Esther L.B. Childers

Extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck

We report 14 cases of extranodal sinus histiocytosis with massive lymphadenopathy involving a variety of head and neck sites. The patients ranged in age from 3 to 70 years (median, 43 years). Nine cases occurred in women and five occurred in men. The clinical presentation varied depending on the site of occurrence and included nasal obstruction, stridor, proptosis, ptosis, decreased visual acuity, facial pain or tenderness, cranial nerve deficits, mandibular tenderness, and mass lesions. Head and neck sites involved by disease included the nasal cavity, paranasal sinuses, nasopharynx, parotid gland, submandibular gland, larynx, temporal bone, infratemporal fossa, pterygoid fossa, meninges, and orbital region. The majority of patients presented with involvement of more than one site. Nodal involvement was identified in four patients. Special stains for microorganisms were negative. The sinus histiocytosis with massive lymphadenopathy cells demonstrated an immunophenotypic profile supporting derivation from macrophage/histiocytic lineage. Treatment varied and included surgical excision with or without adjuvant therapy (chemotherapy, radiotherapy) or steroids. Several patients required more extensive surgery as a result of extension of their disease to adjacent structures or due to recurrent disease. Twelve patients are alive and either free of disease or have persistent disease. Two patients died, one as a result of complications of disease.

Lingual alveolar soft part sarcoma; 14 cases: novel clinical and morphological observations

Aims:  Alveolar soft part sarcoma (ASPS) is a rare sarcoma in the buttocks or thigh of young adults, often with metastases to lung, brain, or bone. This study examines the morphological and clinical features of lingual ASPS.

Oral and salivary gland angiosarcoma: a clinicopathologic study of 29 cases.

Angiosarcomas of the oral and salivary gland area are extremely rare, mostly presented as case reports. We wanted to study the clinicopathologic features of a series of oral and salivary gland angiosarcomas. Cases coded as “angiosarcoma” were retrieved from the Oral and Maxillofacial Pathology Department of the Armed Forces Institute of Pathology. Patient folders and pathology were reviewed and recorded; immunohistochemistry and follow-up were obtained. Inclusion required oral or salivary gland location, vasoformative growth, cytologic atypia, mitoses, and vascular markers. Skin, bone, and subcutaneous angiosarcomas were excluded. Primary and secondary (metastatic) oral angiosarcomas were included. The 22 primary angiosarcomas involved tongue (n = 9), parotid (n = 4), lip (n = 4), submandibular gland (n = 3), and 1 each of soft and hard palate. The 7 secondary angiosarcomas involved the gingiva (n = 4) and parotid gland (n = 3). Overall, patient ages ranged from 6–90 years (mean, 55 years). There were 15 males and 14 females. Symptoms included a mass with recent enlargement and bleeding. Tumor sizes ranged from 0.8–7.0 cm (mean, 2.6 cm). Histologically, all tumors were vasoformative; 86% had solid and 17% had distinctive papillary areas. Eight (28%) were classified as the epithelioid subtype. Immunohistochemical stains showed that the tumor cells were positive for Factor VIIIrag in 19/21, CD31 in 16/19, CD34 in 7/12, and Ulex in 1/1. Primary tumors were classified as low grade (n = 7, in all locations except salivary gland), intermediate (n = 7), and high grade (n = 8); all secondary tumors were high grade. Follow-up was available on 14/22 primary and 7/7 secondary angiosarcomas. Of primary tumors, two tongue angiosarcoma patients died at 1 and 9 years, but 4 were alive without disease over a mean of 7.3 years (range, 1–13 years). Four primary salivary gland angiosarcoma patients were alive without disease over a mean of 5.8 years (range, 1–14 years), and 1 had only a late (15 years) metastasis and death (at 20 years). Three primary lip angiosarcoma patients were without disease over a mean of 14.3 years (range, 13–16 years). Of secondary tumors, three salivary gland angiosarcoma patients died within 1 year, and all four secondary gingival angiosarcoma patients died of disease within 3 years. Assessing follow-up of primary oral and salivary gland angiosarcoma patients by grade, 5 patients with high-grade tumors had no evidence of disease over a mean of 7.6 years (range, 1–16 years), 3 patients with intermediate-grade tumors had no evidence of disease over a mean of 12.7 years (range, 11–14 years), 2 patients with intermediate-grade tumors died of disease at 9 and 20 years, 3 patients with low-grade tumors had no evidence of disease over a mean of 6.3 years (range, 1–14 years), and 1 patient with low-grade tumor died of disease at 1 year. Primary oral and salivary gland angiosarcomas, albeit rare, mostly involve the tongue, parotid gland, and lip of adults, often with relatively good outcome. Although the most common angiosarcoma morphology in this area is spindled vasoformative and solid, almost one third of oral and salivary gland angiosarcomas are the rare epithelioid angiosarcoma variant. Most gingival and few parotid angiosarcomas appear to be metastases from other locations, with many patients succumbing to death within 3 years. Despite predominantly high- or intermediate-grade morphology, patients with primary angiosarcoma of the tongue, salivary gland, and lip have a better prognosis than do patients with primary cutaneous or deep soft tissue angiosarcoma, including those patients with secondary oral and salivary gland involvement.

Liposarcoma of the Oral and Salivary Gland Region: A Clinicopathologic Study of 18 Cases with Emphasis on Specific Sites, Morphologic Subtypes, and Clinical Outcome

Liposarcoma is rare in the oral and salivary gland region (OSG), previously described in only case reports and two small series. Clinicopathologic features of a large series of these tumors were studied. Cases coded as “liposarcoma or lipoma” from 1970 to 2000 were searched for in our files. Inclusion required an OSG location and diagnosis by established soft tissue criteria. Dermal, other soft tissue, and intraosseous liposarcomas were excluded. Clinical and pathologic material was reviewed and follow-up obtained. Eighteen liposarcomas were included: 10 from males and 8 from females. The median patient age was 51 years (range, 30–70 years). Specific anatomic locations included buccal mucosa (n = 7), tongue (n = 4), parotid gland (n = 3), soft tissue overlying the mandible (n = 2), and one each of palate and submandibular gland. The average tumor size was 4.2 cm (range, 1.5 to 6.0 cm). Histologically, most tumors were well differentiated, including one atypical lipoma (n = 10), followed by myxoid (n = 5) and dedifferentiated (n = 3). OSG liposarcomas of all subtypes had increased numbers of lipoblasts. All patients were treated with surgical excision alone. Follow-up on 15 patients (83%) over a mean of 16.5 years (range, 2 to 53 years) revealed that three patients had between one and six local recurrences over periods of 18 months to 6 years. Twelve patients were without recurrence, with a mean follow-up of 12.8 years (range, 2–23 years). No patients, including those with dedifferentiated liposarcoma, had metastases or died of disease. OSG liposarcomas are rare tumors of adults, occurring most commonly in the buccal mucosa, tongue, and then parotid gland. There were no pleomorphic liposarcomas in this series; well-differentiated liposarcoma was the most common subtype, which can locally recur but, even with high-grade dedifferentiation, does not necessarily predict poor outcome. Therefore, OSG liposarcomas have better prognosis than liposarcoma in other soft-tissue locations, perhaps based on smaller size at presentation. Complete local excision and careful patient follow-up, without adjuvant therapy, appears to be the best treatment for OSG liposarcoma.

Allelic loss of tumor suppressor genes in ameloblastic tumors

Ameloblastoma is an odontogenic tumor with a variety of histologic appearances and an unpredictable biologic behavior. Little is known about allelic losses of tumor suppressor genes in ameloblastomas. This study surveyed DNA damage in ameloblastomas and correlated this with histologic sub-type and clinical outcome. There were 12 ameloblastomas (two peripheral, eight solid, and two unicystic) and three ameloblastic carcinoma studied for loss of heterozygosity of tumor suppressor genes on chromosomes 1p, 3p, 9p,10q, and 17p (L-myc, hOGG1, p16, pten, and p53). The frequency of allelic loss and the intratumoral heterogeneity were calculated. L-myc (71% frequency of allelic loss) and pten (62% frequency of allelic loss) had the most frequent allelic losses. Overall frequency of allelic loss and intratumoral heterogeneity were higher in mandibular and in unicystic tumors and lower in tumors that recurred/metastasized. The rate of allelic loss in the three carcinomas was similar to that seen in benign tumors. The frequency of allelic loss and intratumoral heterogeneity did not correlate with age, gender, histologic subtype, or prognosis. Since tumors that behaved aggressively did not harbor more allelic losses, it is likely that DNA damage in ameloblastomas and ameloblastic carcinomas is sporadic and cumulative. We conclude that other genetic or epigenetic mechanisms may be responsible for malignant behavior in ameloblastic carcinomas.

An immunohistochemical analysis of anti-amylase antibody reactivity in acinic cell adenocarcinoma.

In many salivary acinic cell adenocarcinomas, well-differentiated serous acinar-type cells may be few and inconspicuous. In these cases it may be difficult to distinguish acinic cell adenocarcinoma from other types of salivary gland neoplasms such as cystadenocarcinoma. The usefulness of antisalivary amylase antibody immunohistochemical staining as a diagnostic aid was assessed on paraffin-embedded tissue sections from 27 typical acinic cell adenocarcinomas. Only 4 of 27 tumors showed reactivity in tumor cells. We conclude that anti-amylase antibody is of limited value in the recognition of acinic cell adenocarcinoma when light morphologic features are insufficient for diagnosis.

Comparison of rapid diagnostic methodologies for chlamydia and gonorrhea in an urban adolescent population: A pilot study

PURPOSE To compare a combination DNA probe test which detects both N. gonorrhoeae (GC) and C. trachomatis (CT) to the current culture methodologies among a population of female adolescents at an urban teaching center. In addition, the probe test for CT was compared to a direct immunofluorescence test. METHODS All sexually active female adolescents between the ages of 13-21 years who sought care at an urban teaching center from June 1991 through November 1991 and who required testing for sexually transmitted diseases (STDs) were recruited for this study. RESULTS The probe test was demonstrated to be 66.6% sensitive and 94.9% specific when compared to tissue culture for CT and 50% sensitive and 98.2% specific when compared to culture for GC. We found an overall prevalence of 23.5% for CT and 3.5% for GC. CONCLUSIONS The two rapid diagnostic tests for CT evaluated in this study demonstrated similar sensitivities. However the probe test offers advantages in that it is easier to perform, skill at reading fluorescence is not required, and one specimen yields results for both CT and GC.

Giant Peripheral Ossifying Fibroma: A Case Report and Clinicopathologic Review of 10 Cases From the Literature

Peripheral ossifying fibroma (POF) is most often a self-limiting, sessile or pedunculated, gingival nodule that is believed to be a reactive rather than neoplastic pathologic process. The lesion is typically <2cm, however it has been recognized that some examples may grow quite large and may displace teeth. The mass-like clinical presentation and radiographic appearance of soft tissue calcification may lead to misclassification; however the histologic appearance is diagnostic. Giant POFs (GPOF) have been referred to in the literature by several other names (large, atypical, huge, gigantiform). The distinguishing characteristics of GPOFs and the factors that contribute to their growth have primarily been explored through case reports. We present a new case of POF that was giant and review 10 previously reported giant lesions, with focus on the clinical presentation, radiographic features, and outcome to explore the possibility that this represents a distinct clinical subset of lesion, with a unique set of features that warrant recognition for accurate diagnosis.