Deep-seated sarcomas of the penis alberto a. Antunes, luciano j. Nesrallah, pierre d. Goncalves, yuri a. Ferreira, joao c. Campagnari, miguel srougi p

Yüklə 49.98 Kb.
PDF просмотр
ölçüsü49.98 Kb.



Clinical Urology

International Braz J Urol

Official Journal of the Brazilian Society of Urology

Vol. 31 (3): 245-250, May - June, 2005




Portuguese Beneficence Hospital of Sao Paulo and Syrian Lebanese Hospital, Sao Paulo, SP, Brazil


Mesenchymal neoplasias represent 5% of tumors affecting the penis. Due to the rarity of

such tumors, there is no agreement concerning the best method for staging and managing these patients.

Sarcomas of the penis can be classified as deep-seated if they derive from the structures

forming the spongy body and the cavernous bodies. Superficial lesions are usually low-grade and

show a small tendency towards distant metastasis. In contrast, deep-seated lesions usually show behavior

that is more aggressive and have poorer prognosis.

The authors report 3 cases of deep-seated primary sarcomas of the penis and review the

literature on this rare and aggressive neoplasia.

Key words: penis; sarcoma; neoplasm metastasis

Int Braz J Urol.  2005; 31: 245-50


Cancer of the penis is infrequent, with an

incidence ranging from 0.6 to 1/100,000 patients in

developed countries. Epidermoid carcinoma is the

dominant histological type; however, other tumors,

including basal cell carcinoma and melanoma, can

be found as well (1). Malignant and benign

mesenchymal tumors represent 5% of tumors

affecting this organ (2).

The sarcomas of the penis can be classified

as superficial or deep-seated according to the tissues

from which they derive (3). Superficial lesions rarely

reach deep tissues, are usually low-grade and show a

small tendency to distant metastases. In contrast, deep-

seated lesions, which include those originating from

the glans, those involving the smooth muscle of the

spongy and/or cavernous bodies, and those

representing advanced lesions that were initially

superficial, usually show more aggressive behavior

and have poorer prognosis (4).

In the present study, the authors report 3 cases

of primary deep-seated sarcomas of the penis and

review the literature on this rare and aggressive



Case 1

A 61-year old Caucasian man presented

penile pain and a tumor at the base of the penis for 60

days. Physical examination of the penis revealed a

constant state of semi-erection, which the patient

reported as having started 30 days earlier. On

palpation, a tumor mass with hardened consistency

was observed in the pre-pubic region measuring

approximately 5 cm in diameter and extending to the

right cavernous body and perineal region.

Laboratory tests, chest x-ray and abdominal

ultrasound (USG) were normal. Magnetic resonance

imaging (MRI) of the pelvis showed a solid infiltrative

mass at the base of the penis and signs suggestive of



thrombosis in the cavernous bodies (Figure-1). There

was no evidence of enlarged lymph nodes.

The patient underwent emasculation (Figure-

2) and definitive suprapubic cystostomy with closure

of the urethral stump at the level of the membranous

urethra due to the perineal extension of the tumor.

There were no postoperative complications and the

patient was discharged from the hospital on the 4th

postoperative day. Pathological examination revealed

leiomyosarcoma involving the cavernous bodies and

extending to the deep perineal planes (Figure-3).

Surgical margins were free from neoplasia.

One month following surgery, patient

presented respiratory stress and the radiological

examination revealed the presence of bilateral

pulmonary nodules and metastases characteristics.

Chemotherapy was then started with adriamycin,

ifosfamide and dimethy-l-triazeno-imidazol-

carboxamide (DTIC). Upon completion of

chemotherapy, the patient presented a reduction in

pulmonary lesions but he died in the eighth

postoperative month.

Figure 1 – Magnetic resonance imaging. The coronal T1-

weighted image of the base of the penis shows an infiltrative

tumor leading to destruction of cavernous bodies (arrows).

Figure 2 – Emasculation. Resection of the entire penis and


Figure 3 – Leiomyosarcoma of the penis (HE, X100).



Case 2

A 56-year old man complained of a painful

perineal tumor for 2 months. Physical examination

revealed a fixed perineal mass measuring

approximately 6.0 cm in diameter.

Pelvic computerized tomography revealed a

solid nodule measuring 6.5 cm with involvement of

cavernous bodies. Laboratory tests were normal, as

were radiological examinations of the chest and


Emasculation was performed with closure of

the urethral stump followed by definitive cystostomy.

The patient evolved with no intercurrences and was

discharged from the hospital on the 5th postoperative


The pathological examination revealed

leiomyosarcoma of cavernous bodies with

involvement of surgical margins. The patient was then

referred to adjuvant radiotherapy, however he

complained of respiratory stress and control exams

evidenced pulmonary nodules suggestive of

metastases at the end of the treatment. Systemic

chemotherapy was indicated, but the patient died in

the 9th postoperative month.

Case 3

A 72-year old man presented perineal

discomfort with increasing severity for approximately

6 months, especially during sexual intercourse. He

reported the appearance of a tumor in the penis 30

days earlier. Physical examination revealed a

hardened mass measuring approximately 8.0 x 8.0 cm

located at the base of the penis, infiltrating to the

cavernous bodies and extending to the perineum.

Laboratory tests were normal and pelvic

ultrasound showed no abnormalities. Abdominal and

pelvic computerized tomography showed a solid

lesion in cavernous bodies measuring 5.9 cm in

diameter. MRI allowed a more accurate assessment

of the local extension to the perineum (Figure-4).

There was no evidence of enlarged lymph nodes in

the chest x-ray.

The patient underwent emasculation where

an extensive involvement was observed in the

perineum and right ischiorectal fossa. Again,

suprapubic definitive cystostomy was performed.

There were no postoperative complications and the

patient was discharged from hospital on the 5th

postoperative day.

The pathological study together with an

immunohistochemical analysis revealed a

fibrohistiocytic sarcomatous tumor (Figure-5) with

imprecise limits located 0.8 cm from the surgical

margins. The patient received adjuvant radiotherapy;

however, pulmonary metastasis was diagnosed in the

3rd postoperative month. The patient received

Figure 4 – Magnetic resonance imaging. Coronal T1-weighted

image shows tumor in soft tissues affecting the root of the right

cavernous body and extending to the pubis.

Figure 5 – Malignant fibrohistiocytoma of the penis.

Immunohistochemical analysis for CD-68 (X400).



chemotherapy, which was unsuccessful, and died on

the 6th postoperative day.


It is estimated that less than 5% of soft tissue

sarcomas originate from the urogenital tract, and they

represent only 1 to 2% of neoplasias in this system

(5). In the present study, we present 3 cases of deep-

seated sarcomas of the penis in patients aged between

56 and 72 years old that were diagnosed based on

clinical presentation and pelvic imaging studies. The

cases reflect the lesion’s aggressive characteristics

leading to death after a mean period of 7.6 months

despite aggressive surgical treatment and adjuvant


Despite being rare, some characteristics

relative to the pattern of recurrence and metastatic

dissemination for sarcomas of the penis have been

well established. Local recurrence seems to be a

frequent phenomenon, and total amputation of the

penis should be considered even for superficial lesions

(6). On the other hand, regional metastases appear to

occur less frequently, and if there is no apparent lymph

nodal disease, lymphatic dissection is not

recommended (7). The most frequent sites of distant

metastases are the lungs, liver and brain (8).

The best treatment method for sarcomas is

complete resection of the tumor. One study showed

that no patient with retroperitoneal sarcoma had

survived beyond 5 years when the sarcomas had not

been not completely resected, while the 5-year

survival rate for patients who underwent complete

resection was 48% (9). When investigating 43

patients with urogenital sarcoma, Russo et al. (10)

reported that complete tumor resection was possible

in 72% of the cases, and 58% had disease-free

margins. Survival rates after 3 and 5 years were 55%

and 40% respectively. Another factor that contributes

to the poor prognosis in these patients is the fact

that no effective response to adjuvant therapy has

been obtained up to this moment. Radiotherapy has

been used for final control in local disease for

unresectable tumors and for patients with positive

margins, with chemotherapy being reserved for cases

of disseminated disease. In the series by Russo et

al. (10), no patient with disseminated disease was

fully responsive to the use of several chemotherapy


Some previously described prognostic factors

can help to predict the biological behavior of sarco-

mas of the penis (10). These factors are lesion size

(larger or smaller than 5 cm), extension of invasion

(superficial vs. deep-seated), complete resection of

the lesion, presence or absence of metastatic disease

and expression of the retinoblastoma gene (11).

Two of the 3 cases described in this study

were diagnosed as leiomyosarcoma. In 1994, 14

cases of deep-seated leiomyosarcomas of the penis

were reviewed (12). The lesions were primarily

treated with local excision (3), amputation (8),

external radiotherapy (2) and chemotherapy (1), and

patients were followed during a period from 1 to 72

months (mean 16 months). Eight patients died during

follow-up. Among the patients treated by radical

surgery (amputation), 3 developed disseminated

disease and 4 died from the disease. All patients

treated with chemotherapy or radiotherapy had local

recurrence, with 2 of them presenting disseminated


More recently, Fetsch et al. (13) reported 14

cases of leiomyosarcoma of the penis from a single

center. Ages ranged from 43 to 62 years old (mean

51). The penile shaft was the most frequently

affected site. The size of the lesions ranged between

0.5 and 6 cm. Nine tumors were superficial, 2 had

undetermined location, and 3 were deep-seated.

Immunohistochemical analysis was available for 9

patients (64%), and immunoreactivity for desmin

was present in all such cases. Mean follow-up was

12 years and 11 months, and, of 9 patients (64%)

with available information, 3 had multiple local

recurrences, 2 of which were subsequently treated

by wide local excision and partial penectomy. The

third patient refused treatment and developed distant

metastasis 10 months after the fourth recurrence. The

main prognostic factors were the lesion’s depth and


The third case was diagnosed as a

fibrohistiocytic sarcoma. Despite being the sarcoma

that is more frequently described in the elderly, this

histological type rarely involves the urogenital tract



(14). Only 2 cases of malignant fibrohistiocytoma

have been described up to the present time (15,16).

These tumors usually display an aggressive behavior

tending to lymphatic dissemination (15,17). Regional

lymphadenectomy apparently does not benefit those

patients with sarcomas of the penis without evidence

of lymphatic dissemination, however it is highly

recommended for patients with this type of tumor. In

the presence of metastases, chemotherapy seems to

present some benefits (18).

Other types of penile sarcoma have been

described in the literature. According to records from

the Armed Forces Institute of Pathology, Kaposi’s

sarcoma is the most common histological type,

surpassing the cases of leiomyosarcoma by a 2:1 ratio.

In contrast to the latter, the Kaposi’s sarcomas are

strongly reactive to CD34 and CD31 and do not show

desmin expression. Finally, sarcomatoid carcinomas

must always be remembered when a penile sarcoma

is diagnosed. The immunohistochemical analysis for

keratin pattern and extension can distinguish between

both tumors (13).

Though radical surgery with negative margins

is the treatment that provides the best results, our

patients quickly evolved to disseminated disease, with

the lungs being the most frequent site for metastasis.

When a perineal urethrostomy is impossible, and if

the patient’s clinical conditions allow, performing a

continent urinary shunt might make social living more

acceptable for these patients, since they would not

have to use a cystostomy bag (19).

The authors conclude that sarcomas of penis

are rare tumors and usually have poor prognosis when

they involve deep-seated lesions. The analysis of

prognostic factors can help to identify those patients

at higher risk for disease progression. The complete

tumor resection with negative surgical margins

whenever possible seems to offer the best chances of

healing. No effective treatment for disseminated

disease has been developed up to the present time.



Dehner LP, Smith BH: Soft tissue tumors of the penis.

A clinicopathologic study of 46 cases. Cancer. 1970;

25: 1431-47.


Lucia MS, Miller GJ: Histopathology of malignant

lesions of the penis. Urol Clin North Am. 1992; 19:



Pratt RM, Ross RT: Leiomyosarcoma of the penis. A

report of a case. Br J Surg. 1969; 56: 870-2.


Trojani M, Contesso G, Coindre JM, Rouesse J, Bui

NB, de Mascarel A, et al.: Soft-tissue sarcomas of

adults; study of pathological prognostic variables and

definition of a histopathological grading system. Int

J Cancer. 1984; 33: 37-42.


Herr HR: Sarcomas of the Urinary Tract. In: de

Kernion JB, Paulson DF (eds.), Genitourinary Cancer

Management. Philadelphia, Lea & Febiger. 1987; pp.



Webber RJ, Alsaffar N, Bissett D, Langlois NE:

Angiosarcoma of the penis. Urology. 1998; 51: 130-



Lynch Jr. DF, Pettaway CA: Tumors of the Penis. In:

Walsh PC, Retik AB, Vaughan Jr. ED, Wein AJ (eds.),

Campbell’s Urology, 8th ed. Philadelphia, WB

Saunders. 2002; pp. 2945-81.


Antoneli CB, Novaes PE, Alves AC, Cardoso H,

Lopes A: Rhabdomyosarcoma of the penis in a 15-

month-old boy. J Urol. 1998; 160: 2200-1.


Zhang G, Chen KK, Manivel C, Fraley EE: Sarcomas

of the retroperitoneum and genitourinary tract. J Urol.

1989; 141: 1107-10.

10. Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR,

Herr HW et al.: Adult urological sarcoma. J Urol.

1992; 147: 1032-6; discussion 1036-7.

11. Cance WG, Brennan MF, Dudas ME, Huang CM,

Cordon-Cardo C: Altered expression of the

retinoblastoma gene product in human sarcomas. N

Engl J Med. 1990; 323: 1457-62.

12. Pow-Sang MR, Orihuela E: Leiomyosarcoma of the

penis. J Urol. 1994; 151: 1643-5.

13. Fetsch JF, Davis Jr CJ, Miettinen M, Sesterhenn IA:

Leiomyosarcoma of the penis: a clinicopathologic

study of 14 cases with review of the literature and

discussion of the differential diagnosis. Am J Surg

Pathol. 2004; 28: 115-25.

14. Enzinger FM, Weiss SW: Soft Tissue Tumors. St

Louis, Mosby. 1983; pp. 116-98.

15. Parsons MA, Fox M: Malignant fibrous histiocytoma

of the penis. Eur Urol. 1988; 14: 75-6.

16. Fletcher CD, Lowe D: Inflammatory fibrous

histiocytoma of the penis. Histopathology. 1984; 8:


17. Sclama AO, Berger BW, Cherry JM, Young JD Jr:

Malignant fibrous histiocytoma of the spermatic cord:



the role of retroperitoneal lymphadenectomy in

management. J Urol. 1983; 130: 577-9.

18. Williamson JC, Johnson JD, Lamm DL, Tio F:

Malignant fibrous histiocytoma of the spermatic cord.

J Urol. 1980; 123: 785-8.

19. Lemelle JL, Simo AK, Schmitt M: Comparative study

of the Yang-Monti channel and appendix for continent

diversion in the Mitrofanoff and Malone principles. J

Urol. 2004; 172: 1907-10.

Received: January 17, 2005

Accepted after revision: May 3, 2005

Correspondence address:

Dr. Alberto Azoubel Antunes

Rua Três de Maio, 17/31

São Paulo, SP, 04044-020, Brazil

Phone: + 55 11 5573-5385


Verilənlər bazası müəlliflik hüququ ilə müdafiə olunur © 2016
rəhbərliyinə müraciət

    Ana səhifə