Thyroid-like papillary adenocarcinoma of the nasopharynx: a case report in a 19-year-old male.

Primary thyroid-like papillary adenocarcinomas of the nasopharynx are rare, with only a limited number of cases reported in the literature. However, it is important that pathologists and clinicians alike recognize this entity, as these neoplasms are amenable to surgical resection and carry an excellent prognosis. In this article, we describe the case of a thyroid-like papillary adenocarcinoma of the nasopharynx in a 19-year-old male, with emphasis on clinical findings, histologic and immunohistochemical characteristics, and prognosis. In addition, we include a review of the literature to highlight what we know to date about this rare entity.

Pseudomembranous disease (ligneous inflammation) of the female genital tract, peritoneum, gingiva, and paranasal sinuses associated with plasminogen deficiency.

Pseudomembranous disease (or ligneous inflammation) is a rare condition characterized by accumulations of fibrin-rich eosinophilic material. Recent investigations have linked the etiology of this condition to plasminogen deficiency (hypoplasminogenemia). Although much of the literature concerning this disease has focused upon the often clinically striking ocular manifestations, it is important to note that pathologic changes may develop in a variety of anatomic locations, including the oral cavity, upper and lower respiratory tract, female genital tract, kidneys, and gastrointestinal tract. Here, we report an unusual case of a 33-year-old woman who initially presented with gingival inflammation. In subsequent years, she developed additional signs and symptoms related to sinonasal and genital tract involvement. Despite numerous clinical evaluations, biopsies, and laboratory tests, the patient's diagnosis remained elusive for 7 years. Ultimately, it was the distinctive appearance of the gingiva that led to a diagnosis of plasminogen deficiency. Unfortunately, the complicated clinical course and elapsed time between initial presentation and diagnosis illustrated by the present case are not uncommon among patients with this condition. Greater familiarity with the clinical and histopathologic features of this condition among pathologists and treating clinicians is essential for timely diagnosis and management.

Leukocyte adhesion deficiency type 1: an important consideration in the clinical differential diagnosis of prepubertal periodontitis. A case report and review of the literature.

Leukocyte adhesion deficiency type 1 (LAD-1) is a rare, inherited immunodeficiency that affects 1 in 1 million people yearly and usually presents with recurrent, indolent bacterial infections of the skin, mouth, and respiratory tract and impaired pus formation and wound healing. Features of this disease result from mutations in the region of the CD18 gene, which is encoded on chromosome 21q22.3. This gene codes for the common subunit of the leukocyte integrins LFA-1, Mac 1, and p150,95. Failure to produce a functional subunit results in the defective expression of all 3 leukocyte integrins, and the leukocytes of LAD have subnormal adhesion properties. We present a case of the moderate-to-severe form of LAD in a 3-year-old girl who initially presented with generalized swelling and erythema of the gingiva, with slight tooth mobility and a nonhealing labial ulceration. Her medical history was significant for recurrent urinary tract infections. Periodontal pathogens, including Capnocytophaga, Eikenella corrodens, and Candida albicans, were cultured. The patient had a significantly elevated white blood cell count and absolute neutrophil count. The diagnosis of LAD was confirmed with flow cytometry, which revealed significantly decreased subunits. Twenty-four months after the diagnosis was made and after a series of granulocyte transfusions and bone marrow transplantations, she expired as the result of respiratory failure.

Ameloblastic carcinoma: an analysis of 14 cases.

OBJECTIVES: To present the classifications of malignant ameloblastomas, provide histopathologic guidelines for the diagnosis of ameloblastic carcinoma, and discuss treatment and long-term follow-up. STUDY DESIGN: Fourteen archival specimens of ameloblastic carcinoma with detailed treatment and follow-up documentation were identified by the authors. RESULTS: Traditional methods of microscopic observation form the basis for diagnosis. Histopathologic features of ameloblastic carcinoma include lack of differentiation, hypercellularity, high mitotic index, vascular invasion, and neural invasion. CONCLUSION: The long-term follow-up findings in this study support the concept that aggressive surgical intervention provides the best chance for survival. Patients treated otherwise had recurrence. The patients with the highest number of recurrences did not survive the disease.

Epithelioid hemangioendothelioma of the oral cavity: report of two cases and review of the literature.

The epithelioid hemangioendothelioma is an uncommon vascular neoplasm of borderline or intermediate malignant potential. Although numerous sites of involvement are possible, these tumors most commonly arise in soft tissue, liver, and lung. Involvement of the oral cavity is rare. Only 12 cases of intraoral epithelioid hemangioendothelioma have been reported in the English language literature. We review the salient features of these previously reported cases and present 2 additional intraoral cases--one presenting as an asymptomatic radiolucency in the posterior mandible of a 23-year-old female, and the other presenting as an asymptomatic, erythematous to purplish gingival nodule in a 28-year-old female. Intraoral tumors most commonly involve the gingival soft tissues and often are associated with adjacent alveolar bone resorption. Although it is not possible to estimate with accuracy the potential for recurrence and metastasis among intraoral tumors given the small number of previously reported cases, wide local excision with close clinical follow-up appears to be the treatment of choice for these tumors because of their unpredictable clinical behavior.

Transformation of a benign oral pigmentation to primary oral melanoma.

OBJECTIVE: The objective is to report the serendipitous 7-year follow-up and transformation of a melanotic palatal lesion, which was initially diagnosed histologically as a benign oral melanotic macule, into primary oral melanoma and to provide long-term follow-up of a case of oral malignant melanoma. STUDY DESIGN: Nine formalin-fixed paraffin-embedded tissue blocks from several different facilities and microscopic slides of the patient's lesions were reviewed to study the transformation of a benign, oral, pigmented lesion into melanoma. RESULTS: Review of blocks and slides of the patient's lesions suggest that the onset of melanocytic hyperplasia (increased clear cell activity) heralded the transformation of the melanotic macule into melanoma. The histology of the first biopsies was totally benign, but retrospectively, the clinical appearance was not typical and was somewhat worrisome because of the size of the lesion and feathering of the pigmentation at the periphery. This clinical presentation, however, was unknown to the pathologists. CONCLUSIONS: This study documents a case of primary oral melanoma arising from an apparent oral melanotic macule and suggests that even histologically benign-appearing oral melanotic lesions should perhaps be viewed with caution if increased numbers of melanocytes (melanocytic hyperplasia or clear cell activity) are present. Careful correlation of clinical appearance and histology is necessary to arrive at an appropriate diagnosis and prognosis for oral pigmented lesions.