|Ahead of print publication
Extramammary Paget's disease of the penis: A rare case report
Sumir Kumar1, Meenal Makkar1, Sukhmani Kaur Brar2
1 Department of Dermatology, Venereology and Leprology, Guru Gobind Singh Medical College and Hospital, Faridkot, Punjab, India
2 Department of Dermatology, Venereology and Leprology, Fortis Polyclinic, Chandigarh, India
|Date of Submission||03-Jun-2019|
|Date of Decision||27-Aug-2019|
|Date of Acceptance||29-Nov-2019|
|Date of Web Publication||05-Nov-2020|
Department of Dermatology, Venereology and Leprology, Guru Gobind Singh Medical College and Hospital, Faridkot, Punjab
Source of Support: None, Conflict of Interest: None
Extramammary Paget's disease (EMPD) of the penis is a rare, intraepidermal adenocarcinoma. We describe the case of a 60-year-old male who presented with an erythematous plaque on his penis, previously treated with various topical and oral drugs. Punch biopsy from the lesion revealed the diagnosis of EMPD. Early diagnosis and treatment is necessary for this rare neoplastic condition.
Keywords: Erythematous plaque, extramammary Paget's disease, intraepidermal adenocarcinoma
| > Introduction|| |
Extramammary Paget's disease (EMPD) is a rare, neoplastic condition which usually presents as an itchy plaque commonly found in the apocrine gland-bearing areas. EMPD of the scrotum and penis was first described by Crocker in 1889. Vulva is the most common site involved with 65% of EMPD, followed by perianal accounting for 20% of cases and then scrotum and penis accounting for 14% of cases. It is very uncommon in men with a female-to-male ratio about 4:1.
| > Case Report|| |
A 60-year-old male presented with a 6-month history of a pruritic, erythematous lesion over his penis. No history of bleeding or any discharge from lesion was noted. Before presentation, he had been treated with various topical agents, systemic antibiotics, and antivirals. He had no family history of any malignancy. There was no history of any other comorbidity. On examination, a single erythematous plaque measuring 1.3 cm × 1 cm with well-defined irregular border was present on the inner side of prepuce of penis [Figure 1]. Lymphadenopathy was absent. A clinical suspicion of EMPD and Bowen's disease was made and biopsy was sent.
Histopathological examination showed circumscribed collections of large cells within the lower epidermis that had abundant pale eosinophilic to clear cytoplasm and large nuclei that showed variation in size and shape with an increased number of normal and abnormal mitotic figures. At places, these cells were located within mid or even upper epidermis (pagetoid distribution). Intervening epidermis shows mild spongiosis. Dermis shows a moderately dense superficial and midperivascular and interstitial infiltrate of lymphocytes, plasma cells, and neutrophils [Figure 2], [Figure 3], [Figure 4].
|Figure 3: Low magnification view (x10) showing large cells with hyperchromatic nuclei (Paget's cells) in epidermis|
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|Figure 4: High magnification view (x40) showing large cells with hyperchromatic nuclei (Paget's cells) in epidermis|
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Relevant laboratory investigations were normal (chest X-ray, cystoscopy and colonoscopy, and ultrasonography of abdomen–pelvis). Hence, there was no evidence of visceral malignancy.
The patient was referred to an oncosurgeon for further management. He did wide local excision. The patient was in our follow-up till 6 months after surgery with no recurrence and lymphadenopathy.
| > Discussion|| |
Isolated Paget's disease of the penis is extremely rare, and only a few cases have been reported in literature. The disease is usually occurs in older age group between ages of 50 and 70 years. Clinically, the EMPD lesions and the symptoms are nonspecific. The disease is asymptomatic in 10% of the patients.
Various theories proposed for pathogenesis. Three patterns of EMPD are accepted: (1) in situ epithelial form without carcinoma and excellent prognosis, (2) epithelial form with adnexal carcinoma, and (3) epithelial form with visceral malignancy.
Various reports suggest that up to 42% of patients have associated underlying secondary or noncutaneous malignancy. Hence, a complete workup to detect any underlying malignancy is necessary. Recurrence rate is approximately 30%–60%.
Surgery (wide local excision) is the treatment of choice for local EMPD., Other options include Mohs micrographic surgery, topical and systemic chemotherapy, laser therapy, radiotherapy, and photodynamic therapy.,
To conclude, an uncommon condition like EMPD should also be kept in mind when an elderly male presents with pruritic erythematous penile lesion with other differentials, such as Zoon's balanitis, Bowen's disease, lichen planus, and psoriasis. Hence, we highlight the need for a high index of suspicion in the diagnosis of this rare neoplasm.
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Conflicts of interest
There are no conflicts of interest.
| > References|| |
Crocker HR. Paget's disease affecting the scrotum and penis. Trans Pathol Soc Lond 1889;40:187-91.
Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol 2007;2:581-90.
Lam C, Funaro D. Extramammary Paget's disease: Summary of current knowledge. Dermatol Clin 2010;28:807-26.
Helwig EB, Graham JH. Anogenital (extramammary) Paget's disease. A clinicopathological study. Cancer 1963;16:387-403.
Lai YL, Yang WG, Tsay PK, Swei H, Chuang SS, Wen CJ. Penoscrotal extramammary Paget's disease: A review of 33 cases in a 20-year experience. Plast Reconstr Surg 2003;112:1017-23.
Zhu Y, Ye DW, Yao XD, Zhang SL, Dai B, Zhang HL, et al
. Clinicopathological characteristics, management and outcome of metastatic penoscrotal extramammary Paget's disease. Br J Dermatol 2009;161:577-82.
Anolik R, Liang C, Wang N, Rosenman K, Pomeranz M, Joe E. Extramammary Paget disease. Dermatol Online J 2008;14:5.
Banerjee S, Chatterjee M, Chand K. Extramammary Paget's disease. Indian J Dermatol Venereol Leprol 2005;71:417-20.
] [Full text]
Nardelli AA, Stafinski T, Menon D. Effectiveness of photodynamic therapy for mammary and extra-mammary Paget's disease: A state of the science review. BMC Dermatol 2011;11:3.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]