Unusual cutaneous Langerhans cell sarcoma without extracutaneous involvement

Abstract

Langerhans cell sarcoma (LCS) typically presents as cytologic atypia and clinical aggressiveness and may involve multiple organs during the progression of the disease. Primary skin LCS without any extra-cutaneous site association is extremely rare and only a few such cases have been described in the literature. We present a case of unusual primary LCS in skin occurring in a middle-aged male patient. Physical examination revealed a large ulcerated cutaneous lesion and a smaller nodular lesion were located in the skin of the extensor side of his right knee. There was no regional lymph node or any other extra-cutaneous organ involvement. Histologically, typical large and pleomorphological tumor cells with epithelioid appearance and significantly malignant cytological features were observed to infiltrate in dermis and subcutaneous tissue. By immunohistochemistry, the tumor cells were positive for CD1a, S-100 protein and largerin strongly and diffusely. However, these cells were negative for CD3, CD20, CD21, pan-cytokeratin, HMB-45, Melan-A, and MPO. A diagnosis of primary cutaneous LCS was made. The patient received systemic chemotherapy of CHOP regimen, and was on a regular follow-up period for 12 months. There was no sign of relapse of tumor or any other extra-cutaneous organ involvement by whole body positron emission tomography/computed tomography (PET/CT) study. Because LCS is a high-grade malignancy with poor prognosis, it suggests that strict histological analysis and thorough radiographic examination are necessary for accurately diagnosing this tumor even if cutaneous involvement presented only.Virtual slideshttp://www.diagnosticpathology.diagnomx.eu/vs/6527428618381393

Cutaneous Langerhans Cell Sarcoma: A Case Report

“…Myeloid leukemia can originate in the skin, and CD68 and lysozyme positivity may be observed, which could make this condition difficult to distinguish from LCS. The presence of myeloid-specific markers, such as myeloperoxidase (MPO), CD117, CD99, and CD34, should be helpful in differentiating cutaneous myeloid leukemia from cutaneous myeloid sarcoma [10]. In our patient, the tumor cells strongly expressed CD1a, S-100 protein, and langerin, markers specific for LC.…”

Cutaneous and breast Langerhans cell sarcoma

“…It has been suggested that a subtype of patients with purely cutaneous LCS may have a more favourable outcome. 9,10 However, our case showed a rapid progression until death, which suggests that breast involvement in the course of LCS could be a sign of bad prognosis.…”

Vulvar “proximal-type” epithelioid sarcoma: report of a case and review of the literature

“…S 100 and CD31 are negative, while desmin and CD34 are positive in some cases [18]. Langerhans cell sarcoma (LCS) or mammary sarcoma (MS) may be similar to PES but immunohistochemistry, in the LCS the tumour cells are positive for CD1a, S-100 protein and langerin strongly and diffusely [29], while in MS CD10 and vimentin are positive diffusely whereas epithelial markers and other myoepithelial or myogenic markers were all negative [30]. In Prostatic stromal sarcoma (PSS) unlike the PES, immunohistochemically, the tumour cells are widely positive for vimentin, CD56, CD99 and focally positive for synaptophysin, CD10, progesterone receptor, desmin and CD34, but negative for EMA, cytokeratin, estrogen receptor, S-100 and myoglobin [31].…”