Description

Extra mammary Paget disease is an uncommon slow growing intraepithelial malignant neoplasm. It can be classified into primary and secondary subtypes, with secondary subtype associated with underlying malignancy. Extra mammary Paget disease of the pen scrotal region is extremely rare with very limited literature available describing its clinic pathologic characteristics. We present 6 cases of pen scrotal EMPD diagnosed at our academic medical center over a 20 year period. These included 5 cases of scrotal EMPD and 1 case of penile EMPD. The mean age at diagnosis was 68.6 yrs. (Range 61–78 years), One case of scrotal EMPD had history of renal cell carcinoma and prostatic adenocarcinoma, while one other case presented as recurrent EMPD with initial disease in the left groin.

Paget's disease of the bone is a non-malignant skeletal disorder characterized by focal abnormalities in bone remodelling at one or more skeletal sites. Pathological fractures occurring from trivial injuries are a well-known clinical presentation in patients with Paget's disease. An avulsion fracture of the tibial tuberosity is an infrequent injury and has an extremely low occurrence in adults, with only a few cases reported in literature. We describe a case of a patient with undiagnosed Paget's disease of the bone, sustaining a pathological avulsion fracture of the tibial tuberosity. Extramammary Vulval Paget’s Disease (VPD) is rare neoplasm of post-menopausal women. In relatively young premenopausal patients, it can cause both diagnostic dilemma and therapeutic challenges. Majority of VPD is of non-invasive variety. Among invasive Paget’s disease, only 20% cases show invasion more than 1 mm. The present report describes a unique case of an invasive extra-mammary VPD with depth of invasion more than 4 mm presenting at a relatively young premenopausal lady. Only 14 cases of VPD have been treated with primary radiotherapy in literature till date. We report this case to be the 15th case where radiotherapy was solely used to treat an invasive VPD.

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With Regards
Ishaq
Journal Coordinator
Journal of Reproductive Endocrinology & Infertility