ADENOCARCINOMA JOHNNY WALTER
B. ROY, M.D. E. BAUMANN,
OF RETE TESTIS
M.D.
TERRY M. LEWIS, M.D. ALY FAHMY, M.D. JAN PITHA,
M.D.
From the Section of Urology and Department Veterans Administration Hospital, Oklahoma
of Pathology, City, Oklahoma
ABSTRACTAdenocarcinoma of the rete testis is a rare tumor of the genital tract. An advanced case of adenocarcinoma thought to arise from the rete testis is presented. We believe this is the eighteenth reported case.
Tumors of the testis are generally classified into two categories, germinal and nongerminal.’ Nongerminal tumors constitute approximately 13 per cent of all testicular neoplasms. Carcinoma of the rete testis is of the nongerminal variety and is one of the rare tumors of the genital tract. The tumor arises from the rete tubules of the mediastinum testis. Case Report A sixty-two-year-old black man was seen on August 18, 1975, with a draining scrotal sinus and a urethral fistula. The patient had a ten-year history of urethral stricture which required almost monthly dilatation during the previous year. In February, 1975, he had a “scrotal abscess” which was treated with drainage and antibiotics. Since then he required more frequent dilatations. Approximately two weeks before coming to this hospital, swelling and pain in the left side of the scrotum recurred. The swelling drained spontaneously one day prior to admission. The patient appeared to be chronically ill, cachectic, and had urethral induration and a draining left scrotum. On admission his blood urea nitrogen (BUN) was 17 and creatinine 1.4 mg./lOO ml.; there was marked anemia. On the following day the patient underwent incision and drainage of the scrotum. Cystoscopy at that
270
time showed a mid-penile stricture with a papillary neoplasm involving the penile urethra proximal to the stricture, the prostatic urethra, and the bladder neck area. Biopsies taken showed a papillary adenocarcinoma. Antibiotics and localized therapy were begun in an attempt to clear the infection. Workup revealed normal findings on intravenous pyelogram, and bone survey for metastatic disease was negative. The liver and spleen scans were unremarkable. Drainage from the scrotum persisted, and two weeks after admission the patient’s blood urea nitrogen began rising. On September 5, 1975, attempts at ureteral catheterization were unsuccessful. At that time the tumor was noted to extend much further into the bladder, involving the entire trigonal mucosa with trigonal elevation and edema. Radiation therapy was begun, but the patient became lethargic with BUN rising up to 200, and with persistent infection and ureteral obstruction. He died of renal failure forty days after admission. At autopsy the scrotum, penis, prostate, bladder, seminal vesicles, rectum, and pubic bones were resected in one block. The urinary bladder was pressure-inflated with 10 per cent formalin and the external urinary meatus at the prepuce clamped. A close-up view of the bisected specimen showed the artefactually distended bladder, urethra, and prepuce (Fig. 1).
UROLOGY
/ SEPTEMBER1979
/ VOLUMEXIV,
NUMBER3
A large 110 by 8 cm.) tumor replaced the left testicle. It was extensively necrotic and was connected to multiple sinuses draining through the scrotal &in. The infiltration extended around the prostatic urethra to involve the area of the trigone a!rd ureteral orifices in the form of papillary e’kerescences. The right testicle was atrophic but uninvolved. The rest of the bladder, the ureters, and the kidneys were unremarkable except for inflammatory changes and moderat,; bilateral pyelonephritis. Microscopicall! , the tumor in all sites was a papillary
FIGURE 1 Close-up view showing total replacement of I!& testicle by tumor, papillary excrescences in membrxnous urethra, and infiltration of perineum and trigonc>.
adenocarcinoma (Fig. 2A). The cells showed variation in size; nuclei were hyperchromatic, and atypical mitoses were present (Fig. 2B). Branching papillae were often present in places where glandular structures were obse,*ved. Metastases were found microscopically only in the lymph nodes surrounding the right C’(Wtmon iliac and left internal iliac arteries. Comment An informative historical review of the subject was published in 1959 by Schoen and Itush who analyzed the 12 cases reported sir&c: the original description of the tumor by Curhug in 1853.3 Their analysis of the 13 cases (inc+Iding their own) revealed that adenocarcinoma of the rete testis is a highly malignant tumor; half of the patients who were followed were dead within one year of the establishment of the diagnosis. Later, 4 other cases were reprtrt,:d.4V7 Recently, Gisser et al. * reanalyzed SchapirJi and Engel’s case (the fifteenth) and speculateci tbout the possible significance of the finding at autopsy of a concomitant severe pulmonary asbestosis. They also provided a more’ r cent review of the literature. Adenocarcinoma of the rete testis shovm’s no age predilection (age range from twenty-one to eighty years). Tumors were almost vqually divided between the right and left side. ;ind 1 patient had bilateral tumors.” The tumors usually were present as a testicular mass often associated with a hydrocele.8 Criteria &I*- the diagnosis of an adenocarcinoma arising fro~o the rete testis were discussed in 1945 by F~*e~~:and
small cystic form,~tions, FIGURE 2. Photomicrographs: (A) low-power view of papillary nature of tumor, occasional glandular structures, and focal necrosis; (B) high-power view of branching papillae with v jllular pleomorphxsm and atypical mitoses.
LTTHOLOGY i
SEPTEMBER1979
/ VOLUMEXIV,
NUMBER3
1,l
Hunters and later by others.2,5,7,‘0,11 Teilum’s’O criteria characterize the tumor as a multicystic papillary adenocarcinoma with the papillary processes, a vascular core, and a tendency toward acinar arrangement. Both this lesion and Sertoli cell carcinoma arise from nonspermatic epithelium and generally have an intracystic papillary pattern, so that a sharp distinction between the two cannot always be made.“,12 It is equally important to distinguish them from teratoma and mesothelioma by extensive sampling of the lesion, and also from metastatic tumors. The lesion in our patient fits the gross and microscopic descriptions of an adenocarcinoma of the rete testis. Nonhistologic criteria as proposed by Feek and Hunter can be met only in cases with less advanced growth. Stringent criteria under “ideal conditions” include:2 (1) a maximum involvement of the mediastinum testis and only minimum involvement of the corpus; (2) evidence of “outward” rather than “inward” growth horn the mediastinum as manifested by “no more than moderate compression of normal tubules;” and (3) growth outside the testis into the sac of the tunica vaginalis, but sparing the parietal layer of the tunica. Obviously our case was advanced too far to judge it exclusively on its gross characteristics. Its microscopic appearance, however, makes it most likely to be a papillary adenocarcinoma of rete testis derivation. In the differential diagnosis, the possibility of an endometrial carcinoma, originating from the prostate, was contemplated. However, because the degree of gross tumor involvement of the left testicle was far greater than in any other part of the affected organs, this possibility was regarded as unconvincing. There was no histologic evidence of a teratoma, and no other primary tumor was found at autopsy. A test for human chorionic gonadotropin was not performed.
272
The unusual
ably
the
urethral
presentation
eighteenth fistula
of this case (prob-
to be reported),
and scrotal
“abscess,”
with is note-
worthy; and its definitive diagnosis was only established post mortem. The long history of uretheral stricture disease at first pointed to a primary urethral lesion. Retrospectively we postulate that extension of the testicular tumor all along may have been the cause of this patient’s urethral stricture problems. The infiltration of the trigone and eventual bilateral ureteral obstruction are distinctly uncommon complications of this tumor. 921 N.E. 13th Street Oklahoma City, Oklahoma 73104 (DR. ROY)
References 1. Whitmore WF: Tumors of the testis, in Campbell, MF, and Harrison TH. Eds: Uroloev. 3rd ed.. Philadelnhia.I , W. B. Saunders do.,’ 1970, p. 12137’ ’ ’ 2. Schoen SS, and Rush BF, Jr: Adenocarcinoma of the rete testis, J. Urol. 82: 356 (1959). 3. Curling TB: Observations on cystic disease of the testicle, Medico-chir. Trans. 36: 449 (1853). 4. Desberg D, and Tanno V: Adenocarcinoma of the rete testis, J. Urol. 91: 87 (1964). 5. Schapira HE, and Engel M: Adenocarcinoma of rete testis, NY State J. Med. 72: 1283 (1972). 6. Whitehead ED, Valensi QJ, and Brown JS: Adenocarcinoma of the rete testis, J. Urol. 107: 992 (1972). 7. Turner RW, and Williamson J: Adenocarcinoma of the rete testis, report of a case, ibid. 169: 850 (1973). 8. Gisser SD, Nayak S, Kaneko M, and Tchertkoff V: Adenocarcinoma of the rete testis: a review of the literature and presentation of a case associated with asbestosis, Human Pathol. 8: 219 (1977). 9. Feek JD, and Hunter WC: Papillary carcinoma arising horn rete testis, Arch. Pathol. 40: 399 (1945). 10. Teilum G: Special tumors of Ovary and Testis and Related Extragonadal Lesions. Comparative pathology and histological identification, Philadelphia, J. B. Lippincott Co., 1971, p. 467. 11. Shillitoe AJ: Caminomaofthe rete testis, J. Pathol. Bacterial. 64: 659 (1952). 12. Willis RA: Pathology of Tumors, 4th ed., London, Butterworth and Co., Ltd., 1967, p. 589.
UROLOGY
/
SEPTEMBER
1979
/
VOLUME
XIV,
NUMBER
3
B. ROY, M.D. E. BAUMANN,
OF RETE TESTIS
M.D.
TERRY M. LEWIS, M.D. ALY FAHMY, M.D. JAN PITHA,
M.D.
From the Section of Urology and Department Veterans Administration Hospital, Oklahoma
of Pathology, City, Oklahoma
ABSTRACTAdenocarcinoma of the rete testis is a rare tumor of the genital tract. An advanced case of adenocarcinoma thought to arise from the rete testis is presented. We believe this is the eighteenth reported case.
Tumors of the testis are generally classified into two categories, germinal and nongerminal.’ Nongerminal tumors constitute approximately 13 per cent of all testicular neoplasms. Carcinoma of the rete testis is of the nongerminal variety and is one of the rare tumors of the genital tract. The tumor arises from the rete tubules of the mediastinum testis. Case Report A sixty-two-year-old black man was seen on August 18, 1975, with a draining scrotal sinus and a urethral fistula. The patient had a ten-year history of urethral stricture which required almost monthly dilatation during the previous year. In February, 1975, he had a “scrotal abscess” which was treated with drainage and antibiotics. Since then he required more frequent dilatations. Approximately two weeks before coming to this hospital, swelling and pain in the left side of the scrotum recurred. The swelling drained spontaneously one day prior to admission. The patient appeared to be chronically ill, cachectic, and had urethral induration and a draining left scrotum. On admission his blood urea nitrogen (BUN) was 17 and creatinine 1.4 mg./lOO ml.; there was marked anemia. On the following day the patient underwent incision and drainage of the scrotum. Cystoscopy at that
270
time showed a mid-penile stricture with a papillary neoplasm involving the penile urethra proximal to the stricture, the prostatic urethra, and the bladder neck area. Biopsies taken showed a papillary adenocarcinoma. Antibiotics and localized therapy were begun in an attempt to clear the infection. Workup revealed normal findings on intravenous pyelogram, and bone survey for metastatic disease was negative. The liver and spleen scans were unremarkable. Drainage from the scrotum persisted, and two weeks after admission the patient’s blood urea nitrogen began rising. On September 5, 1975, attempts at ureteral catheterization were unsuccessful. At that time the tumor was noted to extend much further into the bladder, involving the entire trigonal mucosa with trigonal elevation and edema. Radiation therapy was begun, but the patient became lethargic with BUN rising up to 200, and with persistent infection and ureteral obstruction. He died of renal failure forty days after admission. At autopsy the scrotum, penis, prostate, bladder, seminal vesicles, rectum, and pubic bones were resected in one block. The urinary bladder was pressure-inflated with 10 per cent formalin and the external urinary meatus at the prepuce clamped. A close-up view of the bisected specimen showed the artefactually distended bladder, urethra, and prepuce (Fig. 1).
UROLOGY
/ SEPTEMBER1979
/ VOLUMEXIV,
NUMBER3
A large 110 by 8 cm.) tumor replaced the left testicle. It was extensively necrotic and was connected to multiple sinuses draining through the scrotal &in. The infiltration extended around the prostatic urethra to involve the area of the trigone a!rd ureteral orifices in the form of papillary e’kerescences. The right testicle was atrophic but uninvolved. The rest of the bladder, the ureters, and the kidneys were unremarkable except for inflammatory changes and moderat,; bilateral pyelonephritis. Microscopicall! , the tumor in all sites was a papillary
FIGURE 1 Close-up view showing total replacement of I!& testicle by tumor, papillary excrescences in membrxnous urethra, and infiltration of perineum and trigonc>.
adenocarcinoma (Fig. 2A). The cells showed variation in size; nuclei were hyperchromatic, and atypical mitoses were present (Fig. 2B). Branching papillae were often present in places where glandular structures were obse,*ved. Metastases were found microscopically only in the lymph nodes surrounding the right C’(Wtmon iliac and left internal iliac arteries. Comment An informative historical review of the subject was published in 1959 by Schoen and Itush who analyzed the 12 cases reported sir&c: the original description of the tumor by Curhug in 1853.3 Their analysis of the 13 cases (inc+Iding their own) revealed that adenocarcinoma of the rete testis is a highly malignant tumor; half of the patients who were followed were dead within one year of the establishment of the diagnosis. Later, 4 other cases were reprtrt,:d.4V7 Recently, Gisser et al. * reanalyzed SchapirJi and Engel’s case (the fifteenth) and speculateci tbout the possible significance of the finding at autopsy of a concomitant severe pulmonary asbestosis. They also provided a more’ r cent review of the literature. Adenocarcinoma of the rete testis shovm’s no age predilection (age range from twenty-one to eighty years). Tumors were almost vqually divided between the right and left side. ;ind 1 patient had bilateral tumors.” The tumors usually were present as a testicular mass often associated with a hydrocele.8 Criteria &I*- the diagnosis of an adenocarcinoma arising fro~o the rete testis were discussed in 1945 by F~*e~~:and
small cystic form,~tions, FIGURE 2. Photomicrographs: (A) low-power view of papillary nature of tumor, occasional glandular structures, and focal necrosis; (B) high-power view of branching papillae with v jllular pleomorphxsm and atypical mitoses.
LTTHOLOGY i
SEPTEMBER1979
/ VOLUMEXIV,
NUMBER3
1,l
Hunters and later by others.2,5,7,‘0,11 Teilum’s’O criteria characterize the tumor as a multicystic papillary adenocarcinoma with the papillary processes, a vascular core, and a tendency toward acinar arrangement. Both this lesion and Sertoli cell carcinoma arise from nonspermatic epithelium and generally have an intracystic papillary pattern, so that a sharp distinction between the two cannot always be made.“,12 It is equally important to distinguish them from teratoma and mesothelioma by extensive sampling of the lesion, and also from metastatic tumors. The lesion in our patient fits the gross and microscopic descriptions of an adenocarcinoma of the rete testis. Nonhistologic criteria as proposed by Feek and Hunter can be met only in cases with less advanced growth. Stringent criteria under “ideal conditions” include:2 (1) a maximum involvement of the mediastinum testis and only minimum involvement of the corpus; (2) evidence of “outward” rather than “inward” growth horn the mediastinum as manifested by “no more than moderate compression of normal tubules;” and (3) growth outside the testis into the sac of the tunica vaginalis, but sparing the parietal layer of the tunica. Obviously our case was advanced too far to judge it exclusively on its gross characteristics. Its microscopic appearance, however, makes it most likely to be a papillary adenocarcinoma of rete testis derivation. In the differential diagnosis, the possibility of an endometrial carcinoma, originating from the prostate, was contemplated. However, because the degree of gross tumor involvement of the left testicle was far greater than in any other part of the affected organs, this possibility was regarded as unconvincing. There was no histologic evidence of a teratoma, and no other primary tumor was found at autopsy. A test for human chorionic gonadotropin was not performed.
272
The unusual
ably
the
urethral
presentation
eighteenth fistula
of this case (prob-
to be reported),
and scrotal
“abscess,”
with is note-
worthy; and its definitive diagnosis was only established post mortem. The long history of uretheral stricture disease at first pointed to a primary urethral lesion. Retrospectively we postulate that extension of the testicular tumor all along may have been the cause of this patient’s urethral stricture problems. The infiltration of the trigone and eventual bilateral ureteral obstruction are distinctly uncommon complications of this tumor. 921 N.E. 13th Street Oklahoma City, Oklahoma 73104 (DR. ROY)
References 1. Whitmore WF: Tumors of the testis, in Campbell, MF, and Harrison TH. Eds: Uroloev. 3rd ed.. Philadelnhia.I , W. B. Saunders do.,’ 1970, p. 12137’ ’ ’ 2. Schoen SS, and Rush BF, Jr: Adenocarcinoma of the rete testis, J. Urol. 82: 356 (1959). 3. Curling TB: Observations on cystic disease of the testicle, Medico-chir. Trans. 36: 449 (1853). 4. Desberg D, and Tanno V: Adenocarcinoma of the rete testis, J. Urol. 91: 87 (1964). 5. Schapira HE, and Engel M: Adenocarcinoma of rete testis, NY State J. Med. 72: 1283 (1972). 6. Whitehead ED, Valensi QJ, and Brown JS: Adenocarcinoma of the rete testis, J. Urol. 107: 992 (1972). 7. Turner RW, and Williamson J: Adenocarcinoma of the rete testis, report of a case, ibid. 169: 850 (1973). 8. Gisser SD, Nayak S, Kaneko M, and Tchertkoff V: Adenocarcinoma of the rete testis: a review of the literature and presentation of a case associated with asbestosis, Human Pathol. 8: 219 (1977). 9. Feek JD, and Hunter WC: Papillary carcinoma arising horn rete testis, Arch. Pathol. 40: 399 (1945). 10. Teilum G: Special tumors of Ovary and Testis and Related Extragonadal Lesions. Comparative pathology and histological identification, Philadelphia, J. B. Lippincott Co., 1971, p. 467. 11. Shillitoe AJ: Caminomaofthe rete testis, J. Pathol. Bacterial. 64: 659 (1952). 12. Willis RA: Pathology of Tumors, 4th ed., London, Butterworth and Co., Ltd., 1967, p. 589.
UROLOGY
/
SEPTEMBER
1979
/
VOLUME
XIV,
NUMBER
3
