CD8+ mycosis fungoides with esophageal involvement: A case report

Abstract

Abstract. Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma and is characterized clinically by an indolent course with slow progression. MF is limited to the skin with widespread distribution, however, extracutaneous involvement of MF occurs during the advanced stages of the disease. Esophageal involvement of MF is a rare event.In the present study, we describe the first documented case of CD8 + MF with esophageal involvement that was endoscopically diagnosed antemortem. A 70-year-old male with a 15-year history of MF presented with difficulty in swallowing. Endoscopic examination revealed a tumorous lesion with ulceration in all regions of the esophagus. Esophagus biopsy demonstrated atypical lymphocytic infiltrates with ulceration. Immunohistochemically, these atypical lymphocytes were positive for CD3, CD8 and cytotoxic granules. Therefore, a diagnosis of CD8 + MF involving the esophagus was made. Extracutaneous involvement of the esophagus in MF is extremely rare and the majority of previously reported cases have been diagnosed postmortem. Only two cases of MF with esophageal involvement endoscopically diagnosed antemortem have been previously reported and this is the first documented case of CD8 + MF with esophageal involvement diagnosed by this method. Early detection of extracutaneous involvement of MF is important for accurate treatment and endoscopic examination is a useful tool for detection of this pathology. IntroductionMycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma accounting for approximately 50% of all primary cutaneous lymphoma (1). MF is characterized clinically by an indolent course with slow progression, over years or occasionally decades, from patches to more infiltrated plaque and eventually tumor stages (1). MF is primarily limited to, but widely distributed on, the skin. However, during the advanced stages of the disease, MF has been identified in various extracutaneous regions, most commonly the lymph node, spleen, liver and lung (2). Esophageal involvement of MF has been previously identified, however cases are extremely rare (2-5).MF is characterized histopathologically by infiltrates of small to medium-sized neoplastic T lymphocytes with cerebriform nuclei demonstrating epidermotropism. The typical phenotype of neoplastic lymphocytes is CD3 + , CD4+ and CD8-(6). CD8 + MF has been identified, however, it is rare (6,7). In the present study, we report the first documented case of CD8 + MF with esophageal involvement that was diagnosed endoscopically antemortem. Patient and methodsCase report. A 70-year-old Japanese male with a 15-year history of MF presented with difficulty in swallowing. From the age of 56 years old, the patient reported observation of a gradually enlarging erythema on his left elbow. Four years following this, the patient was referred to Shiga University of Medical Science Hospital due to enlargement of the erythema across his entire body, accompanied by erosion in specific regions. Biopsy of the erythema led to di...

Aggressive epidermotropic cutaneous CD8+ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features. Report of an EORTC Cutaneous Lymphoma Task Force Workshop

“…Most patients in the study group followed a protracted, relatively benign course in accord with classical CD4 MF. The findings herein convincingly indicate CD8 + MF to be indistinguishable from the usual CD4 + form of disease, differing only in the single immunophenotypical anomaly; furthermore, they are in agreement with previous authors who contend that CD8 expression, in and of itself, cannot be taken to imply a poor prognosis . The same assertion has been made with regard to the CD4 − /CD8 − variant of otherwise typical MF and CD56 + disease .…”

A Rare Case of Mycosis Fungoides in the Oral Cavity and Small Intestine Complicated by Perforation

“…There have been reports of MF cases throughout the GI tract, including the small intestine (Table 1) [3, 4, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43]. Briefly, there have been 67 cases of GI MF in the reported literature.…”

Other Chemotherapeutic Agents in Cutaneous T-Cell Lymphoma

“…Several other cases with modified CHOP regimens have also been described replacing epirubicin for doxorubicin. 94,95 It is currently not known if epirubicin has any advantage over doxorubicin in multiagent regimens for MF/SS. Moreover, foregoing vincristine is not known to be significantly safer or superior to standard CHOP.…”