Max L. Goodman

Olfactory neuroblastoma—A clinical analysis of 17 cases

Seventeen patients with olfactory neuroblastoma seen at the Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary from 1941 to 1971 are presented. Data on age, sex, symptoms, physical, and roentgenographic findings, and therapeutic results are evaluated. A system of pretherapy staging is proposed in which for Group A, the tumor is limited to the nasal cavity; in group B, the tumor is localized to the nasal cavity and paranasal sinuses; and in group C, the tumor extends beyond the nasal cavity and paranasal sinuses. Olfactory neuroblastoma is a radioresponsive, and, to a limited extent, radiocurable tumor and varies in aggressiveness. Of 17 patients in this series, 13 or 76% were alive without disease following treatment by surgery, irradiation, and combination of these two methods. Uncontrolled primary lesions with or without metastases accounted for all therapeutic failures. A treatment policy for this disease is presented.

Solitary fibrous tumor of the nasal cavity and paranasal sinuses

We report two solitary fibrous tumors of the nasal cavity and paranasal sinuses that were histologically and immunohistochemically virtually identical to solitary fibrous tumors (fibrous mesotheliomas) of the pleura. One tumor arose in a 48-year-old woman and the other in a 45-year-old woman. Both patients presented with nasal symptoms, and both patients are alive without evidence of disease 6 months and 1 year after excision. The tumors had a disorganized or “patternless” arrangement of spindle cells in a collagenous background and prominent vascular channels of varying size. Immunoperoxidase stains on paraffin sections showed staining of the cells for vimentin only; there was no staining for keratin, S-100 protein, desmin, and actin. Both cases presented some degree of diagnostic difficulty and had to be distinguished from other spindle cell tumors of the nasal cavity and paranasal sinuses, such as hemangiopericytoma, angiofibroma, and fibrous histiocytoma.

Correlation of antineutrophil cytoplasmic antibodies with the extrarenal histopathology of Wegener's (pathergic) granulomatosis and related forms of vasculitis.

We studied the histologic findings from extrarenal biopsies (especially of the lung or upper respiratory tract) or autopsies of 68 patients who were tested for serum antineutrophil cytoplasmic antibodies (ANCAs). We used antigen-specific assays to detect antibodies against proteinase 3 (PR3) and myeloperoxidase (MPO), the two types of ANCAs of proven diagnostic value for the spectrum of diseases that includes Wegeners (pathergic) granulomatosis, microscopic polyarteritis (microscopic polyangiitis), Churg-Strauss syndrome, idiopathic necrotizing and crescentic glomerulonephritis, and their variants. Twenty-eight patients had antibodies to PR3 and 16 had antibodies to MPO; no patient had antibodies to both. All 44 patients with ANCAs had histologic evidence of this spectrum of diseases. Thirteen patients without histologic evidence of this spectrum of diseases had negative tests for ANCAs. There were no pathologic features that reliably identified patients with one or the other type of ANCA. Eighteen of 31 patients with lesions of Wegeners granulomatosis had antibodies to PR3, seven had antibodies to MPO, and six had neither. Three of four patients with necrotizing arteries without granulomas had anti-MPO antibodies, but similar lesions were seen, together with extravascular granulomas, in three patients with anti-PR3 antibodies. Of 16 patients with alveolar hemorrhage, nine had anti-PR3 and five had anti-MPO antibodies. Two patients diagnosed clinically as having Churg-Strauss syndrome had anti-MPO antibodies. In 16 of the 25 patients with ANCAs and a histologic diagnosis of Wegeners granulomatosis the diagnosis was made on the basis of extravascular granulomatous lesions alone, which argues against the requirement for vasculitis. Of six patients with negative tests for ANCAs and histologically diagnosed Wegeners granulomatosis, none had evidence of renal involvement. We conclude that in the appropriate clinical setting the presence of anti-PR3 or anti-MPO antibodies provides reliable evidence of the above spectrum of diseases, but that subclassification (to the extent this is possible) depends on the presence of distinctive clinical or pathologic features. In patients with negative tests for ANCAs, interpretation of clinical and histologic findings remains the only definitive method of diagnosis.

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.

Photon irradiation of unresectable carcinomas of salivary glands

This paper presents our experience and the local control rates of a group of patients with inoperable and unresectable lesions treated by photon irradiation from 1980 through 1989. The patient material consists of a total of 24 patients, 9 with carcinoma arising from the parotid gland and 15 with lesions in the minor salivary glands, mainly the oral cavity and oropharynx. The pathologic slides were reviewed and malignancy of various cell types confirmed. The 5-year actuarial local control of parotid gland lesions after photon irradiation was 100% and the survival rate was 65%. For the minor salivary gland lesions, the 5-year actuarial local control was 78% and the survival rate with or without disease was 93%. All lesions were irradiated by accelerated hyperfractionated photons (bid) with 1.6 Gy per fraction, intermixed with various boost techniques including electron beam, intraoral cone, interstitial implant, and/or submental photons for a total of 65-70 Gy. Most treatment failures of parotid cancer were due to distant metastases. The present series showed excellent local control and satisfactory survival of inoperable and unresectable salivary gland carcinomas after state-of-the-art photon irradiation, comparable to that achieved by neutron irradiation. The late complications were minimal. A controlled randomized trial may be indicated.

Management of malignancy of the temporal bone

An analysis was made of 31 patients with malignancy of the temporal bone treated at Massachusetts Eye and Ear Infirmary between 1959 and 1975. Treatment modalities included: partial temporal bone resection, subtotal temporal bone resection, total temporal bone resection, radical mas‐toidectomy followed by radiation therapy, radiation therapy alone, and chemotherapy. When tumor was limited to the external ear canal, partial temporal bone resection was effective in 10 out of 11 patients. When tumor involved the middle ear, subtotal temporal bone resection cured 3 out of 5 patients, and radical mastoidectomy followed by radiation therapy cured 5 out of 12 patients. Total temporal bone resection and chemotherapy failed to cure a patient each, but radiation therapy alone was successful in controlling tumor in one patient.

In situ carcinoma of the vocal cords.

Eighty‐one patients with preinvasive carcinoma of the vocal cord, seen over a thirteen year period, were reviewed histologically and analyzed for response to treatment. Sixty‐seven cases were clinically T1 and fourteen cases T2 (by virtue of extension of disease beyond the vocal cord). All patients had mobile cords. Histological criteria for in situ carcinoma and the spectrum of microscopic patterns are presented. Sixty‐nine patients were treated by external beam radiotherapy—the remainder by a variety of surgical procedures. Five and ten year actuarial, recurrence‐free rates 83% and 75%, respectively for the group treated by irradiation. No difference in local control was observed when analyzed for: 1) dose response, 2) initial extent of disease, 3) treatment plan, or 4) histologic diagnosis. Analysis of failures reveals that 15/17 patients with local failure were salvaged, eleven of these patients requiring laryngectomy. Time to appearance of recurrence ranged from seven to one hundred and seven months.

Immunohistologic diagnosis of orbital lymphoid infiltrates.

The distinction between benign and malignant lymphoid infiltrates of the orbit may be impossible on routine histopathologic sections. However, the detection of monotypic immunoglobulin is useful in distinguishing neoplastic from benign infiltrates. Since diagnostic frozen sections are often performed on biopsies of orbital masses to determine the adequacy of the biopsy and to provide a preliminary diagnosis, we stained additional frozen sections of 20 predominantly lymphoid infiltrates by an immunoperoxidase technique with antisera to immunoglobulin heavy and light chains. On routine sections, nine cases were malignant lymphoma, three were follicular hyperplasia, and eight (42%) were dense lymphocytic infiltrates of indeterminate nature. The nine lymphomas had monotypic immunoglobulin staining. The three histologically benign lesions had polytypic immunoglobulin. Six of the eight indeterminate lesions had monotypic immunoglobulin, supporting a diagnosis of lymphoma; two had polytypic staining. There was evidence of disseminated lymphoma at the time of diagnosis in five of nine patients with histologically malignant lesions and three of five with monoclonal indeterminate lesions for whom the information was available. Staining with monoclonal antibodies to T-cells revealed variable numbers of T-cells in all cases; their number and distribution did not distinguish benign from malignant lesions. The immunoperoxidase technique on frozen sections permits optimal use of small biopsy specimens for both morphologic and immunologic diagnosis. The majority of histologically indeterminate orbital lymphoid infiltrates were shown to be monoclonal.

Lingual tonsillectomy: A treatment for inflammatory lesions of the lingual tonsil†‡

Lingual tonsillectomy is a surgical treatment that is seldom performed because lingual tonsillitis is infrequently diagnosed. We have reviewed a group of patients with lingual tonsillitis or lingual tonsil hyperplasia who were treated with lingual tonsillectomy. Lingual tonsil lesions, the anatomy and histology of the lingual tonsil, and surgical approaches to lingual tonsillectomy are discussed.

Laryngeal tuberculosis as manifested in the decades 1963-1983

Laryngeal tuberculosis is usually a complication of pulmonary tuberculosis and the clinical patterns have changed in recent decades. To evaluate the changing patterns, we reviewed 15 patients seen at the Massachusetts Eye and Ear Infirmary over a 20‐year period and diagnosed as having laryngeal tuberculosis.