A true epidermotropic apocrine neoplasm in the form of perianal Paget’s disease: a case report

Јankulovski, Nikola; Spasevska, Liljana; Janevska, Vesna; Dukova, Blagica

doi:10.1186/1752-1947-7-162

Cited by 5 publications

(4 citation statements)

References 10 publications

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“…The present case corresponds to stage I according to Shutze and Gleysteen classification [ 30 ]. Recommendation for stages I and II is a wide local excision [ 13 ]; however, Imiquimod was successfully an alternative treatment in our case. Long-term follow-up is required to exclude the recurrence of the disease and development of associated tumors.…”

Section: Discussionmentioning

confidence: 75%

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Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy

et al. 2018

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BackgroundPerianal Paget’s disease (PPD) is a rare intraepithelial adenocarcinoma of the anal margin. Primary PPD likely represents intra-epithelial neoplasm from an apocrine source, whereas secondary disease may represent “pagetoid” spread from an anorectal malignancy.Case presentationHistologic CDX-2 and CK20 are hallmark markers for colorectal-derived Paget’s cells. Interestingly, our primary PPD patient presented both positive and no internal malignancy was identified. In addition, a negative CK7 marker was observed in our case in contrast with previously reported. Surgical excision is the standard treatment; however, previous studies have demonstrated good response with Imiquimod 5% cream in patients with vulval extramammary Paget disease (EMPD). The efficiency of Imiquimod treatment for PPD has not been well described. Our PPD patient was successfully treated using Imiquimod 5% cream.ConclusionsThis study describes a primary cutaneous PPD patient CDX-2+/CK20+/CK7- without invasion of the dermis and no associated colorectal carcinoma effectively treated using topical Imiquimod therapy, suggesting that Imiquimod might potentially be considered as a first-line treatment for PPD.

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Section: Discussionmentioning

confidence: 75%

“…Differential diagnoses of PPD include Bowen’s disease, contact dermatitis, lichenoid lesions, psoriasis, melanoma, perianal Crohn’s involvement, mycosis fungoides, squamous cell carcinoma and tinea cruris [ 13 ]. Immunostaining markers may enhance the diagnostic accuracy.…”

Section: Discussionmentioning

confidence: 99%

Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy

et al. 2018

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“…Clinical symptoms of PPD mainly include erythematous, whitish gray, dry and raised lesions, but may also develop into eczematoid, ulcerated, nodular, or papillary forms. [8] The differential diagnosis of these lesions includes eczema, contactable or irritant dermatitis, seborrheic dermatitis, or Bowen's disease. [9] The diagnosis of the disease requires biopsy and immunohistochemistry.…”

Section: Discussionmentioning

confidence: 99%

Laparoscopic extralevator abdominoperineal excision for the treatment of perianal Paget's disease

et al. 2019

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Rational: Perianal Paget's disease (PPD) is a very rare intraepithelial adenocarcinoma. Very few cases of PPD have been reported till date, so the treatment remains controversial and more experience is needed. Patient concerns: A 73-year-old female was admitted to several hospitals with diagnosed as “perianal eczema” in perianal lesion. After a variety of treatments, the patient's condition did not improve. Diagnoses: Abdominopelvic computed tomography and rectal magnetic resonance imaging showed thickening of the soft tissue around the anus, with significant enhancement. Histologic examination revealed the state of Paget's cells. Interventions: Laparoscopic Extralevator Abdominoperineal Excision (ELAPE) surgery was performed at our department. Outcomes: The patient recovered well. After 10 months, a check-up revealed that her perianal area was disease-free. Lessons: The aim of this report was to present the characteristics of PPD in order to improve its diagnosis and treatment. Laparoscopic ELAPE is a successful therapy.

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“…The focus is on monitoring disease recurrence and development of an associated cancer. [ 16 ] The margin of the old perianal lesion should be examined by punch biopsy every year, and the patient should be referred to colonoscopy examination every 2 years during the follow-up period. [ 17 ]…”

Section: Discussionmentioning

confidence: 99%

Perianal Paget disease treated with wide excision and thigh skin flap reconstruction

Shen

Hai

et al. 2018

Medicine

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Rationale:Extramammary Peget disease (EMPD) is a rare tumor, which typically occurs in the perianal regions. Perianal Paget disease (PPD) was first reported in 1893, and which has only 180 cases that have been reported in literature. The rarity of the disease means that no large studies have been made, and so the optimal treatment for this disease still remains controversial.Patient concerns:In this case, we reported a 65-years-old female patient with PPD. The patient suffered intermittent pruritus in the perianal region for 1 year. She had neither genitourinary nor gastrointestinal symptoms. Local examination revealed a whitish gray skin lesion in the left perianal area with a 3 × 3 cm size.Diagnoses:The perianal skin biopsy was consistent with EMPD. Then the patient underwent a screening colonoscopy, gynecological ultrasonography, and whole-body computed tomography to exclude underlying malignancy.Interventions:The patient underwent wide local excision with margin control by frozen section examination and posterior thigh flap reconstruction. Subsequently follow-up remains 6 years.Outcomes:The operation was successful. The total operation time was 296 minutes, and the estimated blood loss was 120 mL. The patient recovered without any complication and discharged home on the sixth postoperative day. After 6 years’ follow-up, there was no evidence of recurrence and no influence in the anal bowel control function.Lessons:PPD is a rare disorder; the current knowledge on diagnose and treatment is based on small case series. Thus, it is complicated to elaborate a consensus on diagnostic and treatment guidelines. Wide local excision remains the treatment of choice with a variety of adjuvant therapies. Our method has an advantage which is the posterior thigh flap could be designed in accordance with the defect of the perianal. It is mandatory that the patient must accept a long-term follow-up to detect local recurrence and to distant carcinoma.

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A true epidermotropic apocrine neoplasm in the form of perianal Paget’s disease: a case report

Cited by 5 publications

References 10 publications

Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy

Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy

Laparoscopic extralevator abdominoperineal excision for the treatment of perianal Paget's disease

Perianal Paget disease treated with wide excision and thigh skin flap reconstruction

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