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Primary Carcinoid of Testis
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
PRIMARY CARCINOID OF TESTIS STANLEY WEITZNER*
AND
JACK R. ROBISONt
From the Department of Pathology, University of Mississippi Medical Center, Jackson, Mississippi, and the Departments of Pathology and Urology, University of Texas Health Science Center and Veterans Administration Hospital, San Antonio, Texas
ABSTRACT
A patient with primary solid carcinoid of the testis is described and 8 previous cases are reviewed. Ultrastructural study of our case disclosed pleomorphic granules, typical of the insular type carcinoid of the intestine of midgut derivation. Primary solid testicular carcinoid appears to behave as a benign neoplasm. None of these patients had evidence of metastases or the carcinoid syndrome for periods ranging from 20 months to 25 years. Carcinoid is a most unusual primary neoplasm of the testis, with only 8 cases reported previously (see table).,_, In each instance the tumor was circumscribed, solid, yellow, yellowbrown or brown and usually firm. There were small cystic or necrotic foci in several cases. They were histopathologically identical to the insular type carcinoid found in the intestine derived from the midgut. Diagnosis was not suspected clinically. Primary carcinoid of testis
Reference Kemble' Yalla and associates 2 Brown (6 cases)'
Present case
Age (yrs.) 53 45
Rt. Rt.
27 to 56 (mean 48)*
48
Size (cm.)
Side
5 by 3.5 by 3 6
Metastasis or Carcinoid Syndrome No No
As large as 3.5* · No
Lt.
6 by 5 by 4.5
No
Followup Alive (3 yrs.) Alive (4 yrs.) 5 alive (2, 4, 5, 5 and 15 yrs.) and 1 dead (25 yrs.) Alive (20 mos.)
* Not mentioned for individual cases.
None of the patients had evidence of metastases or the carcinoid syndrome for periods ranging from 2 to 25 years. In 2 otherwise asymptomatic patients, 58 4 and 53 5 years old, the carcinoid was a minor component of a cystic teratoma of the testis. Herein we report on a patient with primary carcinoid of the testis including the ultrastructure. CASE REPORT
A 48-year-old man was hospitalized on August 23, 1974 because of a left testicular mass 3 months in duration. Physical examination revealed a hard, 8 by 7 cm. left testicular mass. Urinary human chorionic gonadotropins, chest x-ray and excretory urogram were normal. Radical orchiectomy was performed 4 days later. The firm testis tumor measured 6 by 5 by 4.5 cm. The cut surface was yellow, lobular and solid, with minute cystic spaces, and contained several broad fibrous bands in the posteroinferior area. Residual compressed testicular tissue was noted in the upper pole. The tunica albuginea was thickened focally. The fibrous, parietal tunica vaginalis ranged to 7 mm. in thickness (fig. 1). The narrowed elongated Accepted for publication June 25, 1976. * Requests for reprints: Department of Pathology University of Mississippi Medical Center, Jackson, Mississippi 39216'. t Deceased.
FIG. 1. Hemisection of testicular carcinoid
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822
WEITZNER AND ROBISON
epididymis and the 6.5 cm. segment of spermatic cord were unremarkable. Microscopically, there were irregular islands of uniform cells with acinar formation in places. The oval and round nuclei were essentially of uniform size and had a single nucleolus or prominent chromatin clumping (fig. 2). FontanaMasson stain revealed argentaffin granules primarily in the cells at the periphery of the islands and the acini. The islands were separated by narrow fibrous bands. There were broad bands of fibrous and hyalin fibrous tissue in the posteroinferior part of the tumor. No teratomatous elements were identified. The residual testicular tissue was atrophic. The tunica albuginea was fibrous thickened focally but otherwise unremark-
able. The parietal tunica vaginalis was sclerotic diffusely. The epididymis and spermatic cord were unremarkable. Diagnosis was carcinoid of the testis. Electron microscopic examination of tumor tissue fixed in formalin for 12 months disclosed an abundance of dense, pleomorphic granules ranging from 50 to 350 µm. In some a narrow electron-lucent zone separated the dense core from a distinct external limiting membrane (fig. 3). These findings were typical of insular carcinoid of midgut derivation. Several urine 5-hydroxy-indole acetic acid (5-HIAA) determinations, an upper gastrointestinal and small intestinal series, and a barium enema were normal postoperatively. The patient was discharged from the hospital and has remained free of disease for 20 months. Periodic urine 5-HIAA determinations and chest x-rays have been consistently normal. DISCUSSION
FIG. 2. Insular carcinoid consisting of islands of essentially uniform cells and nuclei with acinar formation separated by thin bands of fibrous tissue. H & E, reduced from x40.
The testicular carcinoid in our patient had the typical histopathologic and ultrastructural characteristics 6 of an insular carcinoid of midgut derivation. Primary solid carcinoid of the testis may represent the unilateral development of a teratoma or be derived from argentaffin cells within the testis. The latter is supported by amine precursor uptake and decarboxylation cells of neural crest origin, having been observed to invade the developing genitourinary tracts. 7 The possibility of a testicular carcinoid being a metastasis should be excluded. We believe that our case is a primary carcinoid of the testis because roentgenographic studies of the gastrointestinal tract failed to disclose a primary focus and the patient has not had abdominal symptomatology during the 20 months postoperatively. Furthermore, it is unlikely that a primary intestinal carcinoid would have remained clinically silent or undergone self-regression after metastasizing exclusively to an uncommon site, such as the testis. Although the number of cases is small primary solid insular carcinoid of the testis has not been associated with metastases or the carcinoid syndrome and appears to behave as a benign neoplasm. Dr. Virginia G. Lockard assisted with the electron microscopy and Dr. Howard Radwin provided the followup information. REFERENCES
1. Kemble, J. V.: Argentaffin carcinomata of the testicle. Brit. J. Ural.,
FIG. 3. Electron micrograph of insular carcinoid with typical pleomorphic granules and limiting membrane separated by electron-lucent zone from dense central core (arrow). Reduced from x27,875.
40: 580, 1968. 2. Yalla, S. V., Yalla, S.S., Morgan, J. W., Eberhart, C. A. and Olley, J. F.: Primary argentaffinoma of the testis: a case report and survey of the literature. J. Ural., 111: 50, 1974. 3. Brown, N. J.: Miscellaneous tumors of epithelial type. In: Pathology of the Testis. Edited by R. C. B. Pugh. Oxford: Blackwell Scientific Publications, p. 308, 1976. 4. Simon, H.B., McDonald, J. R. and Culp, 0. S.: Argentaffin tumor (carcinoid) occurring in a benign cystic teratoma of the testicle. J. Ural., 72: 892, 1954. 5. Berkheiser, S. W.: Carcinoid tumor of the testis occurring in a cystic teratoma of the testis. J. Ural., 82: 352, 1959. 6. Black, W. C., III: Enterochromaffin cell types in corresponding carcinoid tumors. Lab. Invest., 19: 473, 1968. 7. Pearse, A. G. E.: The APUD cell concept and its implications in pathology. Path. Annu., 9: 27, 1974.
Copyright© 1976 by The Williams & Wilkins Co.
PRIMARY CARCINOID OF TESTIS STANLEY WEITZNER*
AND
JACK R. ROBISONt
From the Department of Pathology, University of Mississippi Medical Center, Jackson, Mississippi, and the Departments of Pathology and Urology, University of Texas Health Science Center and Veterans Administration Hospital, San Antonio, Texas
ABSTRACT
A patient with primary solid carcinoid of the testis is described and 8 previous cases are reviewed. Ultrastructural study of our case disclosed pleomorphic granules, typical of the insular type carcinoid of the intestine of midgut derivation. Primary solid testicular carcinoid appears to behave as a benign neoplasm. None of these patients had evidence of metastases or the carcinoid syndrome for periods ranging from 20 months to 25 years. Carcinoid is a most unusual primary neoplasm of the testis, with only 8 cases reported previously (see table).,_, In each instance the tumor was circumscribed, solid, yellow, yellowbrown or brown and usually firm. There were small cystic or necrotic foci in several cases. They were histopathologically identical to the insular type carcinoid found in the intestine derived from the midgut. Diagnosis was not suspected clinically. Primary carcinoid of testis
Reference Kemble' Yalla and associates 2 Brown (6 cases)'
Present case
Age (yrs.) 53 45
Rt. Rt.
27 to 56 (mean 48)*
48
Size (cm.)
Side
5 by 3.5 by 3 6
Metastasis or Carcinoid Syndrome No No
As large as 3.5* · No
Lt.
6 by 5 by 4.5
No
Followup Alive (3 yrs.) Alive (4 yrs.) 5 alive (2, 4, 5, 5 and 15 yrs.) and 1 dead (25 yrs.) Alive (20 mos.)
* Not mentioned for individual cases.
None of the patients had evidence of metastases or the carcinoid syndrome for periods ranging from 2 to 25 years. In 2 otherwise asymptomatic patients, 58 4 and 53 5 years old, the carcinoid was a minor component of a cystic teratoma of the testis. Herein we report on a patient with primary carcinoid of the testis including the ultrastructure. CASE REPORT
A 48-year-old man was hospitalized on August 23, 1974 because of a left testicular mass 3 months in duration. Physical examination revealed a hard, 8 by 7 cm. left testicular mass. Urinary human chorionic gonadotropins, chest x-ray and excretory urogram were normal. Radical orchiectomy was performed 4 days later. The firm testis tumor measured 6 by 5 by 4.5 cm. The cut surface was yellow, lobular and solid, with minute cystic spaces, and contained several broad fibrous bands in the posteroinferior area. Residual compressed testicular tissue was noted in the upper pole. The tunica albuginea was thickened focally. The fibrous, parietal tunica vaginalis ranged to 7 mm. in thickness (fig. 1). The narrowed elongated Accepted for publication June 25, 1976. * Requests for reprints: Department of Pathology University of Mississippi Medical Center, Jackson, Mississippi 39216'. t Deceased.
FIG. 1. Hemisection of testicular carcinoid
821
822
WEITZNER AND ROBISON
epididymis and the 6.5 cm. segment of spermatic cord were unremarkable. Microscopically, there were irregular islands of uniform cells with acinar formation in places. The oval and round nuclei were essentially of uniform size and had a single nucleolus or prominent chromatin clumping (fig. 2). FontanaMasson stain revealed argentaffin granules primarily in the cells at the periphery of the islands and the acini. The islands were separated by narrow fibrous bands. There were broad bands of fibrous and hyalin fibrous tissue in the posteroinferior part of the tumor. No teratomatous elements were identified. The residual testicular tissue was atrophic. The tunica albuginea was fibrous thickened focally but otherwise unremark-
able. The parietal tunica vaginalis was sclerotic diffusely. The epididymis and spermatic cord were unremarkable. Diagnosis was carcinoid of the testis. Electron microscopic examination of tumor tissue fixed in formalin for 12 months disclosed an abundance of dense, pleomorphic granules ranging from 50 to 350 µm. In some a narrow electron-lucent zone separated the dense core from a distinct external limiting membrane (fig. 3). These findings were typical of insular carcinoid of midgut derivation. Several urine 5-hydroxy-indole acetic acid (5-HIAA) determinations, an upper gastrointestinal and small intestinal series, and a barium enema were normal postoperatively. The patient was discharged from the hospital and has remained free of disease for 20 months. Periodic urine 5-HIAA determinations and chest x-rays have been consistently normal. DISCUSSION
FIG. 2. Insular carcinoid consisting of islands of essentially uniform cells and nuclei with acinar formation separated by thin bands of fibrous tissue. H & E, reduced from x40.
The testicular carcinoid in our patient had the typical histopathologic and ultrastructural characteristics 6 of an insular carcinoid of midgut derivation. Primary solid carcinoid of the testis may represent the unilateral development of a teratoma or be derived from argentaffin cells within the testis. The latter is supported by amine precursor uptake and decarboxylation cells of neural crest origin, having been observed to invade the developing genitourinary tracts. 7 The possibility of a testicular carcinoid being a metastasis should be excluded. We believe that our case is a primary carcinoid of the testis because roentgenographic studies of the gastrointestinal tract failed to disclose a primary focus and the patient has not had abdominal symptomatology during the 20 months postoperatively. Furthermore, it is unlikely that a primary intestinal carcinoid would have remained clinically silent or undergone self-regression after metastasizing exclusively to an uncommon site, such as the testis. Although the number of cases is small primary solid insular carcinoid of the testis has not been associated with metastases or the carcinoid syndrome and appears to behave as a benign neoplasm. Dr. Virginia G. Lockard assisted with the electron microscopy and Dr. Howard Radwin provided the followup information. REFERENCES
1. Kemble, J. V.: Argentaffin carcinomata of the testicle. Brit. J. Ural.,
FIG. 3. Electron micrograph of insular carcinoid with typical pleomorphic granules and limiting membrane separated by electron-lucent zone from dense central core (arrow). Reduced from x27,875.
40: 580, 1968. 2. Yalla, S. V., Yalla, S.S., Morgan, J. W., Eberhart, C. A. and Olley, J. F.: Primary argentaffinoma of the testis: a case report and survey of the literature. J. Ural., 111: 50, 1974. 3. Brown, N. J.: Miscellaneous tumors of epithelial type. In: Pathology of the Testis. Edited by R. C. B. Pugh. Oxford: Blackwell Scientific Publications, p. 308, 1976. 4. Simon, H.B., McDonald, J. R. and Culp, 0. S.: Argentaffin tumor (carcinoid) occurring in a benign cystic teratoma of the testicle. J. Ural., 72: 892, 1954. 5. Berkheiser, S. W.: Carcinoid tumor of the testis occurring in a cystic teratoma of the testis. J. Ural., 82: 352, 1959. 6. Black, W. C., III: Enterochromaffin cell types in corresponding carcinoid tumors. Lab. Invest., 19: 473, 1968. 7. Pearse, A. G. E.: The APUD cell concept and its implications in pathology. Path. Annu., 9: 27, 1974.