Extramammary Paget’s disease of the scrotum

Abstract

Extramammary Paget's disease is an uncommon intra-epidermal malignant neoplasm that arises in area rich in apocrine glands. Common sites of occurrence include the vulva, perianal region, perineum, and scrotum. The lesion may be accompanied by an invasive adenocarcinoma or adenocarcinoma in situ of the apocrine glands. Generally, the prognosis is poor. Herein, we report two cases of extramammary Paget's disease, one involving the penoscrotal area with bilateral inguinal and pelvic lymph node metastases, the other involving the scrotal area without metastases.

Enlarging Bump on the Scrotum

“…It often presents as a well-demarcated, thickened, erythematous, or gray-white scaly plaque with indolent growth that may become ulcerated, crusted, papillomatous, or eczematous over time [1, 2, 6]. Pruritus, pain, and burning sensations are common symptoms; however, none were appreciated by our patient [6].…”

“…Pruritus, pain, and burning sensations are common symptoms; however, none were appreciated by our patient [6]. Histopathologic analysis of EMPD showed round, large vacuolated Paget cells with rich cytoplasm, multiple or large nucleoli, irregular and large nuclei, and mitoschisis [7].…”

“…For this reason, adequate workup should include screening for metastatic malignancy after histologic diagnosis was established [6]. A computed tomographic scan of the abdomen and pelvis is recommended to detect any associated or metastatic abdominal or genitourinary cancers [3].…”

“…Screening with chest X-ray, colonoscopy, cystoscopy, and serum prostate-specific antigen levels is also performed by some physicians. Poor prognostic indicators include perianal disease, dermal invasion, and lymph node metastasis [3, 6]. …”

“…Differential diagnosis includes contact dermatitis, fungal infection, psoriasis, eczema, melanoma, squamous cell carcinoma, and intreaepidermal spread of visceral carcinoma [1, 6]. Many patients are erroneously treated for eczema for months to years before a proper diagnosis is reached.…”