Acta Dermato-Venereologica 99-9CompleteContent | Page 21

826 SHORT COMMUNICATION Meningeal Carcinomatosis in Advanced Extramammary Paget’s Disease: A Case Report Ayano WATANABE, Masazumi ONISHI, Kanako TSUNODA and Hiroo AMANO* Department of Dermatology, Iwate Medical University, Uchimaru 19-1, Morioka 020-8505, Japan. *E-mail: [email protected] and [email protected] Accepted Apr 30, 2019; E-published May 2, 2019 Extramammary Paget’s disease (EMPD) is a rare in­ traepithelial malignant tumour that arises mostly in the epidermis of areas where apocrine glands are distributed, i.e. mainly in the anogenital and axillary skin. In general, the prognosis of EMPD is good when the tumour cells are limited to the epidermis, but poor when they invade the dermis and metastasize to lymph nodes or other organs. Nodal metastasis is reportedly one of the most important prognostic factors in EMPD (1). On the other hand, brain metastasis from EMPD is very rare, and there have been no reports of meningeal carcinomatosis (MC) from EMPD. We report here a rare case of MC in advanced EMPD due to bone metastasis and discuss the features of brain metastasis from EMPD. If cognitive impairment and dementia-like disorientation occur suddenly, MC and brain metastasis should be suspected, and magnetic reso­ nance imaging (MRI) examination should be conducted. CASE REPORT Fig. 1. (A) Physical examination of the Extramammary Paget’s disease (EMPD) lesion on both labia majora with indurated erythema and an ulcerative tumour on the right labium majorum. (B) Paget cells had infiltrated the epidermis and dermis, and the tumour cells also invaded the lymph vessels (haematoxylin-eosin (HE), original magnification ×200). Cytopathological examination confirmed the diagnosis of MC from EMPD (Fig. 3). Disturbance of consciousness gradually progressed, and the patient died approximately one month after diagnosis of MC. DISCUSSION A 65-year-old woman visited our hospital because of genital blee­ EMPD is a malignant neoplasm arising from apocrine ding and bilateral leg oedema. She had a medical history of spinal column stenosis and appendicitis. Physical examination revealed sweat glands, such as those in the anogenital and axillary indurated erythema on both labia majora, accompanied by an regions. The most common pattern of EMPD metastatic ulcerative tumour on the right labium majorum (Fig. 1A). Right spread is via the lymphatic system or intra-epithelial inguinal lymph node enlargement was evident, and a skin biopsy infiltration. The prognosis of EMPD is favourable if of the tumour yielded a diagnosis of EMPD. Positron emission diagnosis and treatment are started early, but poor if tomography (PET) demonstrated 18 F-fluorodeoxyglucose (FDG) uptake in multiple lymph nodes and sagittal T2-weighted MRI distant metastases are present. Nodal metastasis is re­ revealed high signal intensity in the L4 vertebral bone (Fig 2A). portedly one of the most important prognostic factors The genital tumour was resected palliatively. Histopathological in EMPD (1). To our knowledge, only a few cases of examination demonstrated that Paget cells had proliferated in the EMPD with brain metastases have been reported (2, 3), epidermis and dermis, and had also invaded lymph vessels (Fig. and meningeal carcinomatosis (MC) from EMPD has 1B). Docetaxel monotherapy was administered monthly at a dose of 60 mg/m 2 . After 3 cycles, the nodal disease disappeared on not been documented previously. PET images and the high signal intensity in the L4 vertebral bone was reduced. After 5 cycles, gadolinium-enhanced T1-weighted MRI revealed multiple small spots in the brain, suggesting metastases of EMPD to the central nervous system (CNS) (Fig. 2B). We performed 30 Gy of cranial ir­ radiation in 10 fractions. This reduced the brain metastases, but the patient developed nausea, vomiting and gait disturbance, fol­ lowed gradually by memory impairment and trunk dystonia. Gadolinium-enhanced T1- weighted MRI demonstrated enhancement along the cerebellar sulcus of the brain (Fig. 2C). For diagnosis, lumbar puncture was Fig. 2. Magnetic resonance imaging (MRI) image. (A) Sagittal T2-weighted MRI revealed an performed, and analysis of the cerebrospinal axial bone metastasis in the L4 vertebra. (B) Gadolinium-enhanced T1-weighted MRI revealed fluid (CSF) revealed an increased WBC multiple metastatic lesions in the brain. (C) Gadolinium-enhanced T1-weighted MRI revealed count and an elevated CSF protein level. enhancement along the cerebellar sulcus of the brain. doi: 10.2340/00015555-3211 Acta Derm Venereol 2019; 99: 826–827 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica.