Case Report Section Cylindroma of the Breast With Pullllonary Metastases* H. R. NAYER, M.D. New York, New York
Cylindroma of the breast occurs rarely. Among 2,623 malignant breast tumors, Geschickter1 found four such cases. More commonly, this tumor arises in the salivary glands, the oral cavity and the tracheo-bronchial tree.2, a, 4, G There has been considerable confusion with regard to 'pathology and biologic behavior of these tumors. They have been classified variously as adenocarcinoma, mixed tumor, basal cell carcinoma, adenocystic carcinoma, carcinoid and others. This arises largely from the lack of complete agreement with regard to the histogenesis of the tumor. Almost 100 years ago, Billroth used the term "cylindroma" to describe a tumor of the paranasal sinuses that was formed of cylinders of hyaline connective tissue between which lay a network of tumor cells. Subsequently, the term was employed to identify a form of adenocarcinoma of the salivary glands. 6 Various origins have been ascribed to the cylindroma arising in the trachea and bronchi: fetal bronchial buds, the basal layer of the epithelium, the mucous glands and excretory ducts. 7 , 8, 9 In a study of cylindroma of the palate, Bauer and Fox3 described two types of cells composing the epithelium of the isthmus and ducts of the mucous glands: cylindric cells and basket (basal) cells. The latter are located between the cylindric cells and the basement membrane as well as between the cylindric cells themselves. The basket cells have also been termed myo-epithelial cells because they are supposed to be capable of contracting and thereby facilitating movement of secretion into the excretory ducts. Bauer and Fox concluded that the basket or myo-epithelial cells formed the bulk of the parenchyma of the cylindroma and suggested the name "adenomyoepitheJioma." The fibrous stroma which radiates from the capsule invades the tubular epithelial formation and is converted into a hyaline substance by coming into contact with the secretion. Myo-epithelial cells are found in albuminous glands, mammary glands, glands of Moll and apocrine sweat glands. 10 In the mammary gland, they appear as elongated, spindle .shaped cells which lie between the epithelium of the excretory ducts and basement membrane. It is believed that these cells may proliferate either alone or in conjunction with the usual epithelium especially in breasts showing mastopathia cystica or fibroadenomatosis. 11 The epithelial element of the cylindroma is composed of small, regular, darkly staining cells which are arranged in either a tubular or acinar pattern. The stroma is formed of varying combinations of myxomatous *From the Private Service, Beth Israel Hospital. 324
CYLINDROMA OF THE BREAST
or hyalinized connective tissue. Mitoses are rare and the general picture is one of uniformity. There has also been considerable diversity of opinion with regard to the biologic behavior of the cylindroma. It has been classified as definitely benign.12 Others have described it as generally benign but with the potentiality of occasional malignant change.s, 11 ,13 It has been considered locally invasive and frequently associated with distant metastases.2 The case to be reported is felt to be of interest not only because of the
FIGURE 1: Section of cylindroma of breast showing typical arrangement of small, dark staining cells with intervening stroma.
FIGURE 2 FIGURE 3 Figure 2: March 16, 1948. Numerous tumor nodules are present throughout the right lung. The right pleura presents the thickened scalloped appearance associated with tumor invasion. A single nodule is seen in the left upper lung field.-Figure 3: Almost complete opacification of the right chest. Numerous nodules are scattered throughout both IUDgs.
rarity of cylindroma of the breast but also because it illustrates clearly the life history of this tumor.
Case Report: The patient was a single, white, female bookkeeper who first came under my observation in September, 1951. In the Spring of 1939, at the age of 39 years, she had noted a painless lump in the left breast. In March, 1940, she had consulted a physician who confirmed the presence of a mass in the left breast. At that time, she gave a history of recent meno-metrorrhagia. She was immediately hospitalized and a left radical mastectomy was carried out. The pathological diagnosis (Dr. Paul Klemperer) W86 cylindroma (Figure 1) ; the lymph nodes were uninvolved. Dr. James Ewing examined the slides and concurred in the diagnosis. Post-operative recovery was satisfactory. Meno-metrorrhagia persisted and 15 months after mastectomy she was subjected to dilatation and curettage followed by radium insertion. Despite this, irregular bleeding continued for the next three years when menses ceased. In 1943, she complained of vague shoulder and hip discomfort; roentgenograms were negative. In 1945, roentgenograms of the hips were again negative for metastases. Meanwhile, she maintained generally good health and carried on her usual activities. In March, 1948, moderate dyspnea on exertion was first reported. Chest roentgenogram (Figure 2) disclosed numerous nodules throughout the right lung with some involvement of the left side. Despite this, she maintained her weight and strength and was able to continue working. During the next two years, there was progressive increase in lung findings. In March, 1950, she complained of severe right shoulder pain; roentgenograms were negative. Radiation therapy to the right humerus W86 administered with little relief. Testosterone injections (200 to 300 mgm. weekly) were started in March, 1950, and continued until January, 1951. She experienced subjective improvement but there were no objective changes. Voice changes and facial hirsutism developed and estrogen was begun. Increasing weakness, dyspnea and weight loss forced her to take to bed by February, 1951. In April, 1951, right chest tap was attempted at another hospital. The pleura was described as markedly thickened and indurated; no fluid was obtained.
FIGURE 4: Material obtained by aspiration of pleural fluid from left chest: fibrin, red blood cells and cluster of hyperchromatic cells suggestive of a cylindromatous arrange-
CYLINDROMA OF THE BREAST
When I first examined her in September, 1951, she appeared almost cachectic. Facial hirsutism was present. The entire right chest was flat to percussion with bronchial breath sounds at the base. Scattered crackles were heard on the left. The mastectomy scar was well healed. The remainder of the physical examination showed no abnormality. Chest roentgenogram (Figure 3) revealed. extensive changes in both lungs. Estrogen was discontinued in October, 1951. In February, 1952, she was hospitalized because of increasing dyspnea. Signs of pleural effusion were present on the left side. Chest aspiration yielded 300 cc. of sercrsanguineous fluid; dyspnea remained essentially unchanged. The pleural fluid contained clusters of hyperchromatic cells suggestive of a cylindromatous arrangement (Figure 4). After she returned home, she was almost completely bed-ridden. For a short period, she received ACTH injections intramuscularly with no apparent effect. On March 20, 1952, 13 years after the breast tumor was first discovered, she died at home. Autopsy was not obtained.
Discussion This case record illustrates the biologic behavior of which this tumor is capable. Progression was extremely slow. Signs of metastases did not appear until eight years after removal of the primary tumor and the p~tient survived for four years in the presence of extensive pulmonary involvement. Also of interest is her relative well being for almost three years after the lungs showed widespread metastases. The metastatic deposits were apparently confined to the lungs and pleura although, in the absence of an autopsy, this cannot be stated with certainty. The pulmonary foci were completely unaffected by both male and female sex hormones and adrenocorticotropic hormone. Although differences from the more common types of malignant tumors exist, nevertheless, it would appear that the cylindroma should be considered always as a basically malignant lesion and should be treated early and radically. While this tumor, in some instances, does not metastasize distantly, it is obviously impossible to predict this in the individual case. In spite of radical mastectomy in our patient, progression to a fatal outcome occurred.
REFERENCES 1 Geschickter, C. F.: Disease of the Breast, ed. 2, Philadelphia, J. C. Lippincott Company.1946. 2 Quattelbaum, F. W., Dockerty, M. B. and Mayo, C. W.: "Adenocarcinoma. Cylindroma Type, of the Parotid Glands: A Clinical and Pathological Study of Twentyone Cases," Surge Gynec. and Obst., 82 :342, 1946. 3 Bauer, W. H. and Fox. R. A.: "Adenomyoepithelioma (Cylindroma) of Palatal MucouoS Glands," Arch. Path., 39:96, 1945. 4 Clark, P. L.. Clagett, O. T. and MeDonald, J. R.: "Cylindromas of the Trachea," Proc. Staff Meet., Mayo Clinic., 28:513. 1953. 6 Holley, S. W.: "Bronchial Adenoma," Mil. Surgeon, 99 :528, 1946. 6 Ewing. J: NeoplaRtic Diseases: A Treatise on Tumors, ed. 3, Philadelphia, W. B. Saunders Company, 1928. 7 Womack, N. A. and Graham, E. A.: "Mixed Tumors of the Lung: So-called Bronchial or Pulmonary Adenoma," Arch. Path., 26:165, 1938. 8 Dean, L. W., Jr.: "Adenocarcinoma of the Trachea: A Pathological Classification of Assistance in Treatment and Prognosis," Ann. Otol. Rhin. and Laryng. 53:669, 1944. Gland," Arch. Otolaryng. 20:375, 1934. 9 Fried, B. M.: "Adenoma C'f Bronchial Mucou~ 10 Maximow, A. A. and Bloom, W.: A Textbook of Histology, Philadelphia, W. B. Saunders Company, 1952. 11 Kuzma, J. F.: "Myoepithelial Proliferation in the Human Breast," Am. J. Path., 19:473, 1943. 12 Howarth, W.: "Mixed Tumor of the Trachea," J. Laryng. and Otol., 54:205, 1989. 18 Foot, N. C.: Pathology in Surgery, Philadelphia, J. B. Lippincott Company, 1945. , 14 Dockerty, M. B. and Mayo. C. W.: "Cylindroma (Adenocarcinoma, Cylindroma Type) ," Surgery, 13 :416, 1943.