Primary Leiomyosarcoma of the Penis: A Review and Update of the Literature

Less than 50 cases of Primary Leiomyosarcomas of the Penis (PLOP) have been reported despite this PLOPs are the second most common sarcomas of the penis. The usual site for the development of PLOP is the shaft or base of the penis but the malignancy can affect any site of the penis. PLOPs are likely to be superficially located above the tunica albuginea in comparison with deep seated PLOPs. Superficial PLOPs may be asymptomatic or they may present as lumps, ulcers or nodules on the penis. Deep PLOPs may manifest with dysuria and difficulty with micturition when they compress the urethra. Diagnosis of PLOP is based upon the pathology examination features of specimens of the penile lesion that show interlacing fascicles of spindled-cells that have abundant eosinophilic cytoplasm, focal juxta-nuclear vacuoles and blunt ended nuclei and moderate to severe nuclear atypia, rarely atypical multi-nucleated giant cells and focal necrosis tend to be seen. There may be evidence of many mitoses. Immunohistochemistry studies of PLOP tend to show tumor cells that exhibit positive staining for desmin, muscle specific-actin, and alpha smooth muscle actin. Most often superficial PLOPs tend to be treated by wide complete excision of the tumor with tumor free and clear surgical resection margins. Deep seated PLOPs have also been treated by complete excision in various forms depending upon the site and size of the tumor and some of these have included Wide excision, partial penectomy and total penectomy alone and sometimes with adjuvant chemotherapy/radiotherapy. Some cases of deep PLOPs tend to be associated with the development of recurrences and metastases as well as death of the patient and hence deep PLOPs are aggressive tumors. Some people are of the opinion that chemotherapy and radiotherapy are not effective for the adjuvant treatment of PLOP. For this reason it would appear that there is need for the development of new chemotherapy medicaments that would effectively destroy PLOP tumor cells. There is need to undertake a global multi-center treatment trial of immunotherapy, chemotherapy and radiotherapy in order to streamline the treatment of PLOPs especially deep PLOPs.