James T. Castle

Polymorphous Low-Grade Adenocarcinoma

Polymorphous low grade adenocarcinomas (PLGA) are minor salivary gland neoplasms with a predilection for intraoral sites.

Alveolar soft part sarcoma of the tongue: Report of an unusual pattern in a child

A case of an unusual, primary alveolar soft part sarcoma of the tongue in a 3-year-old boy is presented. Alveolar soft part sarcoma is a rare malignant tumor that presents more frequently in the head and neck region of infants and children. To date, only six cases of lingual alveolar soft part sarcoma presenting in the first decade of life have been reported in the English literature. The patient has undergone surgical resection without adjuvant chemotherapy or radiation. Thus far, the patient does not manifest persistent or recurrent disease.

Aggressive (malignant) epithelial odontogenic ghost cell tumor

A 57-year-old man had an expanding cystic lesion of the anterior maxilla that demonstrated destruction and disruption of local structures. A cystic odontogenic neoplasm as well as various forms of odontogenic carcinoma was suspected. Incisional biopsy and microscopic examination revealed an aggressive epithelial odontogenic tumor with histologic features suggesting malignant transformation from an odontogenic cyst. The patient continues to refuse further treatment despite remaining tumor. This case further documents the malignant potential of a calcifying odontogenic cyst and the existence of at least an aggressive or possibly malignant form of epithelial odontogenic ghost cell tumor. To date, metastatic work-up has not been performed.

Lymphangioma of the vallecula.

HistoryA 47 year-old male presented with a chief complaint ofrecurrent neck pain, as well as a ‘‘shooting pain’’ whichtraveled down his arms whenever he coughed or sneezed.Radiographic FeaturesAn axial T2-weighted magnetic resonance imaging studyof the current case (Fig. 1) revealed a small mucosal basedlesion which appeared to be at the level of the left lingualtonsil and involved the vallecula. This mass appeared to beintruding on rather than arising from the vallecula.DiagnosisThe hematoxylin and eosin stained surgical specimen(Fig. 2) is composed of variably dense fibrovascular stromanotable for numerous ovoid and irregularly shaped vesselscontained within. The vessels are lined by unremarkable,flattened endothelial cells which are widely separated. Thesupporting collagenous stroma along with the thin lining,occasionally form into thin papillations which projectslightly into the luminal space. High power view (Fig. 3)shows thin septations separating the luminal space intocompartments which contains thin, frothy, eosinophilicproteinaceous material as well as numerous lymphocytes.These vascular spaces remain superficially placed and donot dissect deep into the connective tissue.DiscussionLymphangiomas are rare congenital lesions of the lym-phatic system that occur mainly within the head and neck,particularly in the posterior triangle, but are commonlyfound within the oral cavity as well [1]. Based on the sizeof the cavities that are involved in the lesion, classificationis separated into variants termed capillary, cavernous, andcystic hygroma. Multiple patterns are frequently noted tobe within the same lesion. Capillary and cavernous variantsof lymphangioma are confined within dense structural tis-sue areas whereas, cystic hygroma occur where expansioninto large, loculated, cystic spaces can develop, such aswithin the neck [2].Involvement of lymphangioma within the larynx isusually the result of direct extension of a congenital lesionoriginating within the neck, although cases of isolated lar-yngeal lymphangiomas have been reported as rare entitiesarising in children and adults [3–8]. The most commonlocation is in the supraglottis within the epiglottis, aryepi-glottic folds and arytenoids [8]. Being difficult to diagnoseunless the lesion is allowed to grow to a large size, symp-toms are non-specific until dyspnea, dysphagia, or a voicechange signals a need for further clinical investigation.The histologic differential diagnosis would includehemangioma and possibly metastatic papillary cystic thy-roid carcinoma. Great histologic similarity exists betweenlymphangioma and hemangioma as both can be capillarysized and show a thin endothelial lining, but in hemangi-oma, the endothelial cells may appear plumper. Lymphan-giomas occasionally show short papillations projecting a

Chondromyxoid Fibroma of the Ethmoid Sinus

Abstract : A 43-year old Black female complained of sinus pressure and was found to have a mass of the right ethmoid sinus. A T1-weighted fat-suppressed (FS) post-clear coronal magnetic resonance imaging (MRI) study revealed right mid-level ethmoid air cells that were expanding the sinus cavity. A slightly lower density signal compared to brain was noted, at 35 Hounsfield units, and was found to be isointense with skeletal muscle. Notably, this coronal image showed that the mass was not descending from the cranial compartment, thereby effectively ruling out an encephalocele. A T1 axial FS MRI showed bone remodeling as the tumor had eroded through the lamina papyracea at the medial wall of the orbit. A fluid attenuated inversion recovery MRI indicated the absence of bright signals to indicate hemoglobin, necrosis, calcification, or proteinaceous debris. No edema was noted in the medial rectus muscle and no infiltration of fat of the orbit was identified, suggesting a non-aggressive nature to this lesion.

Cholesteatoma Pearls: Practical Points and Update

The European Academy of Otology and Neurotology in collaboration with the Japanese Otological Society (EAONO/JOS) recently produced a joint consensus document outlining the definitions, classification and staging of middle ear cholesteatoma. The goals were to provide terminologies in the description of cholesteatoma, classify cholesteatoma into distinct categories to facilitate the comparison of surgical outcomes and to provide a staging system that reflects the severity, difficulty of complete removal and restoration of normal function. Cholesteatoma is considered a benign, expanding and destructive epithelial lesion of the temporal bone that is the result of a multifactorial process. If undetected and left treated, cholesteatoma may lead to significant complications including hearing loss, temporal bone destruction and cranial invasion. Recent advances in imaging modalities have allowed for high sensitivity and specificity in identifying the presence of cholesteatoma. Despite these advances, deficiencies exist around the world with access to health care facilities meaning cholesteatoma remains a serious and challenging entity to manage whether found within the pediatric or adult population. Proper diagnosis and management of each form of cholesteatoma is achieved by a thorough understanding of the etiology, classification, clinical presentation and histology, thereby facilitating prevention, early detection and appropriate treatment.