Karl H. Perzin

Adenoid cystic carcinomas arising in salivary glands. A correlation of histologic features and clinical course

62 cases of adenoid cystic carcinoma (ACC) arising in major and minor salivary glands were studied. Factors which appeared to influence the clinical course included: 1) histologic pattern, 2) presence or absence of tumor on the surgical lines of excision, 3) site, 4) size of primary lesion, 5) presence or absence of tumor in lymph nodes, and 6) degree of cellular atypia. On histologic examination, these neoplasms were classified according to their predominant histologic pattern (tubular, cribriform or solid). Recurrences have been seen in 59% of patients with ACC demonstrating a predominantly “tubular” pattern, as compared to 89% for the “cribriform” lesions and 100% for the “solid” neoplasms. Of patients who eventually died of tumor, those having “tubular” predominant lesions had the longest course (average 9 years before death, in contrast to 8 years for the “cribriform” and 5 years for the “solid” tumors). Our findings suggest that the “tubular” predominant pattern has the best prognosis and represents the best differentiated histologic form of ACC. In contrast, the “solid” pattern is the least differentiated and is associated with the worst prognosis. The predominantly “cribriform” lesions appear to lie between the other two forms both clinically and histologically.

Mucoepidermoid carcinoma of salivary gland origin. Classification, clinical‐pathologic correlation, and results of treatment

Sixty patients with mucoepidermoid carcinoma of salivary gland origin were studied. The pathologic findings and the clinical course were examined separately and then correlated. The tumors are classified as either well, moderately, or poorly differentiated. The criteria used to classify the lesions are discussed, and pathologic features are illustrated. The most important factors in the prognosis are: 1. degree of histologic differentiation, and 2. presence or absence of tumor on the lines of surgical excision. Recurrences correlated with the degree of histologic differentiation. Only one of 31 patients (3%) had a recurrence when careful examination of the surgical specimen failed to demonstrate tumor on the lines of resection. In contrast, 19 of 28 patients (68%) with tumor on the surgical margin had a recurrence. The other 9 patients had no recurrence, having been given immediate subsequent treatment. Recommendations made for therapy are based on the histologic grade of the tumor and on examination of surgical lines of resection.

Malignant mixed tumors arising in salivary glands. I. Carcinomas arising in benign mixed tumors: a clinicopathologic study.

Forty‐seven cases of malignant mixed tumor (MMT) arising in major and minor salivary glands are presented. By definition, all these lesions contained both a benign mixed tumor (BMT) as well as a malignant neoplasm, usually a poorly differentiated carcinoma. In some cases, the carcinoma developed in a previously untreated salivary gland mass which was known to have been present for many years, others evolved in a recurrent previously resected BMT and still others originated in a previously undetected BMT. Adverse prognostic factors included: carcinomatous involvement of the resection lines, perineural invasion, metastases in lymph nodes, and origin in a major salivary gland. Lesions arising in the palate had a better prognosis as compared to major salivary gland tumors. Some patients died of locally uncontrollable tumor and others because of metastatic disease. Local recurrences of MMT were seen frequently; 18 patients (38%) had a total of 32 local recurrences. Of patients followed five years or more, seven (35%) had died of tumor, 11 (55%) had no evidence of disease, and two (10%) had died of other causes. Prolonged followup is required because recurrences and death from tumor may be seen many years following the diagnosis of MMT. Recommendations for therapy are made. Criteria for differentiating MMT from recurrent BMT and from cylindromatous carcinoma are discussed.

Carcinoma arising in median ectopic thyroid (including thyroglossal duct tissue)

Carcinoma may arise in ectopic thyroid tissue. In this report, seven cases of papillary carcinoma found in association with median ectopic thyroid rests are discussed. Six patients presented with midline lesions in the thyrohyoid space. These were diagnosed clinically as thyroglossal duct cysts. The seventh lesion was described as a mass in the region of the submandibular salivary gland. All seven patients were treated initially with local resections. Ectopic apparently normal thyroid follicles were demonstrated in all seven specimens; in four, a thyroglossal duct cyst was also recognized. A separate microscopic focus of papillary carcinoma was identified in the thyroid gland in one of the three individuals who subsequently underwent total thyroidectomy. None of the seven patients, followed for from 6 months to 15 years, has had clinical evidence of recurrent disease. The differentiation between a carcinoma arising in median ectopic thyroid tissue and a metastatic papillary carcinoma from an occult primary in the main thyroid gland is discussed.

Non-epithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: A clinicopathologic study. II. Osseous and fibro-osseous lesions, including osteoma, fibrous dysplasia, ossifying fibroma, osteoblastoma, giant cell tumor, and osteosarcoma

In a study of non‐epithelial tumors involving the nasal cavity, paranasal sinuses, and nasopharynx, 256 lesions, 156 benign and 100 malignant, were identified. Of these, 64 were osseous and fibro‐osseous lesions. Included in this group were osteomas, fibrous dysplasia, ossifying fibromas, benign osteoblastoma, giant cell tumors, and osteosarcomas. The problem of diagnostic terminology and categorization in this group of lesions is discussed. For each type of lesion, clinical features, histologic findings, and results of treatment are presented and clinicopathologic correlations are made.

Adenomatous and carcinomatous changes in hamartomatous polyps of the small intestine (Peutz‐Jeghers syndrome): Report of a case and review of the literature

The authors report the unique case of a patient with the Peutz‐Jeghers (P‐J) syndrome who had multiple small bowel hamartomatous polyps, some of which also showed adenomatous and carcinomatous changes. Over the course of 30 years, over 100 small bowel and colonic polyps were resected; all histologically demonstrated the typical features seen in hamartomatous polyps. Several jejunoileal polyps excised during the last few months of the patients life showed not only hamartomatous features but also adenomatous epithelium. As far as the authors can determine, this is the first documented case of adenomatous changes found within a small bowel hamartomatous polyp. In addition, at least one duodenal polyp demonstrated areas of hamartoma, adenoma, and in situ carcinoma. The literature on the occurrence of gastrointestinal adenomas and carcinomas in patients with the P‐J syndrome is reviewed, and the relationship of hamartomas, adenomas, and carcinomas of the gastrointestinal tract is discussed.

Lobular neoplasia : Long term risk of breast cancer and relation to other factors

Lobular neoplasia (LN), also known as lobular carcinoma in situ, is an incidental histologic finding in tissue removed at breast surgery. Patients with LN are known to be predisposed to develop invasive or intraductal carcinoma (CA). This study investigates factors that influence the cancer risk in LN patients.

Acinic cell carcinomas arising in salivary glands: a clinicopathologic study.

Fifty‐one cases of acinic cell carcinoma (ACC) arising in major and minor salivary glands are reported (primary sites: parotid 37, submandibular, six, and oral cavity, eight.) These lesions usually produced painless slowly growing masses. Of 49 patients with follow‐up information, 17 (35%) developed local recurrences following original surgical resection, and two (4%) had locally persistent disease. At last follow‐up, 27 patients (55%) never had a recurrence, seven (14%) had experienced local recurrences but the tumors were apparently controlled by further surgical excisions, five (10%) were living with persistent disease, and 10 (20%) had died (one due to local tumor and nine due to metastases, usually to bones and lungs). The determinate 5‐, 10‐ and 15‐year survival rates were 78%, 63%, and 44%, respectively. Prognostic factors included : demonstrable tumor on lines of surgical excision, involvement of deep lobe of parotid, size of primary lesion, degree of mitotic activity and nuclear atypia, infiltrative rather than circumscribed borders, and lymph node involvement. Local recurrence was associated with an increased incidence of locally uncontrollable and metastatic disease. Type of initial therapy correlated with prognosis. Locally recurrent or persistent tumor was found in 14 of 15 patients treated with limited local excisions (including one biopsy only). Recurrences were seen in only three of 28 patients treated by wide local excisions and in two of six following radical en bloc resections. Seven of 17 patients with local recurrence were apparently cured by further more extensive resection. Radiotherapy did not appear effective in controlling these neoplasms.

Extramammary Paget's disease.

Extramammary Pagets disease is an in situ skin and mucosal carcinoma frequently associated with and probably arising in a subjacent or regionally proximate carcinoma. Microscopic spread of tumor cells almost always extends beyond clinically apparent disease. Surgical treatment requires carefully planned, systematic excision under precise histologic control. An ideal treatment method remains to be developed. Inadequate excision usually results in recurrences that can be successfully treated by reexcision. Associated invasive carcinomas occur frequently, and mortality is high in these patients.

Papillomatosis of nasal cavity and paranasal sinuses (inverted papilloma, squamous papilloma). A clinicopathologic study

Papillomatosis is an uncommon benign tumor involving the mucous membranes of the nasal cavity and paranasal sinuses. It was approximately 1/25 as frequent as the ordinary nasal polyp. Thirty‐nine patients with this condition have been studied, including clinical and pathologic findings. Recurrences were common (documented 49%, with another 25% probable). Features associated with higher rates of recurrence were epithelial atypia, epithelial mucus droplets, and involvement of sinuses. Carcinoma was associated with papillomatosis, but the extent of this relationship depended on the criteria used. Invasive carcinoma arising in documented recurrent papillomatosis was uncommon (one patient, 3%). In three other cases (8%), relatively small foci of carcinoma were found within extensive papillomatosis. In another four lesions (10%), the specimen resected for a large invasive carcinoma also showed incidental foci of papillomatosis. In these, however, it was difficult to prove that the carcinoma had actually arisen within the papillomatous epithelium. Recommendations for treatment are made.