Extramammary Paget's disease (EMPD) is a rare cutaneous carcinoma of epidermal origin. The diagnosis is frequently delayed, and the disease tends to be associated with an underlying adnexal or internal malignancy. There have been several reports of EMPD associated with carcinoma of the bladder, prostate, kidney, and colon. The association of hepatocellular carcinoma (HCC) with EMPD appears to be exceedingly rare; to our knowledge, it has been reported only once in the English literature. Herein, we report an unusual case of EMPD of the scrotum associated with HCC. EMPD was diagnosed 1 year after the appearance of an erythematous plaque, and HCC was noted 19 months after the diagnosis of EMPD. From our experience and literature review, in patients with nonspecific skin lesions that are unresponsive to conventional treatment, EMPD should be considered and skin biopsy performed. Long-term follow-up is needed to watch for the appearance of adnexal carcinoma or internal malignancy.

Primary cutaneous large B cell lymphoma, leg type (PCLBCL-leg) has recently been identified and recognized as a specific entity. Patients with PCLBCL-leg have a higher relapse rate and a poorer prognosis than the other types of primary cutaneous B cell lymphomas, and disease relapse is confined to the skin in the majority of cases with rare exclusive extracutaneous progression. The late occurrence of lymphoma in patients with a prior history of lymphoma may represent a relapse/progression or a distinct tumor unrelated to the original one. The distinction is of important clinical and therapeutic implications. Here, we report the case of a 90-year-old lady with a history of PCLBCL-leg in complete remission after radiotherapy that developed a huge, solitary pulmonary lymphoma without lymphadenopathy 14 months later. The latter was initially considered as stage IE primary pulmonary lymphoma and was treated with combination chemotherapy resulting in complete remission. Retrospective pathologic review and B cell clonality study revealed that the pulmonary tumor was a diffuse large B cell lymphoma of the same clonal origin as the PCLBCL-leg. This case is unique in the exclusive pulmonary relapse and illustrates the importance of expert pathological review and molecular study in the management of lymphoma patients with unusual clinical features.