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D K Heffner

Publications and source records attributed to D K Heffner.

At least 37 records · Page 2Linked to original sources

Liposarcomas of the larynx and hypopharynx: a clinicopathologic study of eight new cases and a review of the literature.

Laryngeal and hypopharyngeal liposarcomas were studied in seven men and one woman. Patient age ranged from 25 to 81 years, with a median of 64 years. Symptoms included dysphagia, airway obstruction, and the sensation of a foreign body in the back of the throat. Histologically, seven of the tumors were of the biologically favorable types, either representing well-differentiated (lipoma-like) liposarcomas or myxoid liposarcomas. One tumor was a pleomorphic liposarcoma. Six of the eight patients had one or more episodes of recurrent tumor. Surgery is the treatment of choice and can include conservative (organ-sparing) procedures. However, to eradicate the tumor completely and thereby prevent recurrent disease, open surgical approaches (i.e., lateral pharyngotomy), rather than endoscopic techniques should be employed. The morbidity rate for laryngeal and hypopharyngeal liposarcomas is high because these tumors tend to recur over extended periods of time. In this study, however, there were no instances of metastatic disease, and no deaths were attributed to liposarcoma. Prospectively, laryngeal and hypopharyngeal well-differentiated (lipoma-like) liposarcoma is a difficult clinical and histopathologic diagnosis to establish. Often, this diagnosis is made only after one or more episodes of recurrent disease.

Adult↗

Epidermoid cyst (cholesteatoma) and cholesterol granuloma of the temporal bone and epidermoid cysts affecting the brain.

Five cases of petrous apex epidermoid cysts, six cases of petrous apex cholesterol granuloma cysts, and seven cases of intradural cerebellopontine angle (CPA) or intracranial epidermoid cysts are reviewed. Petrous epidermoid cysts (cholesteatomas) and cholesterol granuloma cysts cannot be differentiated definitely from each other on CT scans. On MR imaging, epidermoid cysts exhibit long T1 and long T2 characteristics and can be differentiated from cholesterol granuloma cysts, which exhibit short T1 and long T2 characteristics. Intradural epidermoid cysts involving the CPA or other portions of the brain, also demonstrate long T1 and long T2 characteristics on MR scans. Intradural epidermoid cysts exhibit curvilinear areas of higher intensity on T1-weighted images, which were best evaluated on T1-weighted MR images obtained with more averages (6 to 8).

Adolescent↗

Fetal rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study of 24 cases.

Twenty-four fetal rhabdomyomas (FRMs) of the head and neck occurring in 16 male and seven female patients (sex unknown in one), ranging from 3 days to 58 years of age (median, 4.5 years) are reported. Ten patients (42%) were < or = 1 year old, six lesions (25%) were congenital, and 11 lesions (46%) occurred in patients > or = 15 years of age. The median tumor size was 3.0 cm (range, 1.0 to 12.5 cm). The FRMs presented as well-defined, solitary masses arising within the soft tissue or mucosa (2:1) of the head and neck. The median follow-up in 15 cases was 48 months (range, 2 months to 52 years) after diagnosis. With the exception of one patient with a local recurrence, all patients were either alive and well or dead of unrelated causes. Eight cases, regarded as "classic" FRM, consisted predominantly of bland, primitive spindled cells associated with delicate, elongated skeletal muscle cells reminiscent of fetal myotubules that were haphazardly arranged in an abundant fibromyxoid stroma. The remaining 16 cases, designated as "intermediate" FRM, displayed both a greater degree and a greater number of cells with skeletal muscle differentiation as well as a variety of distinctive cytologic and architectural features. These included the presence of large, ganglion cell-like rhabdomyoblasts with vesicular nuclei and prominent nucleoli, interlacing ribbon or strap-like rhabdomyoblasts with deeply acidophilic cytoplasm, broad bundles of more delicate spindled rhabdomyoblasts arranged in fascicles simulating smooth muscle, an occasional plexiform pattern with infiltration of adipose tissue and skeletal muscle, focal intimate association with peripheral nerves, and rare areas of fibroblastic proliferation. Mitoses were not found in 19 of the 24 FRM cases, but in five tumors there were 1 to 14 mitoses/50 high-power fields. Marked nuclear atypia, anaplasia, and a "cambium layer" were uniformly absent. The FRMs typically stained for myoglobin, desmin, and muscle-specific actin with focal or rare staining for vimentin, smooth muscle actin, S-100 protein, glial fibrillary acidic protein, and Leu-7. Cytokeratin, epithelial membrane antigen, and CD68 antigen (with KP1) were not detected. This study expands on previous reports of FRM and demonstrates that it has both a broader age range and histologic spectrum than previously recognized. The mitotic rates of FRM as well as certain histologic features overlap with rhabdomyosarcoma; the lack of marked nuclear atypia is an important distinguishing feature.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

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.

Adult↗

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.

Adult↗

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.

Adolescent↗

Sinonasal myxomas and fibromyxomas in children.

Childhood sinonasal myxomas are frequently misdiagnosed and improperly treated, with consequent adverse results for the patient. Reasons for this are briefly discussed using illustrative case reports. When both clinicians and their consulting pathologists are familiar with this lesion, the best therapeutic outcome is more likely.

Biopsy↗

Sinonasal fibrosarcomas, malignant schwannomas, and "Triton" tumors. A clinicopathologic study of 67 cases.

BACKGROUND: Sinonasal fibrosarcomatous neoplasms are uncommon tumors and there are no previous studies of a large number of such cases. The clinical and histologic features of 67 fibrosarcomatous neoplasms of the nasal cavity and paranasal sinuses are reported. METHODS: Multiple clinical and histologic parameters (including immunostain results) were analyzed to characterize the features important for histologic recognition of the tumors and for correlation with patient outcomes. RESULTS: Some tumors could be classified as malignant schwannomas or malignant "Triton" tumors, but their behavior was similar to that of the fibrosarcomas. Histologically, most tumors were very low-grade malignant neoplasms; however, 22% of patients died of their tumors. Factors that correlated with death were mitotic rate (greater than 4 mitoses per 50 high-power fields), increased tumor cellularity, and male sex. CONCLUSIONS: Many tumors originally were diagnosed as benign (by others), probably causing initial undertreatment of some patients. Proper recognition and histologic evaluation of the tumor are important to ensure the best therapy and optimal patient survival.

Adolescent↗

Pitfalls in the histopathologic diagnosis of pyogenic granuloma.

The term pyogenic granuloma (PG) is a misnomer. The histopathologic appearance is fairly characteristic, the lesion being in fact a lobular capillary hemangioma. The recognition of PG as a clinically polypoid or exophytic, circumscribed lesion is of importance to both the clinician and the pathologist, as this feature distinguishes PG from most malignant vascular tumors. Although PG may be multiple, especially on the skin, and necrosis is not uncommon, invasion of adjacent structures is not seen. Diagnostic pitfalls occur histologically when the characteristic lobular configuration of PG is not recognized because of a solid growth pattern of endothelial proliferation, the presence of brisk mitosis, intervascular stromal fibrosis or spindle cell proliferation, and occasionally an epithelioid appearance of endothelial cells. The different vascular neoplasms that may be confused with PG are discussed, and the clinical and histopathologic features of PG are emphasized.

Angiolymphoid Hyperplasia with Eosinophilia↗

Granular cell tumors of the trachea.

In contrast to the relative frequency of granular cell tumors (GCT) in the larynx and bronchi, the occurrence of these tumors in the trachea is rare. A 50-year review of the English-language literature disclosed only 24 described cases of tracheal GCT. This report reviews the clinicopathologic data from those 24 cases, along with the data from 2 cases obtained via a personal communication and the data from 4 previously unpublished cases obtained from a 30-year review of the Armed Forces Institute of Pathology archives. Tracheal resection was the predominant mode of therapy and often was performed as a salvage procedure for failed endoscopic excisions. Recommendations for a more uniform approach to surgical management are provided.

Adult↗

Oncocytic metaplasia of the pharynx.

Oncocytic metaplasia of the pharynx has been infrequently described, with only two previous cases in the literature. With the advent of panendoscopy during the last decade, however, a better understanding of this histopathologic diagnosis is desirable. Thirty-three cases are reviewed, with thirty occurring in the nasopharynx. This was most commonly discovered as an unrelated finding during endoscopic evaluation of a head and neck mass or malignancy in twenty-two patients, either histologically after random biopsy or after biopsy of small but visible lesions. An additional eight cases manifested otitis media or eustachian tube dysfunction. The histology, terminology, and benign clinical nature of these lesions are discussed.

Aged↗

Leiomyosarcoma of the sinonasal tract. A clinicopathologic study of nine cases.

The clinicopathologic features of nine cases of sinonasal tract leiomyosarcoma (SNTL) referred to the Armed Forces Institute of Pathology, Washington, DC, during the period from 1970 through 1988 are described. This report represents the largest study to date on SNTL, and our results are compared with the 21 previously reported cases of SNTL. Of the nine cases described, patients ranged in age from 22 to 86 years (mean, 55 years). The most frequent clinical presentation was nasal obstruction unilaterally. The neoplasms were limited solely to the nasal cavity in four cases (44%) and involved both the nasal cavity and paranasal sinuses in the remaining five cases. Light microscopic, immunocytochemical, and ultrastructural features served to characterize these tumors as malignant neoplasms of smooth-muscle origin. In contrast with previous studies, immunocytochemistry was employed to differentiate SNTL from other spindle cell malignancies of the region, using newly available monoclonal antibodies to smooth-muscle antigens. Treatment was surgical. Radiotherapy or chemotherapy did not appear to affect the progression of the disease. Furthermore, no relationship was found between the aggressiveness of SNTL and morphologic parameters (eg, mitotic count and tumor size). Instead, prognosis was dependent on the distribution of disease at presentation. Of all 30 patients with SNTL described to date, 10 had the neoplasm confined solely to the nasal cavity. The 10 neoplasms did not recur. We conclude that SNTL may best be regarded as a locally aggressive neoplasm with only limited metastatic potential and that it could be curable by complete surgical excision.

Adult↗

Teflonomas of the larynx and neck.

Intracordal fluorocarbon (Teflon, Mentor O and O Inc, Norwell, MA) injection has been used for decades to correct paralytic dysphonia, a result of unilateral laryngeal paralysis. Infrequently, the Teflon extravasates and infiltrates into the soft tissues of the neck and larynx producing a mass that clinically simulates a malignant lesion. We report eight cases of so-called "Telonomas" of the larynx and neck that have been identified in the files of the Armed Forces Institute of Pathology Otolaryngic Tumor Registry and from the Cytopathology Service at the George Washington University Medical Center. Patients ranged in age from 31 to 72 years. Vocal cord paralysis, treated by Teflon injection, was caused by primary laryngeal carcinoma or metastatic carcinoma (breast, lung) involving the recurrent laryngeal nerve, surgical trauma to the recurrent laryngeal nerve, or postviral neuritis. Subsequent symptomatology, related to extravasation of the Teflon with a resulting "Teflonoma", included a neck mass or persistent hoarseness. Infrequently, there was associated airway obstruction or voice changes. Diagnosis was made by fine needle aspiration or by excision of the suspicious mass and subsequent identification of a foreign body granulomatous reaction with associated birefringent material. Infrared absorption spectrophotometry identified the foreign material as a fluorocarbon which was further substantiated by scanning electron microscopy and energy dispersive x-ray analysis. Surgical removal of the mass alleviated all symptoms.

Adult↗

Haemangioma in a cervical lymph node.

Haemangiomas of the head and neck are mainly cutaneous or mucosal; rarely, they may be found in bone or soft tissue. Haemangiomas of lymph nodes are extremely rare; here we present such a case in a cervical lymph node. This is, to the best of our knowledge, only the second report of such an entity.

Adolescent↗