Dennis K. Heffner

Spindle cell (sarcomatoid) carcinomas of the larynx: A clinicopathologic study of 187 cases

Laryngeal spindle cell (sarcomatoid) carcinomas are uncommon tumors, frequently misdiagnosed as reactive lesions or mesenchymal malignancies. The records of 187 patients with tumors diagnosed as laryngeal spindle cell (sarcomatoid) carcinoma were retrieved from the files of the Otorhinolaryngic Tumor Registry of the Armed Forces Institute of Pathology. There were 174 men and 13 women, 35–92 years of age (average, 65.6 years). Nearly all patients experienced hoarseness (n = 165 [88%] patients) for a mean duration of 11.0 months. Patients admitted to smoking (n = 162 [87%] patients) and/or alcohol use (n = 90 [48%] patients). Most tumors were glottic (n = 132 [71%]), T1 (n = 111 [59%]), 1 and polypoid (n = 185 [99%]), with a mean tumor size of 1.8 cm. Histologically, squamous cell carcinoma (n = 157 [84%]) was noted, ulcerated, and blended with the spindle cell component, which was most frequently arranged in a storiform pattern (n = 92 [49%] tumors). Foci of benign or malignant cartilage and/or bone (n = 13 [7%]) were noted in the spindle cell component. All patients were treated with surgery (n = 90 [48%] patients) or surgery with radiation (n = 97 [52%] patients). Recurrences developed in 85 (45%) patients. Overall, T1 glottic tumors managed by complete surgical eradication had the best outcome (mean follow-up, 7.8 years).

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.

Aggressive psammomatoid ossifying fibromas of the sinonasal region. A clinicopathologic study of a distinct group of fibro‐osseous lesions

Background. Psammomatoid ossifying fibromas represent a unique subset of fibro‐osseous lesions of the sinonasal tract. They have distinctive histomorphologic features and a tendency toward locally aggressive behavior, including invasion and destruction of adjacent anatomic structures.

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.

Synchronous tumors arising in a single major salivary gland

Multiple separate tumors arising in the major salivary glands are distinctly unusual. The records on 25 patients with synchronous unilateral salivary gland tumors on file at St Louis University and at the Armed Forces Institute of Pathology were reviewed with similar cases in the literature. Bilateral tumors arose more frequently than multiple unilateral ones. The most common tumors with bilateral synchronous or metachronous development are the Warthin tumors, with more than 100 reported cases; the mixed tumor with at least 34 cases; and the acinic cell carcinoma, with 12 reported cases. The Warthin tumor also was the most likely tumor to present with multifocal unilateral involvement with 29 tumors having three or more separate foci of tumor. The next most common tumor combinations are a Warthin tumor and a mixed tumor (20 cases), the multifocal membranous basal cell adenoma (12 cases), and multiple oncocytomas (nine cases). Numerous other tumor combinations were found covering the range of salivary gland neoplasia, however one of the tumors usually was a mixed tumor or a Warthin tumor.

Ossifying fibromyxoid tumour (of soft parts) of the head and neck: A clinicopathological and immunohistochemical study of nine cases

Ossifying fibromyxoid tumour (OFT) is a recently described, mesenchymal neoplasm originally defined as a borderline or low-grade malignant lesion. Prior reports of OFT characterize it as a slow growing lesion with a propensity to occur in both the upper and lower extremities. Most OFTs have occurred within the deep subcutis or skeletal muscle. We report nine cases which arose in the head and neck region. Six of the nine tumours were classified as ossifying variants of OFT while two were non-ossifying variants that lacked a discernable shell of lamellar bone. One tumour was classified as a malignant OFT. Seven lesions occurred in a subcutaneous site while two lesions occurred intraorally beneath the gingival and palatal mucosa. The OFTs occurred in six men and three women (age range of 29-75 years). The tumours had histological features compatible with previously described OFTs and consisted of lobulated nests of small, cytologically bland round cells (with the exception of one malignant OFT), with a myxoid to hyalinized stroma and were surrounded in part by dense fibrous connective tissue. Six cases had an incomplete rim of lamellar bone with occasional perpendicularly oriented spicules of bone. Five lesions were immunostained. S-100 protein, neuron specific enolase, and Leu-7 were found in three out of five tumours. Glial fibrillary acidic protein, smooth muscle actin (SMA), and muscle specific actin (MSA) were detected in two out of five lesions, although staining for SMA and MSA was weak in reactivity. Staining for vimentin was strongly positive in all five cases tested. The tumours were not reactive with antibodies directed against cytokeratin, epithelial membrane antigen or neurofilament protein. Follow-up information, available in eight cases, revealed multiple local recurrences in the one tumour believed to be a malignant OFT. The histogenesis of these tumours is uncertain, although the preponderance of evidence suggests a Schwann cell origin.

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).