THE JOURNAL OF UROLOGY
Vol.
Copyri1;ht © 1975 by The Williams & Wilkins Co.
II"f,
truly
Printed in U.S.A.
PRIMARY CARCINOID TUMOR OF THE URETHRA H. 0. SYLORA, H. M. DIAMOND, M. KAUFMAN, FRANCIS STRAUS, II
AND
E. S. LYON
From the Departments of Surgery (Urology) and Pathology, University of Chicago, Chicago, Illinois
ABSTRACT
The first reported case of a carcinoid tumor of the urinary tract is presented. Because of rapid local recurrence and distant spread early radical surgery is recommended. Several methods of palliation of the carcinoid syndrome-surgical, pharmacologic and chemotherapeutic-are discussed. Since the first gross description of an appendiceal carcinoid tumor by Merling in 1838 this endocrine malignancy has captivated the imagination of physicians. 1 Wilson and associates recently reviewed the subject and analyzed 3,718 cases.2 While most carcinoid tumors arise in the gastrointestinal tract and occasionally in the bronchial tree and ovaries, this is the first reported case of a carcinoid tumor arising in the urinary tract. Having first arisen in the urethra the primary lesion and recurrences were originally labeled transitional cell carcinoma. However, with the development of extensive hepatic involvement and consequent carcinoid syndrome, a retrospective analysis of all histologic material confirmed the correct tissue diagnosis. CASE REPORT
Admission 1. A 47-year-old man (112-40-49) was first seen at our hospital in October 1971 with a 3-month history of a non-tender, palpable mass located ventrally on the mid-penile shaft. The lesion measured 0.5 by 0.5 by 0.8 cm. Physical examination and metastatic evaluation were negative. Urethroscopy revealed a firm mass in the midpenile urethra. Excisional biopsy was performed using a Kerrison punch and the pathologic diagnosis was infiltrating transitional cell carcinoma (fig. 1, A). The patient underwent segmental resection of the mid-penile urethra with a primary anastomosis. At this time 2 small pimple-like lesions biopsied at the fossa navicularis yielded the same diagnosis. Admission 2. In December 1971 the patient was readmitted to the hospital for routine followup examination. Again, urethroscopy revealed a 1 cm. nodule adjacent to the fossa navicularis. On biopsy the pathologic diagnosis was again transitional cell carcinoma. Penectomy was recommended but refused by the patient. Instead a penile urethrectomy and penoscrotal urethrostomy were performed. Admission 3. In April 1972 the patient was again admitted to the hospital for re-evaluation. CystosAccepted for publication December 6, 1974. 150
copy revealed multiple small papillomas in the bladder. Biopsy of these lesions was again interpreted as transitional cell carcinoma. There was no clinical suspicion of metastatic disease. The bladder findings prompted a 6-week outpatient course of thio-tepa bladder instillations (60 mg. in 60 cc water weekly). Followup cystoscopy in June was normal. Admission 4. In October 1972 the patient was found to have a palpable lymph node in the right groin and was readmitted to the hospital. Cystourethroscopy and metastatic evaluation were negative. A lymph node biopsy was performed and the pathologic diagnosis was metastatic transitional cell carcinoma (fig. 1, B). The patient underwent right radical groin dissection but no tumor was found in 51 lymph nodes. Admission 5. The patient was admitted to the hospital for the last time in January 1973. At that time he complained of low back pain referred to the shoulders associated with facial flushing, sweating, diarrhea and weakness 2 to 3 months in duration. The patient had lost 20 pounds in the 2 to 3 weeks prior to hospitalization. In addition, he volunteered that alcohol ingestion often triggered the attacks of pain, flushing, sweating and weakness. His face and neck were edematous and erythematous. The liver was grossly enlarged, hard and nodular. Palpation of the liver on several occasions triggered waves of pain, flushing and sweating. Laboratory examinations, including hemograms, urinalysis, serum electrolytes, blood urea nitrogen, creatinine, glucose, calcium, phosphate, collagen battery and urinary porphyrins, were normal. Roentgenographic examinations of the chest, skeletal system and the entire alimentary tract were normal. An isotope bone scan was normal. For the first time liver function tests were elevated and isotope liver scan demonstrated extensive hepatic involvement. Twenty-four hour urine collections for quantitative 5-HIAA were elevated on 2 occasions-130 mg. per 24 hours (normal 1 to 7 mg. per 24 hours). Likewise serum serotonin levels were elevated on 2 measurements0.43 mcg. per cc (normal 0.05 to 0.20 mcg. per cc). An open liver biopsy confirmed the clinical
PRIMARY CARC!NOID TUMOR OF URETHRA
151
F1G. 1. A, first submucosa! mid-urethral mass is shown beneath intact transitional mucosa. Tumor consists broad sheets and alveolar nests of rather regular closely packed palely eosinophilic cells with moderately vesicular even nuclei without evident mitoses. Irregular amounts of collagenized tumor stroma accompany tumor. metastatic periurethral tumor nearly replacing inguinal lymph node is similar in morphology to part A except few are now evident. H & E, reduced from >< 180.
impression of a carcinoid a1
Vol.
Copyri1;ht © 1975 by The Williams & Wilkins Co.
II"f,
truly
Printed in U.S.A.
PRIMARY CARCINOID TUMOR OF THE URETHRA H. 0. SYLORA, H. M. DIAMOND, M. KAUFMAN, FRANCIS STRAUS, II
AND
E. S. LYON
From the Departments of Surgery (Urology) and Pathology, University of Chicago, Chicago, Illinois
ABSTRACT
The first reported case of a carcinoid tumor of the urinary tract is presented. Because of rapid local recurrence and distant spread early radical surgery is recommended. Several methods of palliation of the carcinoid syndrome-surgical, pharmacologic and chemotherapeutic-are discussed. Since the first gross description of an appendiceal carcinoid tumor by Merling in 1838 this endocrine malignancy has captivated the imagination of physicians. 1 Wilson and associates recently reviewed the subject and analyzed 3,718 cases.2 While most carcinoid tumors arise in the gastrointestinal tract and occasionally in the bronchial tree and ovaries, this is the first reported case of a carcinoid tumor arising in the urinary tract. Having first arisen in the urethra the primary lesion and recurrences were originally labeled transitional cell carcinoma. However, with the development of extensive hepatic involvement and consequent carcinoid syndrome, a retrospective analysis of all histologic material confirmed the correct tissue diagnosis. CASE REPORT
Admission 1. A 47-year-old man (112-40-49) was first seen at our hospital in October 1971 with a 3-month history of a non-tender, palpable mass located ventrally on the mid-penile shaft. The lesion measured 0.5 by 0.5 by 0.8 cm. Physical examination and metastatic evaluation were negative. Urethroscopy revealed a firm mass in the midpenile urethra. Excisional biopsy was performed using a Kerrison punch and the pathologic diagnosis was infiltrating transitional cell carcinoma (fig. 1, A). The patient underwent segmental resection of the mid-penile urethra with a primary anastomosis. At this time 2 small pimple-like lesions biopsied at the fossa navicularis yielded the same diagnosis. Admission 2. In December 1971 the patient was readmitted to the hospital for routine followup examination. Again, urethroscopy revealed a 1 cm. nodule adjacent to the fossa navicularis. On biopsy the pathologic diagnosis was again transitional cell carcinoma. Penectomy was recommended but refused by the patient. Instead a penile urethrectomy and penoscrotal urethrostomy were performed. Admission 3. In April 1972 the patient was again admitted to the hospital for re-evaluation. CystosAccepted for publication December 6, 1974. 150
copy revealed multiple small papillomas in the bladder. Biopsy of these lesions was again interpreted as transitional cell carcinoma. There was no clinical suspicion of metastatic disease. The bladder findings prompted a 6-week outpatient course of thio-tepa bladder instillations (60 mg. in 60 cc water weekly). Followup cystoscopy in June was normal. Admission 4. In October 1972 the patient was found to have a palpable lymph node in the right groin and was readmitted to the hospital. Cystourethroscopy and metastatic evaluation were negative. A lymph node biopsy was performed and the pathologic diagnosis was metastatic transitional cell carcinoma (fig. 1, B). The patient underwent right radical groin dissection but no tumor was found in 51 lymph nodes. Admission 5. The patient was admitted to the hospital for the last time in January 1973. At that time he complained of low back pain referred to the shoulders associated with facial flushing, sweating, diarrhea and weakness 2 to 3 months in duration. The patient had lost 20 pounds in the 2 to 3 weeks prior to hospitalization. In addition, he volunteered that alcohol ingestion often triggered the attacks of pain, flushing, sweating and weakness. His face and neck were edematous and erythematous. The liver was grossly enlarged, hard and nodular. Palpation of the liver on several occasions triggered waves of pain, flushing and sweating. Laboratory examinations, including hemograms, urinalysis, serum electrolytes, blood urea nitrogen, creatinine, glucose, calcium, phosphate, collagen battery and urinary porphyrins, were normal. Roentgenographic examinations of the chest, skeletal system and the entire alimentary tract were normal. An isotope bone scan was normal. For the first time liver function tests were elevated and isotope liver scan demonstrated extensive hepatic involvement. Twenty-four hour urine collections for quantitative 5-HIAA were elevated on 2 occasions-130 mg. per 24 hours (normal 1 to 7 mg. per 24 hours). Likewise serum serotonin levels were elevated on 2 measurements0.43 mcg. per cc (normal 0.05 to 0.20 mcg. per cc). An open liver biopsy confirmed the clinical
PRIMARY CARC!NOID TUMOR OF URETHRA
151
F1G. 1. A, first submucosa! mid-urethral mass is shown beneath intact transitional mucosa. Tumor consists broad sheets and alveolar nests of rather regular closely packed palely eosinophilic cells with moderately vesicular even nuclei without evident mitoses. Irregular amounts of collagenized tumor stroma accompany tumor. metastatic periurethral tumor nearly replacing inguinal lymph node is similar in morphology to part A except few are now evident. H & E, reduced from >< 180.
impression of a carcinoid a1
