Correspondence

Primary malignant melanoma of female urethra: A rare neoplasm ABSTRACT Primary malignant melanoma of urethra is an extremely rare entity. It has very poor prognosis. A 62‑year‑old post‑menopausal female presented with complaints of voiding difficulty and a mass projecting from external urethral meatus. External genital examination revealed a growth arising from urethral meatus with blood‑stained discharge from its surface. Biopsy from lesion confirmed the diagnosis to be malignant melanoma. Metastatic work up for the malignancy was negative. We describe the surgical management of this pathology at our tertiary care center and discuss the various treatment options possible in this scenario. KEY WORDS: Female, malignant melanoma, urethra neoplasm

INTRODUCTION

CASE REPORT

On general examination, no skin discoloration or abnormality was found. On genital examination, a 2 × 2 × 1.5 cm globular growth was seen protruding from her urethral meatus [Figure 1]. Her per vaginal and speculum examination was normal. No inguinal or regional lymph node enlargement was noted. However, her urinary bladder was palpable just above the pubic symphysis. Biopsy from the growth and histopathological examination (HPE) showed atypical or malignant 758

Departments of Urology, Institute of Post‑Graduate Medical Education and Research, 1Pathology and Microbiology, Sagar Dutta Medical College, Kolkata, India For correspondence: Dr. Praveen K. Pandey, Department of Urology, Institute of Post‑Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, 244, AJC Bose road, Kolkata - 700 020, India. E‑mail: drpandeypraveen@ gmail.com

Malignant melanoma is a rare neoplasm comprising approximately 1.2% of all malignancies. Though it may affect any part of human body, genitourinary tract involvement is observed in less than 1% of cases.[1] Clearly, primary malignant melanoma of urethra is most rare entity and comprises only 0.2% of malignant melanomas. It has a grave prognosis with poor 5‑year survival rates.[2] We present a case of primary malignant melanoma of urethra in a female and its surgical management at our tertiary care center.

A 62‑year‑old post‑menopausal female presented to us with complaints of difficulty in micturition and a mass protruding from her urethral meatus since 2 to 3 months. Sometimes, she also noticed blood‑stained discharge from the surface of mass. But, there was no history of hematuria, urinary retention or any urethral instrumentation. No medical co‑morbidity was observed.

Praveen K. Pandey, Mukesh K. Vijay, Hemant Goel, Suruchi Shukla1

Figure  1: Urethral malignant melanoma presenting as a mass protruding from external urethral meatus. Blood‑stained discharge can be seen on the surface of the neoplasm.

cells with aberrant nucleus‑cytoplasmic ratio and prominent nucleoli. Further, deposition of melanin pigment confirmed the diagnosis to be malignant melanoma [Figure 2]. Immuno‑histochemical analysis of the malignant cells was positive for HMB‑45 and protein S‑100. Ultrasonography of abdomen showed significant post‑void residual urine (96 ml). Subsequently, cysto‑urethroscopic examination revealed the growth to be localized to distal 1 cm of the urethra. The proximal urethra and bladder were free from the malignancy. Her chest X‑ray findings were normal. The abdominal‑pelvic contrast‑enhanced computed tomography (CECT) scan revealed that

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Pandey, et al.: Melanoma of female urethra

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Figure 2: (a) A scanner view of malignant melanoma showing sheets of atypical cells with brown color melanin pigment deposition. (H and E, ×4) (b) A low power view showing sheets of atypical cells with high N/C ratio and vesicular nucleus with prominent eosinophilic nucleolus. Deposition of melanin pigment, lymphocytic infiltration, and band of fibro‑collagenous tissue could be seen. (H and E, × 4)

peri‑urethral tissue and bladder were not involved. No regional or distant lymphadenopathy was noted. A routine surgery was planned, and wide local surgical excision of the growth was performed. The growth was removed along with the normal‑appearing surrounding tissue. HPE of excised specimen showed that all the margins were free of the cancerous tissue. The patient is on monthly follow up with us, and no recurrences have been observed after 1 year of operative procedure. However, she complains of occasional stress urinary incontinence, which does not cause significant bother due to her sedentary lifestyle. DISCUSSION Malignant melanoma of urethra was first reported in a female by Reed in 1896.[3] Females are more commonly affected as compared to males (3:2).[4] Though it is reported in different age groups, it is more common in 6th to 7th decade of life.[5] The patient may present with a urethral mass, dysuria, urethral discharge, or hematuria. The differential diagnosis may include vulvar or vaginal melanoma, urethral polyps, and caruncle.[6] It commonly involves distal urethra or urethral meatus.[5] However, proximal urethra involvement has also been described.[4] The diagnosis is confirmed after biopsy from the lesion. The metastatic evaluation should be done before embarking on the surgical treatment for this highly invasive neoplasm. An abdominal‑pelvic CECT scan provides necessary information regarding nodal involvement and metastatic foci. If required, whole body scan may yield necessary information regarding metastasis. The TNM classification by the American Joint Committee on Cancer (AJCC) is an important tool for staging this neoplasm.[7] The definite management of primary neoplasm is surgical excision with tumor‑free margins to achieve control of local disease. It has yielded 3‑year overall cancer‑specific survival as high as 27%.[5] Oliva and co‑workers have proposed various

surgical treatments including local excision, urethrectomy, vulvectomy with groin lymphadenectomy, urethrectomy with vaginectomy and even pelvic exanteration.[4] DiMarco and colleagues have also advocated radical surgeries. They believed that high rate of urethral recurrences (69%) in their cases of partial urethrectomy were due to inadequate surgical margins. Overall disease‑specific survival observed was 39% approximately.[5] In case of non‑metastatic deeply infiltrating tumor (>1 mm), radical urethrectomy with appendicovesicostomy or ileovesicostomy might be preferred over wide surgical excision.[2] We discussed this option with patient prior to surgery, but unfortunately she did not agree to it. The concept of sentinel lymph node (SN) dissection is still controversial in primary urethral melanoma. Though the incidence of false‑negative SN dissection has gone down, it is still not mandatory to perform this procedure in all cases.[8] Further, it is highly demanding procedure and requires the expertise of a trained surgical oncologist. We did not perform SN dissection in our case. The importance of inguinal lymph node dissection for therapeutic purpose is not yet established. This procedure, though adds to the morbidity of the patient, has failed to improve disease‑specific survival in affected individuals. So, role of radical surgery in case of inguinal lymph node involvement is not clear.[5] Adjuvant therapy may be required in almost all cases due to high risk of cancer‑specific mortality rates.[9] Different regimens described include interleukin 2 (IL‑2), interferon alpha or interferon beta with or without dacarbazine, vincristine, and cyclophosphamide chemotherapy.[2] Until 2011, Food and Drug Administration (FDA) of the United States had only approved dacarbazine and high dose IL‑2 (HD IL‑2) for metastatic melanoma therapy. But, none of these were able to increase median overall survival rates. Further, Dacarbazine and HD IL‑2 were limited by a low response rate (15% and 10%, respectively) and severe toxicity. However, recent FDA approval of vermurafenib and ipilimumab usage is expected to have a promising response rate.[10] There are no definite guidelines for management of this extremely rare pathology. The most rational treatment option appears to be radical surgery if possible along with adjuvant therapy. However, prospective randomized trials on treatment options and their outcomes are required to manage this dilemma. REFERENCES 1. Stein BS, Kendall R. Malignant melanoma of the genitourinary tract. J Urol. 1984;132:859‑68. 2. Kim CJ, Pak K, Hamaguchi A, Ishida A, Arai Y, Konishi T, et al. Primary malignant melanoma of the female urethra. Cancer 1993;71:448‑51. 3. Reed CA. Melanosarcoma of the female urethra: Urethrectomy recovery. Am J Obstet Gynecol. 1896;34:864‑72.

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4. Oliva E, Quinn TR, Amin MB, Eble JN, Epstein JI, Srigley JR, et al. Primary malignant melanoma of the urethra: A clinicopathologic analysis of 15 cases. Am J Surg Pathol 2000;24:785‑96. 5. DiMarco DS, DiMarco CS, Zincke H, Webb MJ, Keeney GL, Bass S, et al. Outcome of surgical treatment for primary malignant melanoma of the female urethra. J Urol 2004;171:765‑7. 6. Alvarez Kindelan J, Merchan Garcia JA, Olmo Cerezo I, Moreno Rodriguez MM, Gonzalez Arlanzon MM. Primary malignant melanoma of the female urethra. Report of a case. Actas Urol Esp 2000;24:488‑90. 7. Kim CJ, Reintgen DS, Balch CM; AJCC Melanoma Staging Committee. The new melanoma staging system. Cancer Control 2002;9:9‑15.

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8. Kelley MC, Ollila DW, Morton DL. Lymphatic mapping and sentinel lymphadenectomy for melanoma. Semin Surg Oncol 1998;14:283‑90. 9. Wagner JD, Gordon MS, Chuang TY, Coleman JJ 3rd. Current therapy of cutaneous melanoma. Plast Reconstr Surg 2000;105:1774‑99. 10. Finn L, Markovic SN, Joseph RW. Therapy for metastatic melanoma: The past, present, and future. BMC Med 2012;10:23. Cite this article as: Pandey PK, Vijay MK, Goel H, Shukla S. Primary malignant melanoma of female urethra: A rare neoplasm. J Can Res Ther 2014;10:758-60. Source of Support: Nil, Conflict of Interest: Nill.

Journal of Cancer Research and Therapeutics - July-September 2014 - Volume 10 - Issue 3

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Primary malignant melanoma of female urethra: a rare neoplasm.

Primary malignant melanoma of urethra is an extremely rare entity. It has very poor prognosis. A 62-year-old post-menopausal female presented with com...
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