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Carcinoid Tumour of the Thymus Gland: Report of a Case

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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 thymus gland: 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. Carcinoid 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 carcinoid syndrome. 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 chest pain. 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, flushing, 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 chest radiograph 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 ^*~~~~~~~~~~~~% _4itt.' sss * copyright. http://thorax.bmj.com/ on September 25, 2021 by guest. Protected 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 carcinoma histolo- affecting valves of the right side of the heart. 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 (serotonin) 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 liver and lungs 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 pancreas (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 appendix and terminal ileum, and be seen in the mass. also in the rectum, colon, stomach, 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 gallbladder chemodectoma, bronchial adenoma, thymoma, (Shiffman and Juler, 1964), ovaries (Gabrilove, neurogenic tumour, neurofibroma, teratodermoid Nicolis, and Kirschner, 1969), lungs (Steel, Baerg, tumour (teratoma), cyst (pericardial or broncho- and Adams, 1967), pancreas (Peart et al., 1963), genic), lymphoma (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 epithelium is coarctation, hiatal hernia, and metastatic cancer. 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, cough, shortness of 2 cm in greatest dimension. Typically, they are breath, dysphagia, pneumonia, hoarseness, and well-circumscribed, homogeneous, and yellow to upper respiratory tract 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
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