Bilateral Carcinoid Tumors of the Mediastinum

Bilateral Carcinoid Tumors of the Mediastinum

Bilateral Carcinoid Tumors of the Mediastinum Stanley C. Fell, M.D., Seymour Sprayregen, M.D., and Nonvin H. Becker, M.D. B ronchial adenomas of the...

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Bilateral Carcinoid Tumors of the Mediastinum Stanley C. Fell, M.D., Seymour Sprayregen, M.D., and Nonvin H. Becker, M.D.

B

ronchial adenomas of the carcinoid type resemble carcinoids of the gastrointestinal tract and are believed to originate from bronchial mucous glands. Perhaps the rarest site for occurrence of a carcinoid tumor is the mediastinum; 5 such cases have been reported [Z-41 previously, 1 within the wall of a bronchogenic cyst. It is the purpose of this communication to report a case of bilateral mediastinal carcinoid tumors; apparently it is the first such case to be described. A. Z., a 43-year-old Caucasian man, was admitted to the Montefiore Hospital on July 21, 1965. One week prior to admission the patient had noted the onset of cough, vague right anterior chest pain, and facial fullness; a chest roentgenogram performed at that time revealed a mediastinal mass. Physical examination and blood and urine analyses were not remarkable. Chest roentgenograms revealed an ovoid lobulated mass approximately 16 cm. in greatest diameter occupying the right anterior mediastinum and a mass in the left anterior mediastinum (Fig. 1). An intravenous angiogram demonstrated posterior displacement of and partial obstruction of flow through the superior vena cava by the mass, with reflux of contrast material into the azygos vein. The left anterior mediastinal density seen was interpreted as being an extension of the tumor across the midline. On July 23, 1965, a right posterolateral thoracotomy was performed through the bed of the resected fifth rib. The tumor occupied the entire right side of the anterior mediastinum, compressing the right lung and superior vena cava, and was intimately adherent to the parietal pericardium. The internal mammary and pericardiophrenic veins and their collaterals were markedly dilated. After dissecting the superior vena cava and the phrenic nerve free, the neoplasm was removed in its entirety with a 3 x 5 cm. segment of adherent pericardium. Residual tumor was not noted in the right anterior mediastinurn. The chest was closed in routine fashion. The postsurgical course was uneventful, but postoperative posteroanterior and apical lordotic roentgenograms of the chest demonstrated persistence of the ovoid left anterior mediastinal density (Fig. 2). Blood serotonin and urinary 5-hydroxyindoleacetic acid studies performed prior to the second thoracotomy were within normal limits. On October 13, 1965, a left anterolateral thoracotomy was performed. The tumor, approximately 6 cm. in diameter, lay anterior to the pulmonary artery; the left phrenic nerve coursed over its lateral border. It was readily excised along with From the Divisions of Surgery and Diagnostic Roentgenology and the Laboratory of the Montefiore Hospital and Medical Center, New York, N.Y. Serotonin studies were performed in the Serotonin Laboratory of the Mount Sinai Hospital, New York, N.Y., through the courtesy of Richard R. P. Warner, M.D. Accepted for publication Jan. 28, 1966.

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FIG. 1. Roentgenogram taken at time of admission, showing a lobulated right anterior mediastinal mass with a small component overlying the left hilar structures.

FIG. 2. Postoperative apical lordotic chest roentgenogram showing left hilar mass. a portion of the contiguous mediastinal fibroadipose tissue. The postoperative course was uneventful, and roentgenograms of the chest revealed a normal mediastinurn. On gross inspection, the tumor removed at the first operation consisted of an ovoid mass which measured 13.0 X 11.8 x 7.5 cm. in greatest dimension and weighed 800 gm. Cross-section (Fig. 3) revealed patchy zones of congestion and necrosis in what was otherwise a homogeneous, somewhat granular, tan-colored

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CASE REPORT:

Carcinoid Medinstinal Tumors

FIG. 3. Cross section o f formalin-fixed tumor. T h e mass appears well encapsulated. Note zones of hemorrhage and necrosis.

FIG. 4. Carcinoid tumor. Note typical cords and nests of uniform cells with a vascular stroma. HhE, ~ 2 0 0 before , 25% reduction. tumor. The tumor was grossly surrounded by a thin fibrous capsule, adherent to which were small portions of an adipose-like tissue. Microscopic examination of the tumor revealed a histological picture identical to that of a classic carcinoid tumor (Fig. 4). The tumor cells were small and uniformly cuboid-columnar in shape, with acidophilic granular cystoplasm and nuclei which were normochromatic with a few fine chromatin clumps. Some nuclear atypia and an occasional mitotic figure were seen. The tumor cells were arranged in cords and nests supported by a delicate vascular fibrous stroma. Acinar arrangements were present but uncommon. Zones of hemorrhagic necrosis were VOL. 2, NO.

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F I G . 5. Lymph node with nest of carcinoid tissue in a lyml>h sinus. H h E , X65, before 25% reduction.

present. The tumor cells contained neither argentaffine-positive granules nor periodic acid-Schiff-positive material. In the adipose tissue surrounding the fibrous capsule, thymic tissue unassociated with tumor was evident. Although the tumor appeared well encapsulated, nests of tumor cells were present in some lymphatic channels of the pericapsular soft tissue. The tumor removed at the second operation consisted of an oval mass which measured 6 X 5 x 4 cm. in greatest dimension and weighed approximately 70 gm. Its gross and microscopic appearance was similar to that of the first tumor, except necrosis and hemorrhage were absent. Again, thymic tissue was noted in the pericapsular soft tissue. Of note was the presence of tumor in this pericapsular soft tissue, in free-lying nests, in the lymphatics intermingled with thymic tissue, and in the sinusoids of lymph nodes (Fig. 5). T h e second tumor was assayed for serotonin content, which was within normal limits. DISCUSSION

Five mediastinal tumors of similar histological type have been reported previously (Table 1). Four were located in the anterior and one in the posterior mediastinum. In one case [3] tumor metastasis was later found in a supraclavicular lymph node, and in another [4],an autopsy revealed tumor to be present in the pulmonary hilum without evidence of distant metastases. The presence in our patient of mediastinal spread with lymphatic invasion reemphasizes the aggressiveness, albeit low grade, of carcinoid tumors. The origin of these tumors remains obscure. The report [Z] describing a carcinoid tumor arising in a bronchial cyst located in the anterior mediastinum is of particular interest since it lends support to the hypothesis that these neoplasms are derived from displaced bronchial anlagen. However, in no other case, including the present one, was there evidence of such anlagen, bronchial cyst, or connection with the bronchial tree. 432

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64

43

39

43

M

F

M

M

M

M

Ringertz & Lidholm 141

Pachter & Lattes [31

Greenfield & Howe 121

Fell et al. [present report1

43

37

Age

Sex

Reporter

L. Ant.

R. Ant.

L. Ant.

Ant. Sup.

R. Ant.

L. Post. Inf.

R. Ant. Sup.

Mediastinal Location

Total excision

Total excision

Total excision

mass surrounding left innominate vein

9 x 11 x 5 cm. bronchogenic cyst with 5 cm.tumor in its wall 16 x 10 X 10 cm. encapsulated mass adherent to pericardium 6 x 5 x 4 cm.encapsulated mass anterior to pulmonary artery

Totally excised; patient well 6 years later Biopsy only; patient died postoperatively. Autopsy disclosed locally invasive tumor without distant metastases. Total excision; 1 year later metastases to a supraclavicular node. Two years after node biopsy, no evidence of recurrent disease.

.

Totally excised; patient well 5% years later

Clinical Course

10 x 7 x 3 cm. encapsulated

Encapsulated grapefruitsized mass adherent to right lung Encapsulated mass posterior to breast Cocoanut-sized tumor infiltrating right chest and lung hilus

Surgical Findings

TABLE 1. MEDIASTINAL CARCINOID TUMORS

FELL, SPRAYREGEN, AND BECKER

It has been suggested that these tumors are unilaterally differentiated teratomas. This hypothesis, if true, would account for their predominantly anterior mediastinal location. Carcinoid tumors have been demonstrated arising in ovarian and testicular teratomas [l]. In most of these, gastric, intestinal, or respiratory epithelium was noted. Teratoid elements have not been noted in carcinoid tumors; thus it appears more likely that the carcinoids arising in teratomas derive from the enteric or respiratory components of the teratoma. Finally, the possibility exists that tumors are histologic variants of thymoma; the carcinoid tumors often lie in intimate relation to thymic tissue. Indeed, in normal adult thymic tissue, palisading arrays of epithelial cells which bear some resemblance to those comprising the carcinoid tumor may be demonstrated. T h e following observations, however, militate against the possibility that the carcinoid tumors are variants of thymoma: In no case have carcinoid elements been found mixed with the usual cytological variants of thymoma. Periodic acid-Schiff-positive granules, occasionally seen in thymic epithelial cells, were absent in the cells of both carcinoid tumors. T h e invasion of lymph node sinusoids by the tumor is inconsistent with the usual invasive pattern of thymoma. Of all the aforementioned hypotheses of origin, we prefer to consider these tumors as most likely arising from bronchial anlagen; these small remnants would be most easily obscured by the neoplastic growth. SUMMARY

A case of bilateral mediastinal carcinoid tumors is reported, and five previously reported unilateral tumors of this type are reviewed. These tumors are thought to originate from mediastinal remnants of bronchial anlage and may be invasive. REFERENCES

1. Betson, J. R., and Golden, M. L. Primary argentaffin carcinoid arising in ovarian teratoma: Report of two cases. Amer. J . Obstet. Gynec. 77:1345, 1959. 2. Greenfield, L. J., and Howe, J. S. Bronchial adenoma within the wall of a bronchogenic cyst. J . Thorac. Cardiov. Surg. 49:398, 1965. 3. Pachter, M. R., and Lattes, R. Uncommon mediastinal tumors. Dis. Chest 43:519, 1963. 4. Ringertz, N., and Lidholm, S. 0. Mediastinal tumors and cysts. J . Thorac. Surg. 31:458, 1956.

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