Papillary adenocarcinoma of therets testis: A case report and review of the literature

CASE R E P O R T I

PAPILLARY ADENOCARCINOMA OF THE RETE TESTIS: A CASE REPORT AND REVIEW OF THE LITERATURE JOHN P. STEIN, M.D. JOHN A. FREEMAN, M.D. DAVID ESRIG, M.D. PARAKRAMA T. CHANDRASOMA, M.D. DONALD G. SKINNER, M.D. From the Department of Urology and Clinical Pathology, University of Southern California School of Medicine, Los Angeles, California

ABSTRACT--Adenocarcinoma of the rete testis is a rare neoplasm with 40 reported cases in the world literature to our knowledge. Although the natural history of this tumor is not well known, it appears highly malignant even in localized forms. Aden0carcinoma of the rete testis is highly resistant to adjuvant radiotherapy and any known chemotherapeutic regimen. We recommend radical orchiectomy followed by a retroperitoneal lymphadenectomy as the treatment for clinically localized Stage A tumors and minimal or microscopic Stage B disease. We report a case of adenocarcinoma of the rete testis and review the world literature.

Primary adenocarcinoma of the fete testis is a rare testicular neoplasm with 40 previously reported cases in the literature to our knowledge. 1-39 The diagnosis of this t u m o r is based on documenting its origin within the rete testis. Without early diagnosis and treatment, the prognosis is uniformly poor. Treatment of localized clinical Stage A adenocarcinoma of the rete testis in the past has included orchiectomy followed by adjuvant radiation or chemotherapy, or both, with a reported mortality of nearly 50% at 2 years. 4° Because of the poor response of this tumor to adjuvant therapy, some authors propose that radical o r c h i e c t o m y f o l l o w e d by a retroperitoneal lymph node dissection provides optimal treatment for localized disease. 26,31,37,38 We herein report a case of Stage A adenocarcinoma of the rete testis treated with a radical orchiectomy and retroperitoneal lymph node dissection (RPLND). We summarize the world literature to describe the presentation, biologic attributes, and treatment of this unique neoplasm more accurately. Presented at the annual meeting of American Urological Association Western Section, Palm Desert, November 14-18, 1993. 5ubmitted: March 25, 1994, accepted (with revisions): May 18, 1994

588

CASE REPORT A 64-year-old Korean mau m o n t h history of right scrol ment. The patient denied an history of maldescent, infect testicles and was otherwise h icant past medical or surgical examination, the only perti~ was a 2 x 2 cm smooth, hard, mass on the superior pole of left testicle was normal. The patient's workup inclu the testicles, which revealed cystic, intratesticular mass lc aspect of the right testicle, , appearing testicular parench cele was also present. The let Laboratory studies included human ehorionic gonadotro g SMAC, c o m p l e t e blood co Chest radiograph and electro normal. A right inguinal exploratio control of the spermatic co~ area of brownish discoloratio3 of the testicle. This was incis olate viscous fluid was drair UROLOGY~ / Oc~ros~ 195

1. Papillary configuration of tumor with pseudocation of malignant epithelial cells (hematoxylin i!~osin, original magnification ×200).

FIGURE 2. Epithelial cells with moderate to marked pleomorphism (hematoxylin ancl eosin, original magnification ×400).

i x 2 cm mass in the testicle. A radical orchiec}ywas then performed. he pathologic specimen consisted of the right iis and spermatic cord. The testis measured 6 x cm and had a smooth surface. The spermatic was 8 cm long and 1.5 cm in diameter. There at the proximal pole of the testis igin of the vas deferens. The insmooth except for scattered paps that measured 0.2 cm in great]e wall was thin and intact. The testis was normal. unination of the cystic structure ose from the fete testis (Figs. 1 d by a flat cuboidal epithelium in • In the areas of the papillary exr a n g epithelium was complex, ~ g stratified and thrown up into complex papil~ a n d glandular structures. The lining epithelial ~is showed cytologic features of malignancy, with enlargement, nuclear enlargement and hyper~0masia, granular chromatin distribution, and ~rninent nucteoli. Mitotic figures were present, i!f not n u m e r o u s . There was i m p i n g e m e n t of l.eSe malignant glands into the fibrous wall, but iere was no penetration of the fibrous wall at any ii~t. The histologic features were those of a well~ferentiated papillary cystadenocarcinoma of the }~ testis There was no extension of tumor bemd the cystic mass, with the surgical margins ~d tunica free of tumor. ii}urther workup included a computed axial tomoe~m of the chest, abdomen, and pelvis. No evi~nce of metastatic disease was found. Subsequently, !e patient u n d e r w e n t a right thoracoabdominal

retroperitoneal lymph node dissection with a unilateral pelvic node dissection for clinical Stage A disease. There was no gross or microscopic evidence of metastatic disease, placing the patient in the pathologic Stage A category. The patient received no adj uvant therapy and is currently doing well 4 years postoperatively without evidence of disease.

~

~ROLOGY ~ / OCTOBE~ 1994 / VOLUME44, NUMBER4

COMMENT Adenocarcinoma of the fete testis is an extremely rare testicular neoplasm first reported by Feek and Hunter in 1945.1 This unique neoplasm is characterized by strict pathologic criteria. A recent case report and review of carcinoma of the fete testis referenced 43 cases in the literature; however, not all cases truly meet the pathologic criteria of primary adenocarcinoma of the rete testis) 9 Due to its rarity, it is unlikely that any single institution will accumulate sufficient cases to describe accurately the natural history and biologic capabilities of this tumor. With a review of the literature, some in-sight may be gained into the presentation, natural history, treatment, and eventual outcome of adenocarcinoma of the rete testis (Table I). PRESENZAIION

Adenocarcinoma of the fete testis appears to present in an older age group than other testicular carcinomas. The age at presentation ranged from 17 y e a r s 1°'15 to 91 years, 32 with a mean age of 54 years. There were 22 patients (51%) over the age of 55 years and 1.5 patients (35%) over the age of 60 years at the time of presentation. The condition affected predominantly white patients, with only 1 b l a c k 23 a n d 3 oriental p a t i e n t s d e s c r i b e d , 25y

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591

including the present case. Adenocarcinoma of the rete testis showed a slight predilection for the right side in 23 patients (59%). A single case was bilateral. 7 Patients with adenocarcinoma of the fete testis most commonly present with painless scrotal enlargement. Of the documented cases, 34 of 36 patients (94%) presented with complaints of a scrotal mass and associated scrotal pain was found in only 11 patients (27%). Symptoms of metastases were present in 6 patients (19%) at presentation and included: hemiplegia and incontinence, ~lumbar back pain, 2,24abdominal pain with constitutional symptoms, n lower extremity swelling and an inguinal mass, 18 and a draining scrotal s i n u s . 23 Adenocarcinoma of the rete testis is commonly associated with other scrotal abnormalities. The most common disorder accompanying this tumor is a hydrocele found in 19 patients (46%). In a number of reports the hydrocele contained serosanguineous or bloody fluid contents, 1,~°'n'2<27m including the present case. Although most patients developed tumors in a previously normal testis, a few documented cases were associated with preexisting testicular abnormalities, including cryptorchidism
The diagnosis of carcinoma of the fete testis is difficult and made only pathologically. The generally accepted criteria for the diagnosis of adenocarcinoma of the rete testis includes: (1) location of the tumor within the posterior aspect of the testis, (2) exclusion of any germ cell, nongerm cell, or other neoplasm either locally or at a distant site, (3) a histologic pattern compatible with that of malignant tumor of rete origin, and (4) a transition between normal fete testis to neoplastic structures in the rete testis. 1,3,24,27,32 The differential diagnosis includes both benign and malignant tumors of the testis and the adnexa. There are no k n o w n laboratory markers for adenocarcinoma of the fete testis, with s e r u m alpha-fetoprotein and b e t a - h u m a n chorionic gonadotropin abvays within normal limits. Gynecomastia has never been reported with adenocarcinoma of the rete testis. STAGING

Staging of adenocarcinoma of the rete testis is based on Boden and Gibb's 41 classification of tes-

592

ticular neoplasms. The three stages Stage A, tumor confined to the testic] idence of metastasis; (2) Stage B, tun ticle with only intra-abdominal meta, Stage C, tumor of the testicle with ex! metastasis. Tumors that recurred in thq cision or scrotum, or had extension i imal cut end of the cord, were classit tumors.

Accurate staging of this tumor in early reported cases is limited by poo tion and available technology. Conse, reported cases may have been under.' 41 reported cases of adenocarcinon testis, 25 cases (61%) were clinical St 4 cases (10%) were clinical Stage B ! cases (15%) were clinical Stage C1 time of presentation. There were 6 t whose clinical stage was not reporte( be determined. 7,9,1°,m21 NATURAL HISTORY

Adenocarcinoma of the fete testis is tumor with approximately two thir( cases either presenting with or dev, static disease within 18 months. N{ metastatic disease at presentation s than 10 months. Metastases occur in toneal l y m p h nodes, lung, local s lymph nodes, liver, and bone, in or( ing frequency. Local recurrence is r', scrotal lymphatic vessels have been tients undergoing a previous scroL aspiration c o m m o n l y develop loca tases.10,13,19.27.32 Metastases have also to the supraclavicular lymph node adrenal gland, 2~ bladder and urethra, 2 adrenal gland, thyroid, and pericard TREAIMENT AND RESULTS

Initial treatment for all patients wi noma of the rete testis is radical orct establishes the diagnosis and provide: apy for the primary tumor w i t h o u t , tal lymphatics. Of the 8 patients w local skin recurrence or inguinal a both, all had previous scrotal incisic underscoring the morbidity of scrot~ contrast, no patient who u n d e r w e n chiectomy without scrotal violatim local recurrence or inguinal adenop~ Additional therapy for clinical Sta and Stage C lesions is controversial. various management schemes for cl

U R O L O G Y ~ / O c I o ~ R 1994 / VoLt

TABLE 11. Treatment

Response to treatment for clinical Stage A tumors* Alive Without Metastases

Orchiectomy Orchiectomy and radiation Orchiectomy and chemotherapy Qrchiectomy and radiation and chemotherapy Orchiectomy and RPLND Total patients *Numbers

in

4 (8.5) 2 (5) t (36) . . . 4 (33) 11 (20)

.

Alive With Metastases

Dead

Unknown

t (24) 1 (7) 1 (9)

3 (12) 2 (4) 3 (22)

1 1

2 (21) . . . . 3 (12) 10 (17)

Total Patients

2

2 4 26

parentheses indicate average follow-up in months.

narized in Table II. Radical ored by RPLND offers optimal therith clinically localized Stage A dis:nts treated w i t h o r c h i e c t o m y aD were alive with no evidence of tverage follow-up of 33 months. s (50%) not treated with RPLND mnths from the time of orchiecmr patients (15%) metastases de7e, 65% of evaluable patients with .esions not treated with RPLND died or developed metastases. .tients (18%) with clinical Stage A th radiation therapy with or withy remained free of disease. These e been cured by the orchiectomy ff evaluable patients treated by oremained alive with no evidence of follow-up. Because neither radichemotherapy appear to eradicate [1volume disease and because fer.ess important in this older age vith the typically younger patients mars of the testis, a full bilateral nended. lesions were identified in 4 pation. RPLND was effective in the th small volume retroperitoneal it was used; he remained alive e at 42 months follow-up after re: periaortic metastasis. Radiation effectively on an inguinal metas~t the 12-month follow-up; it was mbination with stilbestrol in a ha died at 2 months. The fourth at an undefined open operation .p but was d o c u m e n t e d to have ;ented witlh clinical Stage C adenofete testis. Five patients died re?y within 14 months (mean, 3.8 aosis. The other patient had no

'~R 1994 / VOLUME4q, NUMBERq.

documented follow-up. At the present time, there appears to be no effective therapy for clinical Stage C tumors of the rete testis. THERAPEUTIC RECOMMENDATIONS

We recommend a full bilateral RPLND for all patients with clinical Stage A.and surgically resectable Stage B disease. The rationale for RPLND is based on its staging value, on the probable coexistence of nodal metastases at the time of presentation, and on its potential therapeutic value. The true incidence or probability of positive nodal disease at presentation is unknown; however, at least 10 of 38 evaluable patients (26%) appeared to have metastatic disease at presentation. To date, the only patient that presented with proven retroperitoneal nodal metastases and treated with RPLND is alive without evidence of disease at 42 months following surgery. The other 4 patients treated with RPLND for clinical Stage A disease have all survived without evidence of disease. At the present time, only 1 of 7 evaluable patients with clinical evidence of metastases at presentation treated with radiation and/or chemotherapy, without RPLND, has survived with evidence of metastases at 12 months. This demonstrates h o w ineffective radiation therapy or chemotherapy is in the treatment of metastatic adenocarc i n o m a of the rete testis. Because of the low morbidity of RPLND, the propensity of adenocarcinoma of the rete testis to metastasize~ and the ineffective treatment of radiation and chemotherapy, m addition to its staging and potential therapeutic value, we believe that RPLND should be offered to the appropriate surgical candidate with clinical Stage A or surgically resectable Stage B disease. CONCLUSION

Adenocarcinoma of the rete testis is an aggressive and extremely malignant tumor of the testis, even in clinically localized forms. Currently, there is little evidence to support the use of adjuvant radiation therapy or chemotherapy other than for palliative

593

purposes. Retroperitoneal lymph node dissection has d e m o n s t r a t e d a p o t e n t i a l t h e r a p e u t i c v a l u e in a l l 5 cases in w h i c h it w a s u s e d , i n c l u d i n g 1 p a t i e n t w i t h p a t h o l o g i c Stage B disease. A l t h o u g h r a d i c a l o r c h i e c t o m y a l o n e m a y also be effective t h e r a p y in s o m e clinical Stage A p a t i e n t s , o n l y 2 o f 6 p a t i e n t s treated in this m a n n e r h a d a d o c u m e n t e d d i s e a s e free s u r v i v a l c o m p a r e d w i t h 100% efficacy in p a tients t r e a t e d w i t h R P L N D . T h e r e is c u r r e n t l y n o w a y to p r e d i c t w h i c h p a t i e n t s will r e s p o n d to orclaiectomy a l o n e a n d w h i c h will r e q u i r e a R P L N D for cure. T h e r e f o r e , w e r e c o m m e n d R P L N D after radical o r c h i e c t o m y for all p a t i e n t s w i t h clinical Stage A a d e n o c a r c i n o m a o f the rete testis as well as p a t i e n t s w i t h s u r g i c a l l y resectable clinical Stage B disease. J o h n R Stein, M.D.

Department of Urology and Clinical Pathology University of Southern California School oJ Medicine Los Angeles, California 90033 REFERENCES 1. Feek jD, and Hunter WC: Papillary carcinoma arising from rete testis. Arch Pathol 40: 339-402, 1945. 2. Scully RE, and Parham AR: Testicular tumors. Interstitial cell and miscellaneous neoplasms. Arch Pathol 46: 229-242, 1948. 3. Badenoch AW, and Dukes CE: Case of adenocarcinoma of rete testis. BrJ Urol 23: 230-232, 1951. 4. Dundon C: Carcinoma of fete testis occurring ten years after orchidopexy. BrJ Urol 24: 58-63, 1952. 5. Shillitoe AJ: Carcinoma of rete testis. J Pathol Bacteriol 64: 650-651, 1952. 6. Willis RA: Pathology of Tumors, 2nd ed, London, Butterworth & Co, 1953, p 577. 7. Willis RA: Pathology of Tumors, 4th ed, London, Butterworth & Co, 1967, p 589. 8. Laird RM: Adenocarcinoma of excretory ducts of testicle. J Urol 72: 904-907, 1954. 9. Fraser WE: Cited by Schoen and Rush. ~ 10. Thurzo R, and Sagi T: Adenopapillares Karzinom des rete testis. Zentralbl Allg Pathol Anat 98: 546-549, 1958. tl. Schoen SS, and Rush BF Jr: Adenocarcinoraa of the fete testis. J Urol 82: 356-363, 1959. 12. Collins DH, and Pugh RCB: The pathology of testicular tumours. BrJ Urol (Suppl) 36: 74, 1964. 13. Desberg D, and Tanno V: Adenocarcinoma of the rete testis. J Urol 91: 87-89, 1964. 14. Nair MB, and Mohankumar V: Carcinoma of the rete testis resembling sertoli cell tumor. IndianJ Pathol Bacteriol 9: 337-339, 1966. 15. Martinazzi M, and Carnevali L: Eadenocarcinoma della fete testis. Riv Anat Pathol Oncol 32: 181--191, 1967. t6. Moghe KV, Agarwal RV, andJunnarkar RV: Histological features of testicular tumours. Indian J Cancer 7: 98-109, 1970. 17. Moghe KV, and Soni PH: Carcinoma of rete testis--a case report. IndianJ Pathot Bacteriol 14: 184-188, 1971. 1.8. Whitehead ED, Valensi QJ, and BrownJS: Adenocarcinoma of the fete testis. J UroI 107: 992-999, 1972.

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19. Schapira HE, and Engel M: Adenocarcinoma ofrd testis. NY StateJ Med 72: 1283-1285, 1972. 20. Turner RW, and WilliamsonJ: Adenocarcinoma ofii re-re testis: a report of a case. J Urol 109: 850-851, 1973. 7 21. Symington T, and Cameron KM: Endocrine andi~ netic legions, in Pugh RCB (Ed): Pathology of the Testis, ~o! don, Blackwell Scientific Publications, 1976, pp 259-303: 22. Mehan DJ, and Chehval MJ: Adenocarcinoma of~a testis. Urology 9: 459-460, 1977. 23. Roy JB, Baumann WE, Lewis TM, Fahmy A, and ~[ii J: Adenocarcinoma of fete testis. Urology 14: 270-272, D,7 24. Jacobellis U, Ricco R, and Ruotolo G: Adenocarcino~ of the rete testis 21 years after orchiopexy: a case report and~i view of the literature. J Urol 125: 429-431, 1981. 25. Winter CC, Puente E, Lai DY, and Sharma HM: lary carcinoma of the fete testis. Urology 18: 168-170,198': 26. Goldstein J, and Moses M: Adenocarcinoma oft{ testis. Urology 18: 298-299, 1981. 27. Fukunaga M, Aizawa S, Furusato M, Akasaka Y,at Machida T: Papillary adenoearcinoma of the fete testis. Ag~ report. Cancer 50: 134-138, 1982. 28. Nocbomovitz LE i and Orenstein JM: Adenocarcin6r of the rete testis: case report, ultrastructural observations, ai clinicopathologic correlates. Am J Surg Pathol 8: 625-fi~ 1984. 29. Sarma DR and Weilbaecher TG: Adenocarcmoma the rete testis. J Surg Oncol 30: 67-71, 1985. 30. Erlandson RA, and Lieberman PH: Paratesticular tUgi in a 44-year-old male. Ultrastruct Pathol 8: 107-t13, 1985 31. Haas GP, Ohorodnik JM, and Farah RN: Cystade~ carcinoma of the fete testis. ,J Urol 137: 1232-1233, 1987. 32. Crisp-Lindgren N, Travers H, Wells MM; and Cawl LP: Papillary adenocarcinoma of the rete testis: autopsy fm ings, histochemistry, immunohistochemistry, ultrastructul and clinical correlations. AmJ Surg Pathol 12: 492-501, 19i 33. Visscher DW, Talerman A, Rivera LR, and Mazur N Adenocarcinoma of the rete testis with a spindle cell co~f nent. A possible metaplastic carcinoma. Cancer 64: 770=7! 1989. 34. Ohigashi T, and Yamamoto I: Adenocarcinoma Qf rete testis. Hinyokika Kiyo 35: 1787-1790, 1989. 35. Watson PH, and Jacob VC: Adenocarcinoma of the/! testis with sertoliform differentiation. Arch Pathol LabN 113: 1169-1171, 1989. 36.. Mrak RE, Husain MM, and Schaefer RF: Ultras!Zt ture of metastatic fete testis adenocarcinoma, Arch Path0lE Meal 114: 84-88, 1990. 37. Burns MW, Clrandler WL, and Krieger JN: Aderlee cinoma of rete testis. Role of inguinal orchiectomy i,p:l retroperitoneal lymph node dissection. Urology 37:57!~5 i 1991. 38. Hode E, Desablens B, Gamier J, Quenum C, Pefii and Abourachid H: Adenocarcinoma of the fete testis Revi of the literature apropos of a new case. Ann Urol 25: 3 ~ 1991. 39. Orozco RE, and Murphy WM: Carcinoma of the,ri testis: case report and review of the literature. J Urol 1{ 974-977, 1993. 40. Gisser SD, Nayak S, Kaneko M, and TcherkoflV: nocarcinoma of the rete testis: a review of the literature ali4,P sentation of a case with associated asbestosis. Hum pathi~l 219-224; 1977. 4i. Boden G, and Gibb R: Radiotherapy and testictdar.ili plasms. Lancet 2: 1195-1197, 1951.

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UROLOGY* / OCTOBER1994 / VoLUt,~44,