Adult rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study.

Twenty-seven cases of adult rhabdomyoma (ARM) of the head and neck are reported. The 20 male and seven female patients ranged in age from 33 to 80 years (median age, 60 years). Symptoms included airway obstruction and a mass within the mucosa or soft tissue. Median tumor size was 3.0 cm (range, 1.5 to 7.5 cm). Seven patients (26%) presented with multinodular tumors and one tumor was multicentric. Follow-up was available in 19 cases and ranged from 2 months to 18.5 years after diagnosis (median, 6.0 years). Lesions recurred locally in eight cases (42%) 2 to 11 years after diagnosis (median, 6 years). One recurrence was multicentric. Histologically, ARM was composed of closely packed, large polygonal cells having abundant, eosinophilic, granular, or vacuolated glycogen-rich cytoplasm with focal cross-striations. Immunohistochemical stains confirmed skeletal muscle differentiation; the majority of tumors stained for myoglobin (21 of 21 tumors), muscle-specific actin (21 of 21 tumors), and desmin (19 of 21 tumors). Focal or rare immunoreactivity for vimentin (six of 17 cases), alpha-smooth muscle actin (17 of 20 cases), S-100 protein (14 of 21 cases), and Leu-7 (10 of 20 cases) also was detected. Cytokeratin, epithelial membrane antigen, glial fibrillary acidic protein, and CD68 antigen (with KP1) were not found. The characteristic histology and immunophenotype distinguish ARM from other lesions with which it is frequently confused, including granular cell tumor, hibernoma, oncocytoma, and paraganglioma. The expression of alpha-smooth muscle actin has not been reported previously in ARM; its presence could reflect aberrant expression of smooth muscle actin in skeletal muscle or possibly be a recapitulation of early skeletal muscle embryogenesis.

Crystal-storing histiocytosis associated with lymphoplasmacytic neoplasms. Report of three cases mimicking adult rhabdomyoma.

Massive crystal deposition is rare in lymphoplasmacytic (LPc) or plasma cell neoplasms. We report three cases in which the accumulation of crystals in histiocytes closely reproduced the histologic features of adult rhabdomyoma. The patients, all female, aged 18, 77, and 78 years, presented with tumor of cervical lymph nodes (two cases) or the otolaryngic mucosa (two cases). In addition, two patients had monoclonal serum or urine immunoglobulin (IgM-kappa-1, unknown-1), and one had renal and bone marrow involvement on biopsy. This last patient died of acute renal failure at 5 months, another was alive without disease at 8 years, and the remaining one was lost to follow-up. Lymph nodes, mucosae, and kidney showed a neoplastic LPc infiltrate masked by sheets of large benign histiocytes containing sheaves of crystals. Paraffin-section immunohistochemistry demonstrated monoclonal staining of the LPc cells in all cases (IgM-kappa-2, IgA-kappa-1) and of the crystals (IgM-kappa) in one case. In all patients, the crystal-containing cells were positive for KP-1 (CD68), but not for desmin, muscle-specific actin, or myoglobin. These findings suggest that, in any case of adult rhabdomyoma in which the histologic findings are not typical, a crystal-storing histiocytosis should be ruled out: recognition of the atypical LPc component and the histiocytic immunophenotype of the crystal-storing cells will help prevent a serious misdiagnosis.

Pathologic findings in the labyrinthine segment of the facial nerve in a case of facial paralysis.

The histopathologic findings for a patient with acute facial paralysis caused by herpes zoster oticus who obtained no return of active facial function after 1 year are presented. All imaging studies were nondiagnostic. Biopsy of the labyrinthine segment was performed. Histopathologic analysis showed a sharp line of demarcation between sclerotic nerve proximal to and necrotic nerve distal to the meatal foramen area of the fallopian canal. This finding is consistent with observations that the lesion producing Bell's palsy and herpes zoster oticus usually is situated at the meatal foramen.

Neurothekeoma (peripheral nerve sheath myxoma) of the external auditory canal.

A patient who presented with an isolated soft-tissue tumor in the lateral portion of the external auditory canal, which after removal was diagnosed histopathologically as a peripheral nerve sheath neurothekeoma, is presented. Peripheral nerve sheath myxomas (neurothekeomas or perineural myxomas) have been reported to occur on the central area of the face, arms and shoulders. This is the first case in which a tumor of this type has been reported to develop in the external auditory canal. The distinguishing histopathological characteristics and the differential diagnosis of this tumor are discussed.

Predominance of squamous cell carcinoma and rarity of adenocarcinoma of maxillary sinus among Japanese.

The reasons for the prevailing predominance of maxillary sinus squamous cell carcinoma and the rarity of maxillary sinus adenocarcinoma among Japanese were investigated in a cooperative study at two institutions. There was little difference in the histological diagnostic criteria. It is hypothesized that the predominance of squamous cell carcinoma and the rarity of adenocarcinoma of the maxillary sinus can be related to the extensive use of softwood in the Japanese furniture industry, to the high prevalence of chronic sinusitis among Japanese in the past or to the popularity of cigarette smoking among Japanese.

Moderately differentiated neuroendocrine carcinoma of the larynx. A clinicopathologic study of 54 cases.

Fifty-four cases of primary laryngeal moderately differentiated neuroendocrine carcinoma from the Armed Forces Institute of Pathology Otolaryngic Tumor Registry (AFIP-OTR) are reported. The tumors most often present in men in their sixth and seventh decades of life and are heralded by an array of symptoms, the most frequent being hoarseness. The primary site was most often the supraglottic larynx. The investigation has included light-microscopic, histochemical, immunocytochemical, and electron microscopic analyses which support expression of both neuroendocrine and epithelial differentiation. Conservative surgery alone can be utilized if early identification of the tumor and complete surgical removal are assured. The follow-up of the patients reveals 62% as remaining tumor-free after surgical extirpation over periods ranging from 1 month to 16 years (median: 3 years, 9 months). Factors adversely affecting prognosis include metastatic disease at initial presentation, incomplete surgical removal, and vascular or lymphatic invasion. There was no correlation between tumor size, morphologic pattern, mitoses or necrosis, and survival. Sixty-eight percent of the patients gave a history of long-term cigarette smoking. The classification and pathogenesis of these neoplasms remains the focus of much speculation. They are suggested as arising from the cells of the dispersed neuroendocrine system (DNES). However, a more uniform and descriptive nomenclature is necessary. This study resolves this and other issues along with a presentation of clinicopathologic data of the tumor entity.

Nasopharyngeal papillary adenocarcinoma. A clinicopathologic study of a low-grade carcinoma.

We present the clinicopathologic features of nine cases of a primary nasopharyngeal (NP) papillary adenocarcinoma. Patients ranged in age from 11 to 64 years (median, 37). The ratio of male to female patients was 5:4. They most frequently presented with airway obstruction. Histomorphologic evaluation showed an invasive carcinoma with papillary and glandular features. The tumor displayed transition from normal surface epithelium to neoplasm. Cytologically it was composed of moderately pleomorphic, columnar, and pseudostratified cells with eosinophilic cytoplasm. The nuclei were round to oval and had vesicular or optically clear chromatin. Histochemical analysis confirmed production of epithelial mucin. Immunohistochemical study demonstrated diffuse keratin and epithelial membrane antigen reactivity and focal carcinoembryonic antigen reactivity. No immunoreactivity was seen with S-100 protein, glial fibrillary acidic protein, or thyroglobulin. Taken together, these findings indicate derivation from the nasopharyngeal surface epithelium and help to differentiate these tumors from seromucous gland lesions and metastatic papillary thyroid carcinoma. The biologic potential was that of a low-grade malignant tumor. Simple and complete surgical excision was the treatment of choice. All patients are presently alive and well, free of recurrent or metastatic tumor (median follow-up, 6 years, 9 months).

Spindle-cell carcinoma of the aerodigestive tract. An immunohistochemical analysis of 21 cases.

Immunohistochemical analysis of 21 prototypic mucosal spindle-cell carcinomas of the aerodigestive tract was performed at the Armed Forces Institute of Pathology (AFIP) to establish the usefulness of selected immunohistochemical markers in distinguishing spindle-cell carcinoma from other mucosal spindle-cell neoplasms. Immunoreactive keratin could be demonstrated in only 13/21 (62%) of cases. Coexpression of keratin and vimentin was demonstrated in 10/17 (59%) of the tumors evaluated for both of these intermediate filaments. All spindle-cell carcinomas lacked S100 protein, which is an immunoreactivity we would expect to find in spindle-cell malignant melanoma, one of the principal considerations in a differential diagnosis. Both alpha-1-antitrypsin (AAT) and alpha-1-antichymotrypsin (ACT) were demonstrated in the tumor cells in all cases. However, albumin had a similar distribution in the tumors, which suggested that passive uptake was a serious confusing factor. The results of this study indicate that AAT and ACT are unreliable markers for distinguishing spindle-cell carcinomas from malignant fibrous histiocytomas.

Chondrosarcoma of the temporal bone. Diagnosis and treatment of 13 cases and review of the literature.

Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with multiple sclerosis, glomus jugulare tumors, meningioma, and chordomas. The cranial nerve palsies frequently observed with the tumors are related to the anatomic locations of the tumors. Thirteen patients with this entity are presented and the eleven other cases in the literature are reviewed. Histologically the tumors are low grade and exhibit myxoid features. The myxoid features must be differentiated from chordoma and chondroid chordoma. The tumor locations preclude surgical excision and conventional radiation therapy can cause unacceptable neurologic sequelae. Proton beam therapy has been effective in short-term results and appears capable of avoiding serious neurologic side effects.

Cystic chondromalacia (endochondral pseudocyst) of the auricle.

We reviewed and studied the histologic features of 23 cases of pseudocysts of auricular cartilage. Our aim was to determine and describe the histologic spectrum of this condition and to delineate the histologic differential diagnostic points. Lesions for which this condition has been mistaken include relapsing polychondritis, chondrodermatitis nodularis helicis, traumatic perichondritis, hemangioma, chondroma, angiosarcoma, and chondrosarcoma. The pathologist who is familiar with the histologic patterns of this condition usually can recognize it readily. When biopsy specimens are small, and the diagnosis is uncertain, clinical features can often suggest the correct diagnosis.

Laryngeal dysgenesis.

A case of a previously undescribed anomaly of the larynx is presented and offers insight and support of current concepts of fetal laryngeal development. The patient possessed unilateral absence of true and false vocal cords, laryngeal ventricle, and saccule. Current concepts of embryological development would place the development of this anomaly and most of the patient's other multiple anomalies in the period of the 6th to 9th weeks of fetal life. This report lends substantiation to the stages of laryngeal development by demonstrating an aberration in the normal sequential development. Additionally, the time period of this maldevelopment is suggested by the concurrence of multiple other anomalies. Aspects of laryngeal embryology relevant to laryngeal maldevelopment are reviewed.

Paraglottic laryngitis in association with epiglottitis.

Infection of the supraglottic larynx is a well-recognized clinical entity in both adults and children. The anatomical relationships of the various components of the larynx have been studied extensively, especially as they relate to the spread of malignant disease. However, the relationship of these spaces and compartments in inflammatory disease has not been heretofore appreciated. This report describes for the first time histologic evidence that infection of the supraglottic larynx may spread to the paraglottic space, thus causing compromise of the glottic region. This potential carries significant implications for planning the appropriate therapeutic measures.

Polypoid lymphangioma of the tonsil: report of two cases and review of the literature.

Benign tumours of the tonsils are uncommon. Among them, lymphangiomas are rare, and only six well-documented cases have been previously reported. We present two additional cases with review of the literature.

Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses A clinicopathologic study of 20 cases.

Twenty cases of a unique type of sinonasal tract neoplasm with combined histologic features of carcinosarcoma and teratoma are described and discussed. The term "teratocarcinosarcoma" is proposed and justified. Patients were adults (age range, 18-79 years; median age, 60 years). The variegated histologic components are illustrated and differences from gonadal germ cell neoplasms are delineated. This neoplastic entity is clearly malignant, with 60% of patients not surviving beyond 3 years (average survival, 1.7 years) following diagnosis, regardless of type of therapy. Aggressive therapy (combined surgery and irradiation) seems justified, however, since 40% of patients survived 3 years or longer with no current evidence of neoplasm (average follow-up, 6.1 years).

Rhabdomyosarcoma of the larynx: a case report and review of the literature.

Rhabdomyosarcoma is rarely diagnosed in the larynx. A case report and five patients from the Otolaryngic Pathologic Files of the Armed Forces Institute of Pathology are added to the world literature. Current modes of treating Stage III rhabdomyosarcomas are reviewed. The recent literature suggests that certain of these neoplasms may be treated successfully without radical surgery.