Abstract

Myeloid sarcoma, considered to herald the onset of a blast crisis in the setting of chronic myeloproliferative neoplasm/dysplasia, typically presents during the course of the disorder. Cutaneous involvement is uncommon and lesions on genital skin are seldom seen. We present a case of a well-differentiated myeloid sarcoma in the penile foreskin in an apparently healthy 29-year-old male presenting with phimosis. The unusual composition of the inflammatory cell infiltrate, and characteristic sparing of dermal blood vessels, nerves and smooth muscle fibres led to the correct diagnosis. Absence of commonly observed changes in the circumcision skin like those of balanitis xerotica was also helpful. Detailed hematological work up revealed a previously undiagnosed chronic myeloid leukemia in chronic phase. The patient also had simultaneous priapism, another rare presentation of chronic myeloid leukemia. One year hence, the patient is in hematological remission with no evidence of extramedullary disease. Although priapism has been described as a rare presenting symptom in chronic myeloid leukemia, the present case is unique as this is the first time a cutaneous myeloid sarcoma has been documented in the penile foreskin.

Highlights

  • Myeloid sarcoma (MS) refers to a rare solid tumor composed of immature myeloid cells at an extramedullary site [1]

  • We report a case of simultaneous phimosis with priapism as the first presentation of a previously undiagnosed case of chronic myeloid leukemia (CML)

  • The present case is unique as a cutaneous MS was identified on histopathological examination of the circumcision specimen of the penile foreskin

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Summary

INTRODUCTION

Myeloid sarcoma (MS) refers to a rare solid tumor composed of immature myeloid cells at an extramedullary site [1]. Most cases develop in the course of a leukemia; its presentation concurrent to or preceding leukemia is uncommon [1,2]. Priapism, sustained penile erection in the absence of physiologic stimuli, indicates the disturbance of the regulatory mechanisms responsible for penile erection and detumescence [5]. It is an uncommon but well-documented complication of sickle cell disease but sparingly reported in leukemias [6]. The present case is unique as a cutaneous MS was identified on histopathological examination of the circumcision specimen of the penile foreskin

CASE REPORT
Turkish Journal of Pathology
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