BRAF wild‐type recurrent indeterminate dendritic cell tumour presenting with leonine facies

Abstract

BRAF wild-type recurrent indeterminate dendritic cell tumour presenting with leonine facies Editor Indeterminate dendritic cell tumour (IDCT) was a rare histiocytosis originating from stromal dendritic accessory cells. 1 Only two studies have described leonine facies as the dominant feature of IDCTs. 2 Here, we report another IDCT presenting with leonine facies. A 37-year-old man presented with 4-year history of diffuse papules on his cheeks and then gradually spreading to his face, back and extremities. Some facial papules fused into bumps and nodules, accompanied by slight pruritus. Skin examinations showed multiple pinkish red nodular lesions coalesced into irregularshaped or rod-like protuberances on his forehead, both cheeks, eyelids, temples and pinnas of ears (Fig. 1a,b). Diffuse red papules scattered throughout his legs, posterior surface of forearms and lower back, with old greyish and wrinkled lesions among them (Fig. 1c). Dermatoscopy revealed patchy, branchy and spiral vessel dilations with keratosis and scales scattered on red background (Fig. 1d). Biopsy showed dermis diffusely infiltrated by histiocytoid cells with nuclear grooves and eosinophilic cytoplasm but atrophic and uninvolved epidermis (Fig. 1e). The histiocytoid cells were CD1a and CD68 positive (Fig. 1f,g), S-100 focally positive and Langerin negative (Fig. 1h). Ki-67 index was 25%. The diagnosis was revised from previous Langerhans cell histiocytosis (LCH) to IDCT based on the absence of Langerin. Since BRAF V600E mutation and BRAF fusions have been reported in malignant histiocytosis, 3,4 a Human BRAF V600E ARMS-PCR Kit and a next-generation sequencing (NGS) array were applied but neither BRAF V600E mutation nor BRAF fusions were found. The details and outcomes of

Overall clinical course of indeterminate dendritic cell tumor patients without skin lesions: A rare case report

“…Therefore, conducting more collective and in-depth studies is critical for clarifying the pathological mechanism of IDCT. First, patients with IDCT should be sent to large specialized medical centers, such as the Peking Union Medical College Hospital[ 13 ]. One reason is that it will be more convenient to conduct related clinical trials.…”

Typical leonine facies in a patient with indeterminate dendritic cell tumor

“…1 Only three cases have reported leonine facies as the major characteristic of IDCTs. 2 Here we report another case presented with typical leonine facies and generalized multiple confluent papules and nodules on the whole body without systemic involvement.…”

Recurrent Chemotherapy Treated Indeterminate Dendritic Cell Tumor: Case Report and Literature Review

“…:DOC 4 [ 21 ] Chen (2018) Male 31 MF N0 No MTX, no response, transfer to isotretinoin and thalidomide Improvement on skin, under follow up: SD 48 [ 17 ] Zhou (2019) Male 37 MF Yes No No Corticosteroids, vinca alkaloids, CTX Utilizing a range of treatments, yet constantly experiencing remission and relapse within 5 years. SD with RR 60 [ 16 ] Fischer (2021) Female 55 MF No No Thalidomide 6 months: lesion progress:PD 6 [ 15 ] Xu (2017) M MF No No Thalidomide PR 10 [ 18 ] Xu (2017) F 53 MF No No Methotrexate CR 10 [ 18 ] Xu (2017) M …”

“…We reviewed the current examples of IDCT with cutaneous symptoms treated with chemotherapy medications to assess the efficacy and safety of chemotherapy drugs 1,5,[11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] (Table 1). We found that remission and relapse occurred in 8 out of 26 chemotherapy-treated patients reported to date.…”

“…We found that remission and relapse occurred in 8 out of 26 chemotherapy-treated patients reported to date. 5,11,16,24,25 Among the reported cases, six of them achieved complete remission. The average follow-up period was 47 months (range: 10-114 months).…”