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Thorax: first published as 10.1136/thx.30.4.470 on 1 August 1975. Downloaded from Thorax (1975), 30, 470.

Carcinoid tumour of the : report of a case

J. PRESTON HUGHES', NELSON ANCALMO', GEORGE L. LEONARD2, and JOHN L. OCHSNER' Departments of Surgery1 and Pathology2, Alton Ochsner Medical Foundation, New Orleans, Louisiana, USA

Preston Hughes, J., Ancalmo, N., Leonard, G. L., and Ochsner, J. L. (1975). Thorax, 30, 470-475. tumour of the thymus gland: report of a case. Carcinoid of the thymus is a rare problem. A case is reported to add to only 16 previously reported. None of these 17 patients had the . Complete surgical excision, if possible, is the treatment of choice.

A primary carcinoid tumour of the thymus is and a large anterior mediastinal tumour was re- very rare. Rosai and Higa (1972) collected eight sected through a median sternotomy. The involved cases and found only eight additional cases in the pericardium and left phrenic nerve were excised literature. A patient with this unusual lesion was with the large discrete mass. Grossly, the tumour treated at Ochsner Foundation Hospital and the measured 12X9X7 cm and weighed 290 g (Fig. case report is presented, emphasizing the import- 3). It was well-circumscribed, oval, and apparentlycopyright. ance of complete excision. encapsulated; the cut surface was homogeneous, smooth, firm, grey-white, and fleshy. Micro- CASE REPORT scopically the tumour was composed of small, A 32-year-old white man visited the emergency closely packed cells with nuclei that were uniform

in http://thorax.bmj.com/ room on 16 February 1974, because of severe size and shape with few mitotic figures (Figs upper back and . The constant aching 4-6). The cytoplasm of the cells was diffusely pain radiated anteriorly and down both arms; it eosinophilic in most areas and vacuolated in was relieved the shoulders but not others. No specific granules could be visualized. by moving by The ferric ferricyanide reduction technique to analgesics. He had had variable, recurrent chest demonstrate and back pain for one year but no history of argentaffin granules was negative. sweats, , or diarrhoea. The cells were arranged in solid nests and The physical examination revealed no abnor- trabeculae, with areas of tubular or acinar forma- mality. Chest radiographs illustrated a large tion and other areas with the anastomosing ribbon- like on September 25, 2021 by guest. Protected anterior mediastinal chest mass that protruded growth pattern characteristic of carcinoid into the left side of the chest (Figs. 1 and 2). tumours. Abundant normal appearing thymic Laboratory studies were within normal limits tissue was found in the adipose tissue surrounding except for mild elevated blood sugar. After intra- an incomplete, irregular, fibrous tissue capsule. venous injection of 5 mCi 99m technetium per- Postoperatively paradoxical movement of the technetate, an image of the thyroid gland was diaphragm was not a problem. Four days post- obtained using a stationary imaging device. The operatively 400 ml of bloody pleural fluid was thyroid was normal with no substernal or media- removed by thoracentesis. The patient was dis- stinal extensions. An arch aortogram illustrated charged one week postoperatively. no compression from the lesion and no vascular He was last seen on 22 July 1974, with no abnormalities. Some of the blood supply to the complaints. The was normal. tumour originated from small left internal mammary artery branches. Pulmonary function DISCUSSION studies were normal. The term carcinoid tumour was introduced by On 21 February the patient was operated on Oberndorfer (1907) to designate a tumour which 470 Thorax: first published as 10.1136/thx.30.4.470 on 1 August 1975. Downloaded from Carcinoid tumour of the thymus gland: report of a case 471

FIG. 1. Posteroanterior chest film illustrating the large size of the mediastinal

tumour above the aortic arch. copyright. I http://thorax.bmj.com/ on September 25, 2021 by guest. Protected

v t~~~~~~~~~~~~~~~~~~~~~~~~~~"'- .g ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~... FIG. 2. Lateral film showing the anterior mediastinal location of the carcinoid tumour of the thymus gland. Thorax: first published as 10.1136/thx.30.4.470 on 1 August 1975. Downloaded from 472 J. Preston Hughes, N. Ancalmo, G. L. Leonard, and J. L. Ochsner

6 1..3.4.2 O 1 2 3 4? 5 t FIG. 3. Gross appearance of the tumour. Firm, grey-white areas alternate with areas of

red-brown discoloration and one region of central necrosis. copyright. http://thorax.bmj.com/ on September 25, 2021 by guest. Protected Thorax: first published as 10.1136/thx.30.4.470 on 1 August 1975. Downloaded from Carcinoid tumour of the thymus gland: report of a case 473

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with abundant eosinophilic or vacuolated Thorax: first published as 10.1136/thx.30.4.470 on 1 August 1975. Downloaded from 474 J. Preston Hughes, N. Ancalmo, G. L. Leonard, and J. L. Ochsner resembled an undifferentiated histolo- affecting valves of the right side of the . The gically but behaved in a benign fashion. When syndrome is thought to be due to the secretion of Pearson and Fitzgerald (1949) reported a series of 5-hydroxytryptamine () by the tumour metastasizing carcinoid tumours, the malignant cells (Gabrilove et al., 1969). The excess 5- potential was recognized. hydroxytryptamine excreted by the tumour cells Carcinoid tumours arise from argentaffin cells, is oxidized in the and by the enzyme originally described by Kultschitzky (1897) and amine oxidase; with the formation of 5-hydroxy- often termed Kultschitsky cells. These cells, ac- indoleacetic acid (5-HIAA) which is excreted in cording to Bensch et al. (1968), 'are present in all the urine. The estimation of urinary 5-HIAA is structures derived from the primitive entodermal used as a diagnostic test for the detection of car- canal and include the derivatives of the pharyngeal cinoid tumours. arches, the tracheobronchial tree, the gastro- None of the 16 patients previously reported with intestinal canal, the pancreatic duct and the bile carcinoid of the thymus had the carcinoid syn- ducts'. Argentaffin granules also have been drome, nor did our patient. No sweats, flushes, demonstrated in the thymus of some animals, and rashes, palpitation, diarrhoea, bronchospasm, nor normal human thymus has some argyrophilic cells paresthesias were reported in these patients. An (Rosai and Higa, 1972). Kultschitsky cells are explanation for this could be that most of the neuroendocrine cells shown by electron micro- endocrine products are metabolized in the lungs scopy to contain neurosecretory granules reported before they reach the systemic circulation due to to resemble alpha cells of the (Macadam an abnormal blood supply to these tumours. Nor- and Vetters, 1969). mally, the thymic veins drain into the brachio- The tumours which arise from these cells, called cephalic and thyroid veins. argentaffinomas or carcinoid tumours, are found With thymic carcinoid, the tumour may displace infrequently. In MacDonald's (1956) study car- all thymic tissue. The pattern of growth (Fig. 4) is cinoids were found at necropsy in 102 (04%) of in completely divided lobules of fibrous trabeculae.

26 401 subjects examined and were found in 254 Large round to oval 'balls' and areas of centralcopyright. (006%) of 418 166 surgical specimens. The most necrosis with calcification can be present. Normal common site of the lesion is in the intestinal tract, thymus may be near the capsule, but it may also especially the and terminal , and be seen in the mass. also in the , colon, , duodenum, The differential diagnoses of mediastinal and Meckel's diverticulum (Postlethwait, 1966). tumour's must include ectopic parathyroid tumour, http://thorax.bmj.com/ They have also been reported in the chemodectoma, bronchial , , (Shiffman and Juler, 1964), ovaries (Gabrilove, neurogenic tumour, neurofibroma, teratodermoid Nicolis, and Kirschner, 1969), lungs (Steel, Baerg, tumour (teratoma), (pericardial or broncho- and Adams, 1967), pancreas (Peart et al., 1963), genic), (Hodgkin's), intrathoracic parotid gland (Rosai and Higa, 1972), testis (Rosai goitre, mesenchymal tumour (fibrosarcoma), and Higa, 1972), bronchus (Gabrilove et al., 1969), vascular tumour (capillary haemangioma), semi- thymus gland (Gabrilove et al., 1969), and in areas noma (Oldham, 1971), aortic aneurysm, pseudo- where ectopic gastric or intestinal is coarctation, hiatal hernia, and metastatic .

known to occur, such as in teratomas. The clinical symptoms of a mediastinal tumour on September 25, 2021 by guest. Protected Many carcinoid tumours are small, less than are chest pain, dyspnoea, , shortness of 2 cm in greatest dimension. Typically, they are breath, , pneumonia, hoarseness, and well-circumscribed, homogeneous, and yellow to upper infections (Heimburger, grey-white in appearance. Microscopically the Battersby, and Vellios, 1963; Oldham, 1971). All tumour is composed of epithelial cells, which have of these are not specific for the presence of a small uniform nuclei, associated with acidophilic mediastinal tumour. The pain may be excruciating or vacuolated cytoplasm. The growth pattern of and radiate to the back and arms, as it did in our the tumour cells is characteristic with cells patient. arranged in alveolar nests, trabeculae, tubules, The evaluation of the patient should include acini, and in an anastomosing ribbon-like pattern. chest films, tomograms of the tumour, fluoro- Occasionally, when a large amount of tumour scopy, barium meal, radioisotope scanning, tissue is present, the 'carcinoid syndrome' may be bronchoscopy, angiography, and mediastinoscopy. present. This consists of attacks of flushing of the However, we would agree with Heimburger et al. skin, cyanosis, and hypertension, perhaps associ- (1963) that special diagnostic procedures are help- ated with lesions of the endocardium especially ful in detecting aneurysms, diaphragmatic hernia, Thorax: first published as 10.1136/thx.30.4.470 on 1 August 1975. Downloaded from Carcinoid tumour of the thymus gland: report of a case 475 and primary tumours of the , but no study Lattes, R. (1962). Thymoma and other tumors of the can give specific diagnosis in the majority of thymus. An analysis of 107 cases. Cancer, 15, primary mediastinal tumours. Thoracotomy, with 1224. Macadam, R. F. and Vetters, J. M. (1969). Fine either or surgical removal, has proved the structural evidence for secretion by a only reliable method of diagnosis. human thymic tumour. Journal of Clinical Complete surgical excision of carcinoid , 22, 407. tumours, if possible, has been recommended con- MacDonald, R. A. (1956). A study of 356 sistently (Lattes, 1962; Heimburger et al., 1963; of the ; report of four new Wilkins, Edmunds, and Castleman, 1966; Postle- cases of the carcinoid syndrome. American thwait, 1966; Cohn and Grimes, 1970). Cohn and Journal of Medicine, 21, 867. that 'can expect not Oberndorfer, S. (1907). Karzinoide Tumoren des Grimes (1970) add patients Dunndarms. Frankfurt Zeitschrift fur Pathologie, only reasonable relief but also complete oblitera- 1, 426. tion of symptoms'. With the current status of Oldham, H. N. Jr. (1971). and thoracic and postoperative care, the . Annals of Thoracic Surgery, 11, 246. operative mortality for excision of tumours of Pearson, C. M. and Fitzgerald, P. J. (1949). Carcinoid the is low. Oldham (1971) reported tumors. A re-emphasis of their malignant nature. a hospital mortality rate of 1-8% for 164 patients. Cancer, 1005. For our patient the median sternotomy offered Peart, W. S., Porter, K. A.. Robertson, J. I. S., good exposure for resection of the anterior Sandler, M., and Baldock, E. (1963). Carcinoid syndrom due to pancreatic-duct secret- mediastinal neoplasm. ing 5-hydroxytryptophan and 5-hydroxytrypta- Postoperative irradiation was not used for two mine. Lancet, 1, 239. reasons: the malignant potential of carcinoid of Postlethwait, R. W. (1966). Gastrointestinal car- the thymus is unknown and there is the possibility cinoid tumors. A review. Postgraduate Medicine, of increased morbidity from mediastinal 40, 445. irradiation. Rosai, J. and Higa, E. (1972). Mediastinal endocrine

neoplasm, of probable thymic origin, related to copyright. carcinoid tumor. Clinicopathologic study of 8 REFERENCES cases. Cancer, 29, 1061. Bensch, K. G., Corrin, B., Pariente, R., and Spencer, Shiffman, M. A. and Juler, G. (1964). Carcinoid of H. (1968): Oat-cell carcinoma of the lung. Its the . Archives of Surgery, 89, 1113. origin and relationship to bronchial carcinoid. Steel, K., Baerg, R. D., and Adams, D. 0. (1967). Cancer, 22, 1163. Cushing's syndrome in association with a car- http://thorax.bmj.com/ Cohn, L. H. and Grimes, 0. F. (1970). Surgical man- cinoid tumor of the lung. Journal of Clinical agement of thymic . Surgery, Gyne- Endocrinology and Metabolism, 27, 1285. cology and Obstetrics, 131, 206. Wilkins, E. W. Jr., Edmunds, L. H. Jr., and Gabrilove, J. L., Nicolis, G. L., and Kirschner, P. A. Castleman, B. (1966). Cases of thymoma at the (1969). Cushing's syndrome in association with Massachusetts General Hospital. Journal of carcinoid tumor. Annals of Surgery, 169, 240. Thoracic and Cardiovascular Surgery, 52, 322. Heimburger, I., Battersby, J. S., and Vellios, F. (1963). Primary neoplasms of the mediastinum. Archives of Surgery, 86, 978. Kultschitzky, N. K. (1897). Zur Frage uber den Requests for reprints to: Dr. J. L. Ochsner, Alton on September 25, 2021 by guest. Protected Bau des Darmkanals. Archiv fur Mikrobiologie Ochsner Medical Foundation, 1514 Jefferson Highway, Anatomie, 49, 7. New Orleans, Louisiana, 70121, USA.