LEIOMYOSARCOMA OF PENIS : A RARE CASE WITH UNUSUAL PRESENTATION

Shankar A 1 , Nagpal P 1 , Mitra S 2 , Kakkar N 2 , Kumar N 1 andGhoshal S 1 . 1. Department of Radiotherapy, PGIMER Chandigarh. 2. Department of Histopathology, PGIMER Chandigarh. ...................................................................................................................... Manuscript Info Abstract ......................... ........................................................................ Manuscript History


ISSN: 2320-5407
Int. J. Adv. Res. 5 (5), 396-400 397 FDG avid lesion in retroperitoneum, pancreas, liver, bilateral lungs suggestive of disease progression. Gemcitabine based chemotherapy was continued for six cycles which completed on 6-12-16. Repeat PET CT done on 30-1-17 revealed FDG avid lesion in penis along with retroperitoneal, pancreas, bilateral lungs,and skeletal muscle metastasis suggestive of progressive metabolic disease. Patient last visited OPD on 7-2-17 where on examination he had multiple nodules in penis from root to tip and nodule in periumbilical region. Patient has been kept on best supportive care.

Discussion:-
The incidence of penile cancer is very low and varies from 0.5-5 /100 000 men. However, sarcomas are extremely rare and constitute less than 5% of all malignant tumors of the penis 3 . To date, less than 50 cases have been reported in the medical literature 4 .Leiomyosarcomas of the penis occur most often in the fifth to seventh decades of life as has been in our case who presented at the age of 51.Symptoms are generally indolent.
Penile leiomyosarcomas can be classified into two types, superficial and deep-seated tumours 5 .
Superficial tumors are usually present over the shaft distally or the prepuce. It most commonly occurs in middleaged men. It is a slow growing tumor.
Deep tumors arise from the glans penis and from the corpora cavernosa or corpus spongiosum 6 as has been in our case. In comparison to superficial tumors deep tumors show greater propensity to develop metastasis 7 . The most common site of spread is the lungs as has been seen in our case who presented with diffuse lung metastasis.
Treatment modality has not yet been established because of few cases reported in the medical literature. Treatment often ranges from conservative surgery to total penectomy, and adjuvant radiotherapy or chemotherapy. Radical penectomy is the treatment choice for deep tumors. Lymph node involvement is very rare, and so elective lymphadenectomy is not recommended 8 . If lymph node is involved then there is a high chance of developing distant metastasis, primarily to the lung as has been in this case who presented with inguinal lymphadenopathy and lung metastasis subsequently.
External beam radiation has not proven to be of value in treating penile leiomyosarcomas 9 . Chemotherapy has provided poor results and generally comprises of anthracyclines or etoposide based regimens. Since our patient presented with metastatic disease at onset he was given palliative chemotherapy with anthracycline based regimen but as given in the literature showed disease progression on chemotherapy.
One rare presentation in our case which in itself is a rare entity is that the patient is doing well even after 6 years of diagnosis and since 2 years after being diagnosed of having such high systemic disease burden and showing disease progression after first line chemotherapy. Patient is well preserved till date. This presentation is in sharp contrast with the available data in the literature. A review with follow-up duration of 12 years showed that local recurrence occurred in 26% of the patients between three to 18 months from the first surgery, and among those patients that had deep lesions, eight died between the first month and 36 months from surgery.

Conclusion:-
Sarcomas of penis are rare tumors and deep-seated lesions are usually associated with poor prognosis. The radical tumor resection with negative surgical margins offers the best chance of cure. No effective treatment for metastatic disease has been developed till date.