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Unusual Penile Malignancies in Circumcised Jewish Men
Vol. 110, Decembe1
THE JovRNAL OF UROLOGY
Copyright© 1973 by The Williams & Wilkins Co.
Printed in U.S.A.
UNUSUAL PENILE MALIGNANCIES IN CIRCUMCISED JEWISH MEN AMIR S. GIRGIS, HARRY BERGMAN, HEDVIG ROSENTHAL AND LYDIA SOLOMON From the Bronx Lebanon Hospital Center, Bronx and the Jewish Memorial Hospital, New York, New York
Malignant tumors of the penis or external urethra are rare in circumcised Jewish men. We have treated 2 such primary malignancies: a primary malignant melanoma of the male urethra and a solitary Kaposi's sarcoma of the penis. We also reviewed 6 reported cases of malignant melanoma of the male urethra'· 2 and 11 cases of Kaposi's sarcoma of the penis. 3 • 4 CASE REPORTS
Case 1. G. W., JMH 239198, a 51-year-old white Jewish man, was hospitalized because of blood staining the underwear 5 weeks in duration. The patient noticed a growth at the external urethral meatus. He had a history of slight discomfort on urination. On examination a bluish tumor was noted through the urethral opening. When the external meatus was everted an irregular indurated growth approximately 1 cm. in diameter was seen at the terminal urethra. The pedicle was not noted and the proximal extension of the tumor could not be detected. The tumor bled easily with gentle palpation. There was no external induration on palpation of the distal penis. No palpable lymph nodes were detected in either groin or femoral triangles. An excretory urogram showed normally bilateral functioning kidneys with incomplete rotation of the left kidney and right pelvic renal ectopia. Radiologically, there was no ureteral deviation or obstruction. Urinalysis did not show any red blood cells. Liver scan showed normal distribution of 198 Au. Preoperative cystoscopy was unsuccessful because of the obstructing mass in the terminal urethra. An excisional biopsy of the urethral tumor through a posterior penile skin incision was performed with general anesthesia, obtaining a good margin of safety. The underlying Buck's fascia was excised to determine the degree 'of infiltration. The biopsy specimen consisted of 2 ovoid seg-
Accepted for publication June 29, 1973. Read at annual meeting of American Urological Association, New York, New York, May 13-17, 1973. 1 Abeshouse, B. S.: Primary and secondary melanoma of the genitourinary tract. Southern Med. J., 51: 994, 1958. Quoted by Weston, P. A. M. and Smith, R. J.: Metastatic melanoma in the bladder and urethra. Brit. J. Surg., 51: 78, 1964. 2 Guinn, G. A. and Ayala, A. G.: Male urethral cancer: report of 15 cases including a primary melanoma. J. Ural., 103: 176, 1970. 3 Cox, J. W., Halprin, K. and Ackerman, A. B.: Kaposi's sarcoma localized to the penis. Arch. Derm., 102: 461, 1970. 4 Summers, J. L., Wilkerson, J.E. and Wegryn, J. F.: Conservative therapy for Kaposi's sarcoma of the external genitalia. J. Ural., 108: 287, 1972.
696
ments of rubbery tissue, measuring 1.5 by 1.0 and 1.2 by 8.0 cm. and showing white cut surfaces with scattered hemorrhagic areas. Microscopic examination revealed a highly cellular infiltrating obviously malignant tumor, consisting of compact groups and sheets of large polygonal cells with vacuolated cytoplasm, conspicuous vesicular nuclei and prominent nucleoli (fig. 1). There was moderate pleomorphism and mitosis was rare. Some coarsely granular brown pigment was in the cells and was most prominent in the hemorrhagic ulcerated areas. The iron stain was negative, thus the possibility of hemoglobin derived pigment was eliminated. The surface squamous epithelium was unremarkable except for an occasional hyperchromatic cell in the basal layer. The tumor extended beneath the well preserved urethral mucosa. In one area occasional pigmented hyperchromatic cells were seen between the cells of the pseudostratified cuboidal urethral epithelium. Biopsy of Buck's fascia was unremarkable. Diagnosis was malignant melanoma of the urethra. After diagnosis was established repeat cystoscopy showed no suspicious tumors in the remaining urethra and bladder. Since the patient refused radical penectomy, partial penectomy with radical lymph node dissection of the groins, external iliacs, superficial and deep femoral and inguinal lymph nodes was done. Forty-four lymph nodes recovered on serial sectioning of the fatty tissue were grossly unremarkable. The amputated distal segment of the penis measured 6 cm. in length and showed the healing distal urethra (fig. 2). The glans penis and corpora cavernosa were grossly unremarkable. Microscopically, no residual tumor was found in the healing previously resected urethra but, in the vicinity of the biopsy site on the glans penis, there were scattered areas of active junctional changes at the margins of the rete ridges (fig. 3, A). One of these areas showed large Paget's cells extending to the stratum granulosum and was accompanied by a rather dense mononuclear leukocytic infiltration of the adjacent dermis. More than 300 sections examined from the 44 lymph nodes were negative for metastases, except for a small lymph node from the right and one from the left femoral groups, which showed invasion of the small capsular lymphatic channels (fig. 3, B). During the first year postoperatively the patient had slight bilateral ankle edema. There was no sign of clinical metastases until a year later when an indurated area of the ventral mid penis was detected. A nodular hard mass occupied the subcutaneous areas of the suprapubic and right iliac
PENILE
lYiEN
697
FIG. L A, specimen removed at excisional biopsy shows diffuse infiltrating tumor cells wit:1 dark pigmentation. B, higher magnification of site of biopsy illustrates groups of large polygonal cells with vacuolated cytoplasm and prominent nuclei.
698
GIRGIS AND ASSOCIATES
FIG. 2. Partial penectomy specimen with site of original biopsy noted in longitudinally opened urethra.
regions. The patient consented to complete penectomy with perinea! urethrotomy. The lower abdominal subcutaneous masses were explored through a transverse lower abdominal incision. Large globular metastases with brownish deposits were found. Although the majority of the su bcutaneous metastases were removed, it was thought that technically these metastases were inoperable. The gross specimen showed the proximal penis and suprapubic soft tissues. The distal end showed the previous hemipenectomy site and the newly constructed urethral meatus. At the right side of the urethral meatus there was a well-circumcised 1 cm. nodule of grayish-tan color. Three smaller subcutaneous nodules were present along the proximal resected edge of the skin. The urethra and corpora cavernosa were grossly unremarkable. The specimen included an irregular fibrofatty structure wb ich contained several large grayish-tan tumor nodules (fig. 4). Microscopic examination of sections of the penis and suprapubic fat revealed large well delineated tumor nodules consisting of polygonal cells, which showed marked cellular pleomorphism, frequent mitoses and giant tumor cells (fig. 5, A). Occasional cells contained finely granular brown pigment. The urethra was not involved but a section through its proximal edge revealed tumor invasion of a vascular space of the corpora cavernosa (fig. 5, B). Diagnosis was metastatic malignant melanoma with vascular invasion of the corpora cavernosa and suprapubic tissues. . After the patient was discharged from the hospital he was treated in Germany with unknown chemotherapeutic agents without any added ef-
feet. No autopsy was available after the patient died approximately 2 years following the first examination. Case 2. L. L., BLHC 829878, a 72-year-old Jewish man, was hospitalized with a tumor of the penis approximately 2 months in duration. The tumor was rapidly increasing in size and was completely asymptomatic. The patient denied any history of trauma or venereal disease. On examination a cauliflower-like tumor was seen arising from the ventral surface of the glans penis. The lesion was approximately 1 cm. in largest diameter. The bearing stalk was approximately 0.5 cm. The surface of the tumor appeared infected and ulcerated. It was not thought that the deeper layers of the glans penis or the urethra were involved. There were no other similar lesions present on general examination. With general anesthesia the tumor was removed thoroughly with a good margin of safety. Convalescence was uneventful. We performed suprapubic prostatectomy on this patient 5 years later for prostatic hyperplasia and there have been no recurrences. The specimen was received in 2 pieces. The larger piece consisted of a mushroom-shaped nodule surrounded by apparently normal tissue. The nodule measured approximately 1 cm. in greatest diameter and raised to approximately 0.3 cm. The surface of the lesion showed superficial ulceration, Its cut surface appeared beefy red and vascular. The second segment consisted of a thin strip of grayish-pink tissue measuring 0.6 by 0.4 cm. in its major dimensions. Microscopically, the dermis was infiltrated by densely packed spindle cells (fig. 6). In most fields these cells grew in a diffusely infiltrative fashion and displayed no particular arrangement. However, in other areas the cells formed circumscribed nodules which exhibited a whorled pattern. In some fields the spindle cells were distributed more loosely and the vascular component was more conspicuous. The intervening tissue harbored dense collections of lymphocytes, histiocytes and plasma cells, some containing hemosiderin. In addition, there was a fair number of unclassified mononuclear cells with hyperchromatic nuclei. Mitotic figures were unevenly distributed but readily demonstrated. A distinctive feature of the lesion was the presence of innumerable vascular channels of variable sizes imparting an angiomatous appearance. They were lined by flattened, or more often, by prominent endothelial cells. Extravasated red cells were scattered throughout but only a few minute granules of hemosiderin were demonstrated with the Prussian blue reaction. Diagnosis was Kaposi's sarcoma of the penis.
DISCUSSION
In 1958 Abeshouse reported 5 primary malignant melanomas in the male urethra and 19 in the
PENILE
FIG.
3.
ill ustrntes
699
of section of glans penis shows large Pagefs cells. B, section of metastatic lymph nodes lymphatics.
700
GIRGIS AND ASSOCIATES
operation seem to have a better prognosis. The prognosis is poor but may be improved by earlier diagnosis and adequate radical operations. The cause of Kaposi's sarcoma is unknown but a herpes virus has been implicated. 5 Kaposi's sarcoma accounts for approximately 10 per cent of malignancies in natives of the Congo. It follows a geographical distribution similar to Burkitt's lymphoma, a disease of the reticuloendothelial system. In 1967 Dayan and Lewis found metallophilic cells in Kaposi's sarcoma.• Since the disease seems to be linked to a generalized disorder, therapy of the cutaneous lesions of Kaposi's sarcoma, regardless of location, should be conservative. The lesions have proved to be radiosensitive .7 CONCLUSION
FIG. 4. Gross specimen of total penectomy (part of penis not removed at partial penectomy). External meatus flagged. Some of suprapubic subcutaneous metastases are shown.
female urethra. Of the 5 tumors in the male urethra, 2 were in the meatal portion, 2 in the peno-scrotal portion and 1 in the prostatic urethra. In 1970 Guinn reported another primary tumor in the meatal portion. Primary malignant melanoma of the male urethra is extremely rare. Spread of the tumor is usually via the lymphatics and often occurs early in the course of the disease. Hematogenous spread to distant organs tends to occur later. Patients who do not have metastases at the time of
Two cases of unusual penile malignancies m circumcised Jewish men are presented. One of these was an anaplastic malignant melanoma of the urethra in a patient who died within 2 years of diagnosis. Documented local and lymph node metastases were aggressive. The second malignancy, a solitary penile Kaposi's sarcoma, was locally excised. Five years later there was no sign of recurrence or metastasis. Drs. Jacob Cohen and Alan Goldberg referred these patients to us and helped to treat them. 5 Henle, G., Henle, W. and Diehl, V.: Relation of Burkitt's tumor-associated herpes-type virus to infectious mononucleosis. Proc. Nat. Acad. Sci., 59: 94, 1968. 'Dayan, A. D. and Lewis, P. D.: Origin of Kaposi's sarcoma from reticulo-endothelial system. Nature, 213: 889, 1967. 7 McCarthy, W. D. and Pack, G. T.: Malignant blood vessel tumors. Report of 56 cases of angiosarcoma and Kaposi's sarcoma. Surg., Gynec. & Obst., 91: 465, 1950.
PENILE IvfALIGr~~ANCIES I~J CIRCU1\1CISED !viEr,-J
F!G. 5. cavernosa
701
photomicrograph of removed subcutaneous suprapubic nodular metastases. B, section through corpora involvement of vascular space with metastatic melanoma.
702
GIRGIS AND ASSOCIATES
FIG. 6. A, photomicrograph of Kaposi's sarcoma shows densely packed dermis with spindle cells and innumerable vascular channels. B, higher magnification of sarcoma illustrates spindle cells and vascular channels.
THE JovRNAL OF UROLOGY
Copyright© 1973 by The Williams & Wilkins Co.
Printed in U.S.A.
UNUSUAL PENILE MALIGNANCIES IN CIRCUMCISED JEWISH MEN AMIR S. GIRGIS, HARRY BERGMAN, HEDVIG ROSENTHAL AND LYDIA SOLOMON From the Bronx Lebanon Hospital Center, Bronx and the Jewish Memorial Hospital, New York, New York
Malignant tumors of the penis or external urethra are rare in circumcised Jewish men. We have treated 2 such primary malignancies: a primary malignant melanoma of the male urethra and a solitary Kaposi's sarcoma of the penis. We also reviewed 6 reported cases of malignant melanoma of the male urethra'· 2 and 11 cases of Kaposi's sarcoma of the penis. 3 • 4 CASE REPORTS
Case 1. G. W., JMH 239198, a 51-year-old white Jewish man, was hospitalized because of blood staining the underwear 5 weeks in duration. The patient noticed a growth at the external urethral meatus. He had a history of slight discomfort on urination. On examination a bluish tumor was noted through the urethral opening. When the external meatus was everted an irregular indurated growth approximately 1 cm. in diameter was seen at the terminal urethra. The pedicle was not noted and the proximal extension of the tumor could not be detected. The tumor bled easily with gentle palpation. There was no external induration on palpation of the distal penis. No palpable lymph nodes were detected in either groin or femoral triangles. An excretory urogram showed normally bilateral functioning kidneys with incomplete rotation of the left kidney and right pelvic renal ectopia. Radiologically, there was no ureteral deviation or obstruction. Urinalysis did not show any red blood cells. Liver scan showed normal distribution of 198 Au. Preoperative cystoscopy was unsuccessful because of the obstructing mass in the terminal urethra. An excisional biopsy of the urethral tumor through a posterior penile skin incision was performed with general anesthesia, obtaining a good margin of safety. The underlying Buck's fascia was excised to determine the degree 'of infiltration. The biopsy specimen consisted of 2 ovoid seg-
Accepted for publication June 29, 1973. Read at annual meeting of American Urological Association, New York, New York, May 13-17, 1973. 1 Abeshouse, B. S.: Primary and secondary melanoma of the genitourinary tract. Southern Med. J., 51: 994, 1958. Quoted by Weston, P. A. M. and Smith, R. J.: Metastatic melanoma in the bladder and urethra. Brit. J. Surg., 51: 78, 1964. 2 Guinn, G. A. and Ayala, A. G.: Male urethral cancer: report of 15 cases including a primary melanoma. J. Ural., 103: 176, 1970. 3 Cox, J. W., Halprin, K. and Ackerman, A. B.: Kaposi's sarcoma localized to the penis. Arch. Derm., 102: 461, 1970. 4 Summers, J. L., Wilkerson, J.E. and Wegryn, J. F.: Conservative therapy for Kaposi's sarcoma of the external genitalia. J. Ural., 108: 287, 1972.
696
ments of rubbery tissue, measuring 1.5 by 1.0 and 1.2 by 8.0 cm. and showing white cut surfaces with scattered hemorrhagic areas. Microscopic examination revealed a highly cellular infiltrating obviously malignant tumor, consisting of compact groups and sheets of large polygonal cells with vacuolated cytoplasm, conspicuous vesicular nuclei and prominent nucleoli (fig. 1). There was moderate pleomorphism and mitosis was rare. Some coarsely granular brown pigment was in the cells and was most prominent in the hemorrhagic ulcerated areas. The iron stain was negative, thus the possibility of hemoglobin derived pigment was eliminated. The surface squamous epithelium was unremarkable except for an occasional hyperchromatic cell in the basal layer. The tumor extended beneath the well preserved urethral mucosa. In one area occasional pigmented hyperchromatic cells were seen between the cells of the pseudostratified cuboidal urethral epithelium. Biopsy of Buck's fascia was unremarkable. Diagnosis was malignant melanoma of the urethra. After diagnosis was established repeat cystoscopy showed no suspicious tumors in the remaining urethra and bladder. Since the patient refused radical penectomy, partial penectomy with radical lymph node dissection of the groins, external iliacs, superficial and deep femoral and inguinal lymph nodes was done. Forty-four lymph nodes recovered on serial sectioning of the fatty tissue were grossly unremarkable. The amputated distal segment of the penis measured 6 cm. in length and showed the healing distal urethra (fig. 2). The glans penis and corpora cavernosa were grossly unremarkable. Microscopically, no residual tumor was found in the healing previously resected urethra but, in the vicinity of the biopsy site on the glans penis, there were scattered areas of active junctional changes at the margins of the rete ridges (fig. 3, A). One of these areas showed large Paget's cells extending to the stratum granulosum and was accompanied by a rather dense mononuclear leukocytic infiltration of the adjacent dermis. More than 300 sections examined from the 44 lymph nodes were negative for metastases, except for a small lymph node from the right and one from the left femoral groups, which showed invasion of the small capsular lymphatic channels (fig. 3, B). During the first year postoperatively the patient had slight bilateral ankle edema. There was no sign of clinical metastases until a year later when an indurated area of the ventral mid penis was detected. A nodular hard mass occupied the subcutaneous areas of the suprapubic and right iliac
PENILE
lYiEN
697
FIG. L A, specimen removed at excisional biopsy shows diffuse infiltrating tumor cells wit:1 dark pigmentation. B, higher magnification of site of biopsy illustrates groups of large polygonal cells with vacuolated cytoplasm and prominent nuclei.
698
GIRGIS AND ASSOCIATES
FIG. 2. Partial penectomy specimen with site of original biopsy noted in longitudinally opened urethra.
regions. The patient consented to complete penectomy with perinea! urethrotomy. The lower abdominal subcutaneous masses were explored through a transverse lower abdominal incision. Large globular metastases with brownish deposits were found. Although the majority of the su bcutaneous metastases were removed, it was thought that technically these metastases were inoperable. The gross specimen showed the proximal penis and suprapubic soft tissues. The distal end showed the previous hemipenectomy site and the newly constructed urethral meatus. At the right side of the urethral meatus there was a well-circumcised 1 cm. nodule of grayish-tan color. Three smaller subcutaneous nodules were present along the proximal resected edge of the skin. The urethra and corpora cavernosa were grossly unremarkable. The specimen included an irregular fibrofatty structure wb ich contained several large grayish-tan tumor nodules (fig. 4). Microscopic examination of sections of the penis and suprapubic fat revealed large well delineated tumor nodules consisting of polygonal cells, which showed marked cellular pleomorphism, frequent mitoses and giant tumor cells (fig. 5, A). Occasional cells contained finely granular brown pigment. The urethra was not involved but a section through its proximal edge revealed tumor invasion of a vascular space of the corpora cavernosa (fig. 5, B). Diagnosis was metastatic malignant melanoma with vascular invasion of the corpora cavernosa and suprapubic tissues. . After the patient was discharged from the hospital he was treated in Germany with unknown chemotherapeutic agents without any added ef-
feet. No autopsy was available after the patient died approximately 2 years following the first examination. Case 2. L. L., BLHC 829878, a 72-year-old Jewish man, was hospitalized with a tumor of the penis approximately 2 months in duration. The tumor was rapidly increasing in size and was completely asymptomatic. The patient denied any history of trauma or venereal disease. On examination a cauliflower-like tumor was seen arising from the ventral surface of the glans penis. The lesion was approximately 1 cm. in largest diameter. The bearing stalk was approximately 0.5 cm. The surface of the tumor appeared infected and ulcerated. It was not thought that the deeper layers of the glans penis or the urethra were involved. There were no other similar lesions present on general examination. With general anesthesia the tumor was removed thoroughly with a good margin of safety. Convalescence was uneventful. We performed suprapubic prostatectomy on this patient 5 years later for prostatic hyperplasia and there have been no recurrences. The specimen was received in 2 pieces. The larger piece consisted of a mushroom-shaped nodule surrounded by apparently normal tissue. The nodule measured approximately 1 cm. in greatest diameter and raised to approximately 0.3 cm. The surface of the lesion showed superficial ulceration, Its cut surface appeared beefy red and vascular. The second segment consisted of a thin strip of grayish-pink tissue measuring 0.6 by 0.4 cm. in its major dimensions. Microscopically, the dermis was infiltrated by densely packed spindle cells (fig. 6). In most fields these cells grew in a diffusely infiltrative fashion and displayed no particular arrangement. However, in other areas the cells formed circumscribed nodules which exhibited a whorled pattern. In some fields the spindle cells were distributed more loosely and the vascular component was more conspicuous. The intervening tissue harbored dense collections of lymphocytes, histiocytes and plasma cells, some containing hemosiderin. In addition, there was a fair number of unclassified mononuclear cells with hyperchromatic nuclei. Mitotic figures were unevenly distributed but readily demonstrated. A distinctive feature of the lesion was the presence of innumerable vascular channels of variable sizes imparting an angiomatous appearance. They were lined by flattened, or more often, by prominent endothelial cells. Extravasated red cells were scattered throughout but only a few minute granules of hemosiderin were demonstrated with the Prussian blue reaction. Diagnosis was Kaposi's sarcoma of the penis.
DISCUSSION
In 1958 Abeshouse reported 5 primary malignant melanomas in the male urethra and 19 in the
PENILE
FIG.
3.
ill ustrntes
699
of section of glans penis shows large Pagefs cells. B, section of metastatic lymph nodes lymphatics.
700
GIRGIS AND ASSOCIATES
operation seem to have a better prognosis. The prognosis is poor but may be improved by earlier diagnosis and adequate radical operations. The cause of Kaposi's sarcoma is unknown but a herpes virus has been implicated. 5 Kaposi's sarcoma accounts for approximately 10 per cent of malignancies in natives of the Congo. It follows a geographical distribution similar to Burkitt's lymphoma, a disease of the reticuloendothelial system. In 1967 Dayan and Lewis found metallophilic cells in Kaposi's sarcoma.• Since the disease seems to be linked to a generalized disorder, therapy of the cutaneous lesions of Kaposi's sarcoma, regardless of location, should be conservative. The lesions have proved to be radiosensitive .7 CONCLUSION
FIG. 4. Gross specimen of total penectomy (part of penis not removed at partial penectomy). External meatus flagged. Some of suprapubic subcutaneous metastases are shown.
female urethra. Of the 5 tumors in the male urethra, 2 were in the meatal portion, 2 in the peno-scrotal portion and 1 in the prostatic urethra. In 1970 Guinn reported another primary tumor in the meatal portion. Primary malignant melanoma of the male urethra is extremely rare. Spread of the tumor is usually via the lymphatics and often occurs early in the course of the disease. Hematogenous spread to distant organs tends to occur later. Patients who do not have metastases at the time of
Two cases of unusual penile malignancies m circumcised Jewish men are presented. One of these was an anaplastic malignant melanoma of the urethra in a patient who died within 2 years of diagnosis. Documented local and lymph node metastases were aggressive. The second malignancy, a solitary penile Kaposi's sarcoma, was locally excised. Five years later there was no sign of recurrence or metastasis. Drs. Jacob Cohen and Alan Goldberg referred these patients to us and helped to treat them. 5 Henle, G., Henle, W. and Diehl, V.: Relation of Burkitt's tumor-associated herpes-type virus to infectious mononucleosis. Proc. Nat. Acad. Sci., 59: 94, 1968. 'Dayan, A. D. and Lewis, P. D.: Origin of Kaposi's sarcoma from reticulo-endothelial system. Nature, 213: 889, 1967. 7 McCarthy, W. D. and Pack, G. T.: Malignant blood vessel tumors. Report of 56 cases of angiosarcoma and Kaposi's sarcoma. Surg., Gynec. & Obst., 91: 465, 1950.
PENILE IvfALIGr~~ANCIES I~J CIRCU1\1CISED !viEr,-J
F!G. 5. cavernosa
701
photomicrograph of removed subcutaneous suprapubic nodular metastases. B, section through corpora involvement of vascular space with metastatic melanoma.
702
GIRGIS AND ASSOCIATES
FIG. 6. A, photomicrograph of Kaposi's sarcoma shows densely packed dermis with spindle cells and innumerable vascular channels. B, higher magnification of sarcoma illustrates spindle cells and vascular channels.