Present lesion commonly originates in the sulcus of the corona glandis. The lesion is clearly visible and palpable after retracting the foreskin. The lesion can be papillary, solid or ulcerating. Depending on the stage at diagnosis, the lesion is superficial or infiltrating into all the tissue layers of the penis. Patients with non-retractile foreskin (phimosis) often present with a foul-smelling discharge as a first sign. The tumor can usually be palpated under the foreskin [3, 16, 17].

Enlarged inguinal lymph nodes are the main clinical feature. Lymph node metastases could be unilateral or bilateral. Signs of inflammation may dominate the clinical picture (hyperemia, pain, edema). Further spread is only manifested by secondary symptoms like lymphedema, because of proximal lymphatic obstruction, flank pain because of ureteric obstruction, bone pain, because of bone metastases or the signs of hypercalciemia [3, 16].

This is a controversial issue with two groups of proponents. One group advocates inguinal lymph node dissection in all but the smallest tumors and the other group advocates a wait and see policy in all patients except in those with unfavorable prognostic characteristics. There is uniform consensus that patients presenting with stage Tcis and well differentiated T1-tumors could be treated expectantly [23, 37, 38]. Based on biopsy protocols of melanoma and breast cancers the so called dynamic sentinel node biopsy was developed for all other categories [39]. A lymph node dissection is done only in sentinel node positive patients. Results show acceptable false negative rates and excellent survival figures compared to a cohort of patients managed conservatively [17, 40].

Treatment usually consists of a straightforward inguinal lymph node dissection. This should be preceded by FNA in order to prove metastatic involvement of the lymph node. Depending on the number of tumor positive nodes, extracapsular growth and the location of tumor positive nodes, a complementary iliac lymph node dissection is mandatory. Patients with fixed nodes and or presenting with retroperitoneal nodes should undergo pre-operative treatment with combination chemotherapy or radiation therapy. After response evaluation subsequent surgery should be scheduled in patients with clinical response. Because of the rarity of this disease this should be done preferably within the framework of a clinical study [27, 41, 42].

Penis preserving treatment is safe, but meticulous follow up or self-examination is of utmost importance as the local recurrence rate varies from 19 to 37 % irrespective of type of treatment. As a local recurrence can be the source of further spread this should be treated at the earliest possible moment. Local recurrences after partial or total amputation is rare. The most common complication is stenosis of the neo-urethra (5–10 %) [22, 43].