Here we report a case of a 72-year-old male patient recurred in bone marrow alone with pulmonary tumor embolism after an excision of extramammary Paget’s disease of scrotum 3 years ago. The patient received paclitaxel/carboplatin chemotherapy with respiratory support in intensive care unit. Four days after chemotherapy, the oxygen demand decreased and the patient was transferred to general ward. The platelet count recovered after 2 weeks. Finally, he died of hepatic failure from Paget’s disease hepatic involvement confirmed by liver biopsy at 10 months after recurrence. This is a rare case of recurred extramammary Paget’s disease in bone marrow alone with pulmonary tumor embolism, which was properly diagnosed with high suspicion and was successfully treated with immediate chemotherapy.
Extramammary Paget’s disease (EMPD) is a rare cutaneous adenocarcinoma [
A 72-year-old male patient presented with anorexia, weight loss and progressive dyspnea of exertion over one month. Eight years earlier, he had been diagnosed of prostate cancer and he was treated with radical prostatectomy. Three years earlier, he was diagnosed for Paget’s disease in scrotum and received two steps operation because residual tumor was detected in resection margin. He was finally treated with wide excision and local flap. He was without evidence of disease on routine surveillance exam.
He had a 20 pack-year history of smoking but never had any breathing difficulty before. The physical examination including chest auscultation was unremarkable. He was afebrile with tachycardia of 108 beats/min, respiratory rate of 33/min, and blood pressure of 146/90 mm Hg. Arterial blood gas analysis showed paO2=59.4 mm Hg and paCO2=27.7 mm Hg while D-dimer levels were slightly elevated (5.87 μg/mL, reference range=less than 0.4 μg/mL). White blood cell, hemoglobin, and platelet count were 4,750/mm3, 13.5 g/dL, and 97,000/mm3, respectively. Prothrombin time was slightly prolonged to 15.4 seconds but partial thromboplastin time and fibrinogen were in normal range.
Pulmonary function test showed normal ventilator pattern, but decreased diffusing capacity of the lungs for carbon monoxide (DLCO=68%). Contrast enhancement computed tomography (CT) scan did not show any intraluminal filling defects of pulmonary arteries and deep veins of low extremities (
Anticoagulation therapy was suggested, but was postponed because progressive thrombocytopenia was detected. Multiple aggregations of large sized abnormal cells were observed in bone marrow specimen, suspecting malignancy. These tumor cells were same histology and immunohistochemical staining pattern (caudal-related homeobox 2 [CDX2, −], carcinoembryonic antigen [CEA, +], cytokeratin [CK] 7 [+], CK19 [+], CK20 [−], epithelial membrane antigen [EMA, +], prostate-specific antigen [PSA, −], thyroid transcription factor-1 [TTF-1, −]) with previous EMPD of scrotum (
The patient received paclitaxel/carboplatin chemotherapy with respiratory support in intensive care unit. Four days after chemotherapy, the oxygen demand decreased and the patient was transferred to general ward. The platelet count recovered after 2 weeks (
EMPD is a rare cutaneous malignancy from intraepidermal region around apocrine gland. Distal metastasis beyond regional lymph nodes is rare and only few cases were reported [
Kane et al. [
Standard chemotherapy regimens for locally advanced or metastatic EMPD have not been established [
This case is a rare presentation of bone marrow metastasis with PTE secondary to EMPD of scrotum, which was properly diagnosed with high suspicion and was successfully treated with immediate chemotherapy.
This case was present in 41th annual meeting of Korean Cancer Association in 2015.
(A) Absence of filling defect on computed tomography pulmonary angiography. (B) No evidence of parenchymal disease on computed tomography scan, pulmonary window setting.
(A) Lung ventilation and (B) perfusion scan: multiple perfusion defects (arrows). ANT, anterior images.
Initial pathologic finding of scrotum. Immunohistochemical staining of surgical specimen (reduced from ×200) with (A) caudal-related homeobox 2 (CDX2, −), (B) carcinoembryonic antigen (CEA, +), (C) cytokeratin (CK) 7 (+), (D) CK19 (+), (E) CK20 (−), (F) epithelial membrane antigen (EMA, +), (G) prostate-specific antigen (PSA, −), and (H) thyroid transcription factor-1 (TTF-1, −).
Pathologic finding of bone marrow involvement. Immunohistochemical staining with (A) caudal-related homeobox 2 (CDX2, −), (B) carcinoembryonic antigen (CEA, +), (C) cytokeratin (CK) 7 (+), (D) CK19 (+), (E) CK20 (−), (F) epithelial membrane antigen (EMA, +), (G) prostate-specific antigen (PSA, −), and (H) thyroid transcription factor-1 (TTF-1, −). (I) Hematoxylin and eosin (H&E) stained image of bone marrow biopsy specimen (reduced from ×200).
Platelet count (green) and CEA level (blue) change after chemotherapy. CEA, carcinoembryonic antigen.