Vincent J. Hyams

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 spindel-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

Low-grade adenocarcinoma of the nasal cavity and paranasal sinuses.

The pathologic features and the behavior of 50 cases of adenocarcinoma (excluding adenoid cystic carcinoma and mucoepidermoid carcinoma) of the sinonasal tract are presented. The cases were divided on histologic grounds into 23 low‐grade and 27 high‐grade neoplams. The low grade lesions had a well developed glandular pattern throughout, very uniform nuclei, and minimal mitotic activity. The high grade tumors had a less uniform glandular pattern, commonly with solid or sheet‐like areas, manifested nuclear pleomorphism, and generally had a higher mitotic rate. The low‐grade group had a prognosis markedly better than the high‐grade group. Since the literature tends to consider all sinonasal adenocarcinomas as relentlessly progressive neoplasms with poor prognosis, it is important to recognize this category of low grade neoplasm in order that treatment and prognostication can be better related to their behavior.

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.

Primary anaplastic small cell (oat cell) carcinoma of the larynx. Review of the literature and report of 18 cases

Anaplastic small cell (oat cell) carcinoma is a neoplasm commonly arising in the lungs. However, it may also occur, though rarely, in the larynx. A series of 43 cases is presented (ten cases from the Armed Forces Institute of Pathology, eight from the Department of Otolaryngology of the Padua University, and 25 from the literature). The tumor often presents in the sixth and seventh decades of life and appears to be highly aggressive, and metastases develop early. The most common presenting symptom is hoarseness. As in pulmonary small cell carcinoma, prognosis is poor and does not seem to depend upon therapeutic modalities, tumor location or the extent of initial local disease. The tumor seems to derive from the Kulchitsky cell present not only in the bronchial mucosa but also in the laryngeal lining. Like pulmonary anaplastic small cell carcinoma, small cell carcinoma of the larynx should be treated with systemic chemotherapy and radiotherapy. The association of small cell carcinoma with squamous carcinoma of the larynx is also reported and problems connected with the histogenesis of this mixed tumor are discussed.

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).

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.

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).

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 histocytes 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 (fgM-k-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 followup. Lymph nodes, mucosae, and kidney showed a neoplastic LPc infiltrate masked by sheets of large benign histiocytes containing sheaves of crystals. Paraffinsection immunohistochemistry demonstrated monoclonal staining of the LPc cells in all cases (fgM-k-2, IgA-k-1) and of the crystals (IgM-K) in one case. In all patients, the crystal-containing cells were positive for KP-1 (CD68), but not for desmin, muscle-specific action, 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.

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.

Mucoepidermoid-Adenosquamous Carcinoma of the Larynx and Hypopharynx: A Report of 21 Cases and a Review of the Literature

Twenty-one cases of mucoepidermoid-adenosquamous carcinoma (MEC/ASC) of the larynx and hypopharynx were collected and reviewed from the Armed Forces Institute of Pathology tumor registry from 1945 to 1979. This is the largest series of such lesions in this location reported to date. In addition, the literature was reviewed and 32 previous cases noted. The overall five-year survival was 77%, although low-grade mucoepidermoid carcinoma had a better survival than did adenosquamous—high-grade mucoepidermoid carcinoma.