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E-IJD® - CORRESPONDENCE |
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| Year : 2022 | Volume
: 67
| Issue : 1 | Page : 96 |
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| Defining surgical margins with dermoscopy and frozen section in recurrent extramammary pagets disease |
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TV Murali1, Amal V Antony2, Vineetha Mary3, Dhiya Philomina3
1 Department of Surgical Oncology, Government Medical College, Kottayam, Kerala, India
2 Department of General Surgery, MES Medical College, Perinthalmanna, Kerala, India
3 Department of Dermatology, Government Medical College, Kottayam, Kerala, India
| Date of Web Publication | 19-Apr-2022 |
Correspondence Address:
T V Murali
Department of Surgical Oncology, Government Medical College, Kottayam, Kerala
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_410_21
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How to cite this article:
Murali T V, Antony AV, Mary V, Philomina D. Defining surgical margins with dermoscopy and frozen section in recurrent extramammary pagets disease. Indian J Dermatol 2022;67:96 |
How to cite this URL:
Murali T V, Antony AV, Mary V, Philomina D. Defining surgical margins with dermoscopy and frozen section in recurrent extramammary pagets disease. Indian J Dermatol [serial online] 2022 [cited 2023 Jun 4];67:96. Available from: https://www.e-ijd.org/text.asp?2022/67/1/96/343301 |
Sir,
Scrotum is a common site for Extramammary pagets disease (EMPD), which is an adenocarcinoma seen in the apocrine gland bearing skin like scrotum, penis, vulva, and anus with or without an underlying visceral malignancy.[1] Surgical resection is the treatment of choice, but defining margin is difficult and hence recurrences are common. Dermoscopy is a non-invasive method for assessing certain features of skin that are normally not visible to naked eye. We describe a case of third recurrence of a margin positive scrotal pagets disease where dermoscopy was used to define surgical resection margins.
A 60-year-old gentleman on treatment for Parkinson's disease presented with history of two surgeries in the scrotum for extramammary pagets disease. First surgery was one year back, and the lesion recurred within 3 month. He underwent excision of the recurrent lesion as well, only to see that the lesion reappeared after 6 months. Reviewing the Histopathology results of the first two surgeries revealed extramammary pagets disease and immunohistochemistry was positive for EMA and CEA and negative for HMB 45. Both the resections were margin positive. Now on examination there was an erythematous macule with ill-defined borders on right side of scrotum with a stretched out scar of previous surgery. The lesion margins were not clear making it difficult to identify with naked eyes. Abdominopelvic Contrast-Enhanced Computed Tomography showed no inguinal lymph nodes and no underlying primary cancer. Because of the vague margin between the normal and diseased skin we were not sure about the extent of resection of scrotum required. Hence dermoscopy was done to define the exact extent of the disease.
Dermascopy showed white structure fewer areas, lava lake structures and linear irregular vessels [Figure 1]. With the help of this tool marking of the lesion was made which was impossible with naked eye [Figure 2]. Patient underwent wide excision of the lesion and margins were sent for frozen section examination to confirm completeness of excision. Medial margin showed pagets cells in frozen section and medial margin was revised leading to a hemiscrotectomy right side with primary closure of the defect. Final histopathology confirmed the frozen findings and only medial margin had positive edge [Figure 3]. The revised margins of excision were negative making an R0 resection possible. With vague plaque like lesions of pagets where naked eye examination does not correspond to exact extend of disease, dermoscopy serve as a tool in mapping out lesions and for early diagnosis. The same is true in repeated margin positive excisions like this case where dermascopy combined with frozen section helped in getting a complete resection. | Figure 1: Dermoscopy picture (10×) showing white structure less areas (yellow arrow), lava lake structures (blue arrow) and linear irregular vessels (red arrow)
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 | Figure 2: Margin of the scrotal lesion marked with the help of dermoscopy
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 | Figure 3: high power view (40x) HE stain showing large pagetoid cells with abundant eosinophilic cytoplasm with large vesicular nucleus and hyperkeratosis
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EMPD is a rare disease, and the common presenting features include pruritus, bleeding, oozing, tenderness, painful burning sensation or hypopigmented/eczematous lesions and depigmented lesions.[2] The major clinical picture is progression despite topical therapy and the sharp raised margins. The differential diagnosis includes many innocuous dermatological diseases like neurodermatitis, eczemas, psoriasis, lichen planus. The cell of origin in primary EMPD is pleuripotent keratinocyte stem cells and Toker cells.[1] It may be a primary intraepithelial neoplasm or arising from primary adenocarcinoma of skin appendage. Secondary EMPD results from direct extension or metastasis from another site such as genitourinary or gastrointestinal carcinoma. The probability of internal malignancy is up to 45% in perianal and 11% in male genital EMPD.[3] Intraepithelial EMPD rarely metastasize but invasive forms may spread to lymphatics, bone, liver or lungs.[4] Due to the non-specific clinical features diagnosis of EMPD is often delayed by months or years. Dermoscopy of extramammary Paget disease has been reported to show two unique features, white small round clods with white structureless areas ('cloud-like structureless areas') and thick branching white lines with intermingled white clods (lava lake structures). In addition to this milky red areas and vascular patterns like glomerular vessels, linear irregular vessels are also reported.[5] Our patient had all these features and it helped in mapping the margins prior to surgery. Dermoscopy can be a useful tool in early diagnosis as well as in delineating the margins during excision. A combined approach by dermatologist and surgeon will ensure attaining better results and prognosis in EMPD.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Zhu Y, Ye D-W. Preneoplastic and primary scrotal cancer: Updates on pathogenesis and diagnostic evaluation. Urol Clin North Am 2016;43:523-30.  |
| 2. | Banerjee S, Chatterjee M, Chand K. Extramammary paget's disease. Indian J Dermatol Venereol Leprol 2005;71:417. |
| 3. | Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol JEADV 2007;21:581-90. |
| 4. | Dai B, Kong Y-Y, Chang K, Qu Y-Y, Ye D-W, Zhang S-L, et al. Primary invasive carcinoma associated with penoscrotal extramammary Paget's disease: A clinicopathological analysis of 56 cases. BJU Int 2015;115:153-60. |
| 5. | Payapvipapong K, Nakakes A, Tanaka M. Lava lake structure and cloud-like structureless area: New clues for diagnosing extramammary Paget disease. J Eur Acad Dermatol Venereol 2017;31:e459-61. |
[Figure 1], [Figure 2], [Figure 3] |
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