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Advanced Extramammary Paget's Disease of the Groin, Penis, and Scrotum.

Isrow D, Oregel KZ, Cortes J, Gomez H, Milikowski C, Feun L, Silva O - Clin Med Insights Oncol (2014)

Bottom Line: The rarity of EMPD, especially of the penis and scrotum, warrants an educated eye and heightened index of suspicion when dealing with eczematous lesions in the groin in any person.There are no guidelines available to treat locally advanced unresectable disease.In addition, further studies are needed to identify genetic defects underlying the pathogenesis of this rare disease, to help improve treatment strategies and decrease morbidity.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology/Oncology, Department of Medicine, Sylvester Comprehensive Cancer Center, Braman Family Breast Cancer Institute Miller School of Medicine in Miami, The University of Miami, Miami, FL, USA.

ABSTRACT
Extramammary Paget's disease (EMPD) is a rare intraepithelial malignancy arising in areas rich in apocrine glands, such as the perineum, vulva, axilla, scrotum, and penis. We describe the case of a man in his 50s who initially presented with a small eczematous lesion on his right groin, treated with topical ointments for eczema, until excisional biopsy of lesion unequivocally revealed invasive EMPD. Despite aggressive surgical interventions, his disease progressed to involve the scrotum and penis. Deemed unresectable, the patient was treated with systemic chemotherapy with minimal response. The rarity of EMPD, especially of the penis and scrotum, warrants an educated eye and heightened index of suspicion when dealing with eczematous lesions in the groin in any person. Early biopsy and histological examination is crucial for early surgical intervention of the lesions. There are no guidelines available to treat locally advanced unresectable disease. In addition, further studies are needed to identify genetic defects underlying the pathogenesis of this rare disease, to help improve treatment strategies and decrease morbidity.

No MeSH data available.


Related in: MedlinePlus

(A) Suprapubic skin: The epidermis is normal. Within the dermis are groups of atypical cells with abundant amphophilic cytoplasm. Nuclei are pleomorphic and hyperchromatic. The cellular features are consistent with an apocrine morphology. (B) Atypical cells present in dermal lymphovascular spaces. (C) Lymph node showing metastatic apocrine carcinoma.
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f1-cmo-8-2014-087: (A) Suprapubic skin: The epidermis is normal. Within the dermis are groups of atypical cells with abundant amphophilic cytoplasm. Nuclei are pleomorphic and hyperchromatic. The cellular features are consistent with an apocrine morphology. (B) Atypical cells present in dermal lymphovascular spaces. (C) Lymph node showing metastatic apocrine carcinoma.

Mentions: A man in his 50s with locally advanced EMPD involving the skin of groin, scrotum, and penis was admitted to our center for his third cycle of cisplatin/doxorubicin chemotherapy in August 2011. The patient’s medical history includes “numerous” squamous and basal cell carcinomas removed in the 1990s from his face and upper extremities, but no melanomas were reported. In addition, the patient has an extensive family history of skin cancers and breast cancer. Both the patient’s parents developed skin cancers at early ages, none of which were reported as melanoma. The patient’s two maternal aunts developed breast cancer at 35 and 70 years of age, and all four of the patient’s sisters developed breast cancers at 35, 40, 45, and 47 years of age. The patient’s history of EMPD began in 2002, when he observed a small eczematous lesion which appeared on his right groin which he initially dismissed it as a transient “rash”. It was not until 2008 that the lesion began to “itch, ooze, and become angrier,” at which time the patient consulted his dermatologist. It was diagnosed as eczema, and different ointments were administered, none of which provided any relief or improvement. In October 2009, the patient underwent punch biopsy of the right groin lesion which was unremarkable; however, excisional biopsy of the same lesion unequivocally revealed invasive apocrine adenocarcinoma and EMPD. Extensive resection and wide excision was performed at this time, but positive margins and focal lymphovascular invasion were observed. In January 2010, the patient developed similar lesions in his left groin and subsequent PET/CT showed diffused areas of high avidity in both left and right groin with bilaterally enlarged lymph nodes. In April 2010, the patient underwent bilateral wide excision and deep dissection of the groin lesions and lymph node dissection. Pathology showed metastatic carcinoma in 3 of the 20 lymph nodes in the right groin (Fig. 1). The left groin surgical specimen showed superficially invasive EMPD with no lymph node involvement. Immunohistochemistry stains for estrogen receptor (ER), progesterone receptor (PR), and Her2/neu were all negative. Yet another groin dissection was performed in December 2010 as well as a punch biopsy of a suspicious suprapubic lesion which was found to be EMPD. Despite all surgical interventions, the disease progressed to involve both the scrotum and penis. It was at this time that the tumor was considered unresectable and the patient was started on systemic chemotherapy in February 2011. He was initially treated with carboplatin and paclitaxel; however, after two cycles, he developed atrial fibrillation and his chemotherapy was changed to carboplatin/gemcitabine.


Advanced Extramammary Paget's Disease of the Groin, Penis, and Scrotum.

Isrow D, Oregel KZ, Cortes J, Gomez H, Milikowski C, Feun L, Silva O - Clin Med Insights Oncol (2014)

(A) Suprapubic skin: The epidermis is normal. Within the dermis are groups of atypical cells with abundant amphophilic cytoplasm. Nuclei are pleomorphic and hyperchromatic. The cellular features are consistent with an apocrine morphology. (B) Atypical cells present in dermal lymphovascular spaces. (C) Lymph node showing metastatic apocrine carcinoma.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4116380&req=5

f1-cmo-8-2014-087: (A) Suprapubic skin: The epidermis is normal. Within the dermis are groups of atypical cells with abundant amphophilic cytoplasm. Nuclei are pleomorphic and hyperchromatic. The cellular features are consistent with an apocrine morphology. (B) Atypical cells present in dermal lymphovascular spaces. (C) Lymph node showing metastatic apocrine carcinoma.
Mentions: A man in his 50s with locally advanced EMPD involving the skin of groin, scrotum, and penis was admitted to our center for his third cycle of cisplatin/doxorubicin chemotherapy in August 2011. The patient’s medical history includes “numerous” squamous and basal cell carcinomas removed in the 1990s from his face and upper extremities, but no melanomas were reported. In addition, the patient has an extensive family history of skin cancers and breast cancer. Both the patient’s parents developed skin cancers at early ages, none of which were reported as melanoma. The patient’s two maternal aunts developed breast cancer at 35 and 70 years of age, and all four of the patient’s sisters developed breast cancers at 35, 40, 45, and 47 years of age. The patient’s history of EMPD began in 2002, when he observed a small eczematous lesion which appeared on his right groin which he initially dismissed it as a transient “rash”. It was not until 2008 that the lesion began to “itch, ooze, and become angrier,” at which time the patient consulted his dermatologist. It was diagnosed as eczema, and different ointments were administered, none of which provided any relief or improvement. In October 2009, the patient underwent punch biopsy of the right groin lesion which was unremarkable; however, excisional biopsy of the same lesion unequivocally revealed invasive apocrine adenocarcinoma and EMPD. Extensive resection and wide excision was performed at this time, but positive margins and focal lymphovascular invasion were observed. In January 2010, the patient developed similar lesions in his left groin and subsequent PET/CT showed diffused areas of high avidity in both left and right groin with bilaterally enlarged lymph nodes. In April 2010, the patient underwent bilateral wide excision and deep dissection of the groin lesions and lymph node dissection. Pathology showed metastatic carcinoma in 3 of the 20 lymph nodes in the right groin (Fig. 1). The left groin surgical specimen showed superficially invasive EMPD with no lymph node involvement. Immunohistochemistry stains for estrogen receptor (ER), progesterone receptor (PR), and Her2/neu were all negative. Yet another groin dissection was performed in December 2010 as well as a punch biopsy of a suspicious suprapubic lesion which was found to be EMPD. Despite all surgical interventions, the disease progressed to involve both the scrotum and penis. It was at this time that the tumor was considered unresectable and the patient was started on systemic chemotherapy in February 2011. He was initially treated with carboplatin and paclitaxel; however, after two cycles, he developed atrial fibrillation and his chemotherapy was changed to carboplatin/gemcitabine.

Bottom Line: The rarity of EMPD, especially of the penis and scrotum, warrants an educated eye and heightened index of suspicion when dealing with eczematous lesions in the groin in any person.There are no guidelines available to treat locally advanced unresectable disease.In addition, further studies are needed to identify genetic defects underlying the pathogenesis of this rare disease, to help improve treatment strategies and decrease morbidity.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology/Oncology, Department of Medicine, Sylvester Comprehensive Cancer Center, Braman Family Breast Cancer Institute Miller School of Medicine in Miami, The University of Miami, Miami, FL, USA.

ABSTRACT
Extramammary Paget's disease (EMPD) is a rare intraepithelial malignancy arising in areas rich in apocrine glands, such as the perineum, vulva, axilla, scrotum, and penis. We describe the case of a man in his 50s who initially presented with a small eczematous lesion on his right groin, treated with topical ointments for eczema, until excisional biopsy of lesion unequivocally revealed invasive EMPD. Despite aggressive surgical interventions, his disease progressed to involve the scrotum and penis. Deemed unresectable, the patient was treated with systemic chemotherapy with minimal response. The rarity of EMPD, especially of the penis and scrotum, warrants an educated eye and heightened index of suspicion when dealing with eczematous lesions in the groin in any person. Early biopsy and histological examination is crucial for early surgical intervention of the lesions. There are no guidelines available to treat locally advanced unresectable disease. In addition, further studies are needed to identify genetic defects underlying the pathogenesis of this rare disease, to help improve treatment strategies and decrease morbidity.

No MeSH data available.


Related in: MedlinePlus