PMID- 35690998
OWN - NLM
STAT- MEDLINE
DCOM- 20230123
LR  - 20230123
IS  - 1600-0560 (Electronic)
IS  - 0303-6987 (Linking)
VI  - 50
IP  - 2
DP  - 2023 Feb
TI  - Microsecretory adenocarcinoma of the skin harboring recurrent SS18 fusions: A 
      cutaneous analog to a newly described salivary gland tumor.
PG  - 134-139
LID - 10.1111/cup.14271 [doi]
AB  - BACKGROUND: Microsecretory adenocarcinoma (MSA) is a newly described salivary 
      gland neoplasm characterized by MEF2C::SS18 fusions. MSA was previously thought 
      to occur exclusively in salivary glands. Here, we expand the spectrum of known 
      primary sites of this tumor by describing a series of cutaneous tumors with 
      analogous findings. METHODS: We identified four cutaneous primary tumors with 
      histopathologic features identical to MSA of the salivary glands. These cases 
      were evaluated by immunohistochemistry, fluorescence in situ hybridization (FISH) 
      for SS18 rearrangement and targeted RNA-sequencing. We also queried a pan-tumor 
      database of advanced carcinomas for MEF2C::SS18. RESULTS: The cases occurred in 
      men ranging from 61 to 74 years (mean, 68). They arose from the skin of the nose, 
      chin, scalp, and external auditory canal. All included cords/microcysts of 
      eosinophilic cells with bland oval nuclei and bluish mucin within fibromyxoid 
      stroma. The scalp tumor also exhibited high-grade transformation (marked atypia, 
      elevated mitotic rate, and necrosis), a feature unreported in salivary MSA. By 
      immunohistochemistry, all cases were positive for S100. Two showed a 
      myoepithelial component positive for p40 and smooth muscle actin or calponin. 
      Three cases harbored MEF2C::SS18 by RNA sequencing, while one with limited tissue 
      had SS18 rearrangement via FISH. Two patients had no evidence of recurrence or 
      metastasis in limited follow-up (3 and 6 months). The pan-tumor database query 
      also did not identify MEF2C::SS18 in any advanced cutaneous carcinomas. 
      CONCLUSION: This report expands the sites that can be involved by MSA. Similar to 
      salivary cases, MEF2C::SS18 represents a recurrent fusion in MSA of the skin. 
      Unusual features in cutaneous cases not seen in salivary MSA include one case 
      with high-grade transformation and two cases with a myoepithelial cell component. 
      Identification of this fusion expands the spectrum of salivary-analog cutaneous 
      tumors and aids in precise tumor classification.
CI  - (c) 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
FAU - Bishop, Justin A
AU  - Bishop JA
AUID- ORCID: 0000-0002-0412-6810
AD  - Department of Pathology, UT Southwestern Medical Center, Dallas, Texas, USA.
FAU - Williams, Erik A
AU  - Williams EA
AD  - UCSF Dermatopathology & Oral Pathology Service, Department of Pathology and 
      Dermatology, University of California, San Francisco, California, USA.
FAU - McLean, Anne C
AU  - McLean AC
AD  - Department of Pathology, UT Southwestern Medical Center, Dallas, Texas, USA.
FAU - Gagan, Jeffrey
AU  - Gagan J
AD  - Department of Pathology, UT Southwestern Medical Center, Dallas, Texas, USA.
FAU - Rooper, Lisa M
AU  - Rooper LM
AD  - Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland, USA.
FAU - Jordan, Richard C K
AU  - Jordan RCK
AD  - UCSF Dermatopathology & Oral Pathology Service, Department of Pathology and 
      Dermatology, University of California, San Francisco, California, USA.
FAU - LeBoit, Philip E
AU  - LeBoit PE
AD  - UCSF Dermatopathology & Oral Pathology Service, Department of Pathology and 
      Dermatology, University of California, San Francisco, California, USA.
LA  - eng
GR  - Jane B. and Edwin P. Jenevein M.D Endowment for Pathology at UT Southwestern 
      Medical Center/
PT  - Journal Article
DEP - 20220711
PL  - United States
TA  - J Cutan Pathol
JT  - Journal of cutaneous pathology
JID - 0425124
RN  - 0 (Biomarkers, Tumor)
SB  - IM
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Biomarkers, Tumor/genetics
MH  - *Adenocarcinoma/genetics
MH  - *Salivary Gland Neoplasms/genetics/pathology
MH  - *Carcinoma/pathology
MH  - *Skin Neoplasms/genetics/pathology
OTO - NOTNLM
OT  - MEF2C::SS18
OT  - SS18 fusion
OT  - cutaneous adenocarcinoma
OT  - microsecretory adenocarcinoma
EDAT- 2022/06/13 06:00
MHDA- 2023/01/24 06:00
CRDT- 2022/06/12 13:54
PHST- 2022/05/27 00:00 [revised]
PHST- 2022/04/29 00:00 [received]
PHST- 2022/06/06 00:00 [accepted]
PHST- 2022/06/13 06:00 [pubmed]
PHST- 2023/01/24 06:00 [medline]
PHST- 2022/06/12 13:54 [entrez]
AID - 10.1111/cup.14271 [doi]
PST - ppublish
SO  - J Cutan Pathol. 2023 Feb;50(2):134-139. doi: 10.1111/cup.14271. Epub 2022 Jul 11.