitivity of EFTs and how this affects patient management.

Topics: 12E7 Antigen; Adolescent; Antigens, CD; Carboxylesterase; Cell Adhesion Molecules; Chondrosarcoma, Mesenchymal; Chromosomes, Human, Pair 22; Diagnosis, Differential; Female; Follow-Up Studies; Gene Rearrangement; Glycogen; Humans; In Situ Hybridization, Fluorescence; Mandibular Neoplasms; RNA-Binding Protein EWS; Sarcoma; Sarcoma, Ewing; Translocation, Genetic

We describe two cases of primary intraosseous hyalinizing clear cell carcinomas of the jaws. One tumor arose in the mandible, the other in the maxilla. Both patients were adult women. Histologically, both tumors were characterized by a population of clear, glycogen-rich cells arranged in solid nests, trabeculae, and occasional single files, surrounded by a markedly hyalinized fibrous stroma. In close admixture with the clear cells, a second component of smaller cells having eosinophilic cytoplasms was also noted. Focal areas of squamous metaplasia were seen in one of the tumors. Neither nuclear pleomorphism nor mitotic activity were conspicuous features. Mucicarmine stains were negative in both tumors. Immunohistochemically, the tumor cells expressed cytokeratins and epithelial membrane antigen. Stains for S100 protein, smooth muscle specific actin, and carcinoembryonic antigen were all negative. Both patients are alive and well 14 and 17 months respectively after the diagnosis without evidence of metastasis or recurrence. The major importance of recognizing the existence of this rare form of salivary gland-type adenocarcinoma arising as a primary lesion in the mandible or the maxilla lies in avoiding misdiagnosing it as other primary or metastatic clear cell neoplasms, including various odontogenic clear cell tumors, other primary intraosseous salivary gland tumors, and metastatic clear cell adenocarcinomas from other organs, particularly the kidney.

Topics: Adenocarcinoma, Clear Cell; Aged; Diagnosis, Differential; Female; Glycogen; Humans; Hyalin; Mandibular Neoplasms; Maxillary Neoplasms; Middle Aged; Odontogenic Tumors; Salivary Gland Neoplasms

A case of clear cell odontogenic tumour, which occurred centrally in the mandible of a 56-year-old Japanese woman, is reported with its histochemical, immunohistochemical and ultrastructural findings. Histologically, the tumour nests were composed of large glycogen-rich clear cells and small non-clear polygonal cells and were separated by thin mature fibrous connective tissue septae. Immunohistochemically, both types of tumour cells showed positive expression of various cytokeratins, in particular cytokeratin 19, and of epithelial membrane antigen. Eosinophilic hyaline deposits and possible dentin-like structures were occasionally formed in contact with the epithelial nests and are regarded as indicative of the epithelial-mesenchymal inductive capacity of this tumour. The aggressive nature of the present tumour was assumed through its invasive growth pattern and occasional mitotic figures. Although it was diagnosed as clear cell odontogenic tumour according to the present WHO classification, the patient must be followed carefully because of its probable malignant nature.

Topics: Connective Tissue; Dentin; Epithelial Cells; Female; Glycogen; Humans; Hyalin; Immunohistochemistry; Keratins; Mandibular Neoplasms; Mesoderm; Middle Aged; Mitosis; Mucin-1; Neoplasm Invasiveness; Odontogenic Tumors

The authors report the case of an 8-year-old boy with a Ewing's sarcoma localized to the mandible. The therapeutic modalities consisting of induction chemotherapy, surgical removal of the involved portion of the mandible with reconstruction using a parascapular osteo-cutaneous free flap, and maintenance chemotherapy are described. Emphasis is placed on the importance of performing a wide resection and the use of a multidisciplinary team approach in the treatment of these unusual tumours.

Topics: Cell Nucleolus; Cell Nucleus; Child; Chromatin; Cytoplasm; Glycogen; Humans; Male; Mandibular Neoplasms; Sarcoma, Ewing; Vimentin

Topics: Carcinoma; Diagnosis, Differential; Female; Glycogen; Humans; Immunohistochemistry; Keratins; Mandibular Neoplasms; Middle Aged; Salivary Gland Neoplasms; Salivary Glands, Minor

An extensive review of the English-language literature has produced only five undoubted cases of peripheral odontogenic fibroma. This article reports ten new cases. The age and sex of the patients and the distribution by site and histology of the lesions are compared with those of other cases of bifrous tumors and neoplasms of odontogenic origin. It is postulated that this lesion may be more closely related to the fibrous euplis than was previously realized. Both lesions show hyperplasia of the basal layer of the covering ipithelium into double strands resembling odontogenic epithelium. It is suggested that this hyperplasia may be related to induction of the covering epithelium by primitive ectomesenchymal remnants within the gingivae. Proliveration of odontogenic rests within the peripheral odontogenic frbroma may be related to the same stimulus.

Topics: Adolescent; Adult; Age Factors; Basement Membrane; Calcinosis; Cell Nucleus; Child; Collagen; Connective Tissue; Connective Tissue Cells; Cytoplasm; Epithelial Cells; Epithelium; Female; Gingival Neoplasms; Glycogen; Humans; Male; Mandible; Mandibular Neoplasms; Maxilla; Maxillary Neoplasms; Middle Aged; Odontogenic Tumors; Sex Factors; Staining and Labeling