Marianne Wolff

Epithelial neoplasms of the vermiform appendix (exclusive of carcinoid). II. Cystadenomas, papillary adenomas, and adenomatous polyps of the appendix

Forty‐two cases of benign epithelial neoplasms of the appendix gleaned from the files of the Laboratory of Surgical Pathology, Columbia University, were studied. The majority were associated with dilatation of the appendix, or “mucocele.” Conversely only 50% of all cystically dilated appendices examined had definite underlying adenomatous changes. Thus it is important to distinguish between cystic neoplasms (cystadenomas) and non‐neoplastic retention cysts of the appendix. The most frequently found benign epithelial neoplasms were papillary adenomas. Even in the presence of atypia we labeled them benign. Appendectomy is usually adequate treatment for all adenomatous lesions occurring in the appendix. Almost a quarter of the cases reviewed were found to have synchronous or metachronous neoplasms elsewhere in the colon, while several patients harbored multiple colonic neoplasms in addition to the appendiceal adenomas. Thus a patient with an adenomatous lesion of the appendix should undergo a systematic search for other colonic lesions.

Lymphangiomyoma: Clinicopathologic study and ultrastructural confirmation of its histogenesis

Two new cases of lymphangiomyoma are added to 28 cases previously reported. One of these was studied ultrastructurally, with findings that support the designation “lymphangiomyoma,” i.e., smooth muscle and lymphatic channels. The disease seems to occur exclusively in females, is not infrequently associated with endocrine disorders, has some features analogous to tuberous sclerosis, and may represent an incomplete expression or forme fruste of Bournevilles disease. The condition consists of one or more nodules of tumor‐like proliferation of smooth muscle fascicles, divided from each other by lymphatic channels, and involves major lymphatic trunks in the retroperitoneum or mediastinum, lymph nodes in these regions and, in about two thirds of cases, lymphatics of the lungs. The most common complaints are referable to chylous effusions, pulmonary insufficiency, and pneumothorax. Three patients have been followed for over 14 years after excision of the nodules and postoperative radiotherapy.

Epithelial neoplasms of the vermiform appendix (exclusive of carcinoid). I. Adenocarcinoma of the appendix

A study of primary epithelial neoplasms of the appendix (with the exception of classical carcinoids), produced 24 adenocarcinomas and 42 benign lesions from the files of the Laboratory of Surgical Pathology of Columbia University, Invasive adenoarcinomas were often complicated by appendicitis and were then unexpectedly discovered during appendectomy. In one third of the cases the carciomas were found in juxtaposition to adenomatous lesions of the appendix. Well‐differentiated adenocarcinomas of the colonic type generally had a better prognosis than signet‐ring cell (or microglandular) tumors. The most reliable criterion for correlating prognosis was the Dukes method of staging. Since invasive adenocarcinomas of the appendix can metastasize to regional lymph nodes, treatment should ideally consist of ileocolectomy.

Delayed distant metastasis from a subcutaneous sacrococcygeal ependymoma. Case report, with tissue culture, ultrastructural observations, and review of the literature

This represents the first reported case of pulmonary metastasis from an extradural ependymoma in a subcutaneous location in the sacrococcygeal region. The nature of the metastasis was documented by tissue culture and ultrastructural examination. The embryologic basis for the occurrence of ependymomas in this region appears to be the coccygeal medullary vestige of the neural tube. Previous reports of metastasizing ependymomas include 16 which were primary in the brain, six in the cauda equina, and one in a postsacral location. Factors potentiating metastasis in these tumors include the influence of surgical intervention and longer survival from neurosurgical procedures, further enhanced by radiotherapy. The most frequent targets for metastasis from ependymomas are lungs, lymph nodes, and liver. Ultrastructural examination of the lung metastasis in this case disclosed features of both normal ependyma and choroid plexus.

A Rational Approach to Total Thymectomy in the Treatment of Myasthenia Gravis

Thymectomy is important in the treatment of myasthenia gravis. Total removal of the gland is considered indicated. Although median sternotomy has been the accepted surgical procedure, the transcervical approach has been advocated as a safer method of achieving total thymectomy. A surgical-anatomical study of the thymus was made in 22 patients. A high incidence of surgically important variations in thymic anatomy was found in the neck and in the mediastinum. We believe wide exposure by way of median sternotomy with direct vision is required to remove all of the extracapsular mediastinal thymus in many patients, and good cervical exposure is required to remove the anomalous tissue in the neck. If a total thymectomy is to be achieved, we recommend a median sternotomy and a cervical incision, using the meticulous dissection described.

Adenoid cystic carcinoma of the breast report of 11 new cases: Review of the literature and discussion of biological behavior

Eleven new cases of adenoid cystic carcinoma of the breast are added to the 95 previously reported. They were studied for their biological behavior, and an attempt was made to correlate histologic features with prognosis. A comparison was made with analogous tumors in extramammary sites, and a possible relationship with carcinoid tumors of the breast explored. The patients in the study followed a biological course very similar to that described by previous studies, i.e., slow progression, local recurrence if inadequately resected, and absence of lymph node metastases. Only one patient developed distant metastases after an interval of ten years following radical mastectomy. If the diagnosis of adenoid cystic carcinoma is reserved for those lesions displaying the distinctive appearance and typical epithelial‐stromal relationships which have been described, the authors feel that simple mastectomy with careful follow‐up surveillance is the treatment of choice.

Leiomyoma and Leiomyosarcoma of the Orbit

Comparison of the clinical and pathologic features of two orbital leiomyomas, two leiomysarcomas, and one embryonal rhabdomyosarcoma showed the leiomyomas occurred in young individuals and the leiomyosarcomas in older patients. The histopathologic diagnosis rested on the intense cytoplasmic eosinophilia and nostriated longitudinal cytoplasmic filaments demonstrated by means of the trichrome stain. The leiomyosarcomas disseminated 15 months and seven years after their orbital presentations. The treatment of both leiomyoma and leiomyosarcoma is surgical. Leiomyomas are encapsulated growths that may have small satellite nodules projecting from the main tumor mass; thus, a margin of normal tissue should also be excised, lest a small lobulation be left behind to serve as the seed for a late recurrence. Once the diagnosis of leiomyosarcoma has been made, and no evidence of metastasis has been found after a thorough systemic evaluation, the orbit should be exenterated, because the tumor is unencapsulated and liable to widespread dissemination. Rhabdomyosarcoma has a much more fulminant course than leiomyosarcoma, and especially more so than that of leiomyoma of childhood. The histopathologic diagnosis of a malignant smooth muscle tumor in a child should always be questioned, since embryonal rhabdomyosarcoma is a much more likely diagnosis.

Meningioma of the parotid gland: An insight into the pathogenesis of extracranial meningiomas

Abstract A patient with a meningioma of the parotid gland is presented. This is believed to be the first reported case of such a tumor confined to a salivary gland. It is postulated that groups of arachnoid cells, displaced during embryonic life, followed the facial nerve into the parotid gland and provided the anlage for this tumor.

Branchiogenic carcinoma — fact or fallacy?

The ongoing debate concerning the existence of malignant transformation of branchial cleft cysts has been analyzed, with the aid of previous reports and reviews in the literature. Six new cases are presented and illustrated. These occurred in 4 male and 2 female patients, having an average age of 60 years. Two of the lesions were within the parotid salivary gland. The longest period of survival was 3 1/2 years. New criteria for the acceptability of tumors as primary branchiogenic cancers are proposed. We believe that, using these strict criteria, rare examples of primary branchiogenic carcinomas do exist; their treatment should include whide local excision, followed by ipsilateral radical neck dissection.

Heterotopic Gastric Epithelium in the Head and Neck Region

Heterotopic gastric epithelium in the head and neck area is a little-known entity, unsuspected by clinician and pathologist alike. We have reviewed the literature and discovered 14 cases of this entity. While previous reviews of the subject have, unfortunately, included reports of cases with heterotopic intestinal epithelium, our study addresses itself to separating the two. Five new cases of gastric heterotopia are reported in 3 female and 2 male patients ranging in age from 2 1/2 weeks to 70 years. Three cystic lesions occurred in the floor of the mouth, while 2 small nudular lesions were found in the region of the larynx. The larger lesions caused obstructive symptoms. An error in embryogenesis is the only plausible explanation for such heterotopic elements as gastric fundic, antral, and mucous neck types of epithelium, often associated with a partial muscularis mucosae, in the head and neck region. Treatment consists of surgical extirpation.