Robert D. Foss

Estrogen and progesterone receptors in salivary gland adenoid cystic carcinoma.

Adenoid cystic carcinomas of salivary glands occur more frequently in women and bear remarkable similarity to adenoid cystic carcinomas of the breast. In addition, breast carcinomas express estrogen and progesterone receptors that impact prognostic significance. This suggests a possible role for sex steroid hormones in the development and progression of salivary gland adenoid cystic carcinoma. On this basis, 12 samples of formalin-fixed, paraffin-embedded salivary gland adenoid cystic carcinomas and 12 samples of normal salivary gland tissue were immunohistochemically evaluated for estrogen and progesterone receptor protein expression. Estrogen receptors were not detected in either group; however, a significantly higher progesterone receptor level was evident in the neoplastic group compared with normal tissue (p < 0.01). These data confirm the presence of progesterone receptors within normal and neoplastic salivary gland tissue. Progesterone receptor expression may be of possible prognostic and therapeutic value in some cases of adenoid cystic carcinoma.

Synovial sarcoma involving the head: analysis of 36 cases with predilection to the parotid and temporal regions.

Synovial sarcoma involving the head is rare, and data on the clinicopathologic characteristics of such tumors are scant. In this study, we examined 36 synovial sarcomas of the head excluding tumors in the oral cavity, sinonasal tract, submandibular area, neck, and intracranial space. There were 19 men and 17 women with a mean age of 35 years (range: 4 to 85 y). There was a marked predilection for the parotid (n=14) and temporal regions (n=9), and cheek (n=4). Other locations included mastoid area (n=2), infratemporal fossa (n=2), and one each from the supra-auricular scalp, maxillary, submaxillary, mandibular, and nasolabial regions. Histologically, 25 examples were of monophasic type, 10 were biphasic. Five of these cases contained a poorly differentiated Ewing sarcoma-like component and 1 was purely poorly differentiated. Histologically, 9 tumors involved skeletal muscle, 4 parotid gland (focally or in the interlobular septa), and 1 intertrabecular spaces of bone; the others involved subcutis or fascia and rarely skin. The tumor size ranged from 0.6 to 7.0 cm (median: 3.5 cm) and mitotic activity varied from <1 to 85 per 10 high-power fields (HPFs) (median, 6/10 HPFs). Keratin-positive tumor cells were detected in 19 of 19 monophasic and 1 of 1 of poorly differentiated tumors that were examined. SS18 gene rearrangement was confirmed in all 14 cases examined (3 biphasic and 11 monophasic tumors). Follow-up on 29 patients revealed that 11 were alive without disease from 2 to 31 years (median, 14 y). Ten patients died of disease 1 to 18 years after the diagnosis (median, 3 y); most of these patients had a tumor >5 cm and 6 of 10 had mitotic counts >10/10 HPFs. One patient died of an unrelated cause (metastatic melanoma) and 7 died of unknown causes. Four other patients had subsequent malignancies, including carcinomas of the breast, esophagus, rectum, and parotid gland. The latter was possibly radiation-induced, diagnosed 30 years after the synovial sarcoma. Synovial sarcoma of the head has a striking predilection for the parotid and temporal regions and the prognosis varies with many patients having long tumor-free survivals.

Lymphoepithelioma-like carcinoma of head and neck skin: a systematic analysis of 11 cases and review of literature

Lymphoepithelioma-like carcinoma of the skin (LELCS) is a rare tumor of unknown etiology, low malignant potential, and microscopic resemblance to undifferentiated nasopharyngeal carcinoma. Clinically, it presents as a flesh-colored firm nodule or plaque on the face, scalp, or shoulder of middle-aged to elderly individuals. Histologically, LELCS is composed of islands of enlarged epithelial cells with large vesicular nuclei surrounded and permeated by a dense lymphoplasmacytic infiltrate. LELCS exhibits immunoreactivity with high-molecular-weight cytokeratins and epithelial membrane antigen, indicating the epithelial origin. The differential diagnosis includes basal cell carcinoma, squamous cell carcinoma, lymphoma, pseudolymphoma, and Merkel cell carcinoma. We report 11 cases of LELCS of the head and neck region with discussion of the clinical, histopathologic, immunohistochemical, and therapeutic aspects of this rare cutaneous neoplasm. In addition, we systematically review and compare the findings with the previously published cases of LELCS. This study is the largest case series of LELCS reported in the English-language literature. It attempts to more clearly define the diagnostic criteria for LELCS. Its histomorphologic and immunophenotypic features help distinguish this tumor from similar-appearing malignancies, including metastatic nasopharyngeal carcinoma.

Quantitative analysis of activating alpha subunit of the G protein (Gsα) mutation by pyrosequencing in fibrous dysplasia and other bone lesions.

Benign fibro-osseous lesions (BFOLs) frequently display overlapping histological features. The differentiation of fibrous dysplasia (FD) from other BFOLs can be difficult, even for experienced orthopedic pathologists. Accurately distinguishing FD from other BFOLs may have significant clinical and treatment implications. A somatic mutation in gene GNAS encoding the α subunit of the G protein (Gsα) involving the codon corresponding to Arg 201 has been identified in FD and is specifically absent in other BFOLs. We have developed a quantitative assay by pyrosequencing that has a detection sensitivity of 95%. The test allows the identification of the two most common types of mutation (Arg→His and Arg→Cys) in a single reaction, with the ability to analyze other rare mutations. Of the 24 FD cases in this series, 23 (96%) were positive for GNAS/Gsα mutation. Nineteen of 23 positive cases exhibited a G→A mutation (Arg→His), whereas four had a C→T mutation (Arg→Cys). One of three BFOL, not otherwise specified cases was positive for G→A mutation. None of the osteofibrous dysplasia, ossifying fibromas, or other bone lesions were positive for this mutation. Our experience is that pyrosequencing is an easy and accurate quantification method for Gsα mutation detection in fibrous dysplasia. Mutation analysis of the Gsα by pyrosequencing has significant potential for improving discrimination between FD and other BFOLs in problematic cases.

Juvenile Psammomatoid Ossifying Fibroma

Disclaimer: The opinions and assertions expressed herein are those of the authors and are not to be construed as official or representing the views of the Department of the Army, Department of the Navy or the Department of Defense.

Primary anaplastic large cell (KI-1 positive) lymphoma of the mandible as the initial manifestation of acquired immunodeficiency syndrome in a pediatric patient.

A case of an anaplastic large-cell lymphoma with Ki-1 immunohistochemical reactivity presenting in the mandible of a 12-year-old girl with Walter Reed Stage 6 human immunodeficiency virus infection is described.

Right maxillary sinus sarcomatoid carcinoma (sarcomatoid/spindle cell carcinoma)

Carcinomas of the maxillary sinus are uncommon, with an annual incidence of 1:100,000 in the United States. They comprise 0.2 percent to 0.5 percent of all cancer cases, 3 percent of all cases of head and neck carcinoma, and 80 percent of all cases of paranasal sinus carcinoma. Because of the typically high stage presentation of maxillary sinus disease at the time of diagnosis, this carcinoma presents significant therapeutic challenges for treating physicians and an ominous prognosis for the patient. The biological behavior of these uncommon tumors is difficult to predict, although deeply invasive and destructive high-grade tumors tend to have the worst prognosis, thus indicating the importance of early diagnosis. A 54-year-old Hispanic male presented to the emergency room with a 3-week history of progressive swelling of the right side face of the maxilla. The patient complained of localized facial tenderness, increasing dysphagia, generalized weakness, fatigue, subjective hearing loss, and fullness in the right ear. He also noted a 25-lb weight loss and a decreased appetite over the prior two weeks. His past medical history was significant for a 40-pack-year smoking history and 15-year history of alcohol abuse. Previously he was employed as a steel worker. His grandfather had died from complications of an unknown type of cancer. Physical examination was significant for a large swelling (8 4 cm) of the right maxilla extending from the periparotid and molar areas to the submandibular area (Fig 1A). Matted lymphadenopathy was also noted in the ipsilateral neck at levels I and II and in the contralateral neck at level II. Intraoral exam of the right maxilla demonstrated bulging of the buccal gingival sulcus with expansion of the alveolar ridge, and partial erosion of the mucosa at the junction of the hard and soft palate. Nasopharyngoscopy was normal on the left and revealed a fully occluded right nasopharynx. Computed tomography (CT) scans with contrast of the facial mass were obtained and showed a lytic, destructive lesion centered within the maxilla (Fig 1B). There was erosion of the anterior, lateral, and posterior sinus wall with extension into the pterygoid musculature. The orbital floor was thin but appeared intact without involve-

Occult primary parotid gland acinic cell adenocarcinoma presenting with extensive lung metastasis.

Acinic cell adenocarcinoma is a malignant salivary gland neoplasm with a relatively low rate of lymphangitic spread to regional lymph nodes. Distant metastases are rare and their occurrence typically indicates an unfavorable outcome. We encountered an unusual example of acinic cell adenocarcinoma that initially presented in the lung, whereas the primary parotid carcinoma, despite extensive clinical evaluation, only became apparent 1 year after initial diagnosis. The histologic, immunohistochemical, and ultrastructural features of the tumor in the parotid gland and lung were similar. The tumor displayed an aggressive behavior resulting in death within 2 years of the initial presentation. This presentation is unique, showing that peripheral lung tumors of salivary gland type are likely to be metastatic, and careful clinical evaluation is warranted in establishing their primary site of origin.

Ectomesenchymal chondromyxoid tumor: report of a case with description of histologic and immunohistochemical findings.

The ectomesenchymal chondromyxoid tumor (ECT) is a rare tumor usually of the anterior tongue, first described by Smith et al 1 in 1995. Since that time, a total of 44 cases of ECT have been reported in the literature. 2 ECT is a painless, slowly growing nodule that is usually seen on the dorsum of the tongue. It can occur over a wide age range and can be present for many months to many years before the patient seeks treatment. Histologically, it is a lobular non-encapsulated proliferation of ovoid, round, and fusiform cells in a chondromyxoid background, often showing blunt infiltration of surrounding tissues. Focal cytologic atypia and occasional mitotic figures may be seen. From the immunohistochemical standpoint, the cells are consistently reactive for glial fibrillary acidic protein (GFAP) but variably reactive to cytokeratins, smooth muscle actin, S-100 protein, epithelial membrane antigen, and CD57. 1-4

Seromucinous Hamartoma of the Nasal Cavity

Seromucinous hamartoma (SMH) is a rare benign epithelial proliferation occurring in the sinonasal tract. The clinical, radiographic, and histologic appearance of SMH may mimic several benign and malignant entities. Presented is a novel case, with a review of the literature focused on potential histologic diagnostic pitfalls.