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Syringocystadenoma papilliferum

MedGen UID:
98362
Concept ID:
C0406803
Neoplastic Process
Synonyms: Papillary Syringocystadenoma; Papillary Syringocystadenomas; Papilliferum, Syringocystadenoma; Syringocystadenoma Papilliferum; Syringocystadenoma Papilliferums; Syringocystadenoma, Papillary
SNOMED CT: Papillary syringadenoma (8934006); Papillary syringocystadenoma (8934006); Syringocystadenoma papilliferum (8934006); Nevus syringocystadenomatosus papilliferus (239121009); Syringocystadenoma papilliferum (239121009)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
Monarch Initiative: MONDO:0019392
Orphanet: ORPHA840

Definition

A rare non-malignant adnexal neoplasm that originates from the apocrine or eccrine sweat glands and is characterized histologically by cystic, papillary, and ductal invaginations into the dermis lined by double-layered outer cuboidal and luminal high columnar epithelium and connected to the epidermis. Dilated capillaries and a dense infiltrate of plasma cells are characteristic. Clinically, lesions are asymptomatic with a heterogeneous, non-distinctive appearance ranging from skin-colored to pink papules or plaques, occurring most commonly in the head and neck area. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSyringocystadenoma papilliferum
Follow this link to review classifications for Syringocystadenoma papilliferum in Orphanet.

Professional guidelines

Recent clinical studies

Etiology

Aggarwal D, Chatterjee D, Keshavamurthy V, Chhikara K, Saikia UN, Radotra BD, De D, Singh MP
Indian J Dermatol Venereol Leprol 2023 Jan-Mar;89(2):266-273. doi: 10.25259/IJDVL_845_20. PMID: 34623039
Konstantinova AM, Kyrpychova L, Nemcova J, Sedivcova M, Bisceglia M, Kutzner H, Zamecnik M, Sehnalkova E, Pavlovsky M, Zateckova K, Shvernik S, Spurkova Z, Michal M, Kerl K, Kazakov DV
Am J Dermatopathol 2019 Apr;41(4):281-285. doi: 10.1097/DAD.0000000000001285. PMID: 30398985
Friedman BJ, Sahu J, Solomides CC, Connolly DM, Lee JB
J Cutan Pathol 2018 Mar;45(3):212-216. Epub 2018 Jan 4 doi: 10.1111/cup.13093. PMID: 29206289
Karg E, Korom I, Varga E, Ban G, Turi S
Pediatr Dermatol 2008 Jan-Feb;25(1):132-3. doi: 10.1111/j.1525-1470.2007.00607.x. PMID: 18304179
Rammeh-Rommani S, Fezaa B, Chelbi E, Kammoun MR, Ben Jilani SB, Zermani R
Pathologica 2006 Jun;98(3):178-80. PMID: 17036946

Diagnosis

Hsieh MS, Bishop JA, Yu Fong Chang J
Surg Pathol Clin 2021 Mar;14(1):43-51. Epub 2021 Jan 5 doi: 10.1016/j.path.2020.09.006. PMID: 33526222
Tseng MC, Amin B, Barmettler A
Orbit 2018 Jun;37(3):171-174. Epub 2017 Oct 20 doi: 10.1080/01676830.2017.1383477. PMID: 29053036
Mundi JP, Rose AE, Boyd KP, Patel RR, Lipkin G
Dermatol Online J 2013 Dec 16;19(12):20722. PMID: 24365013
Ferringer T
Cutis 2011 Dec;88(6):268, 273-4. PMID: 22372162
Barbarino S, McCormick SA, Lauer SA, Milman T
Ophthalmic Plast Reconstr Surg 2009 May-Jun;25(3):185-8. doi: 10.1097/IOP.0b013e3181a39512. PMID: 19454927

Therapy

Pang S, Cevik J, Sreedharan S, Wilks DJ
Ann Plast Surg 2024 Jun 1;92(6):711-719. Epub 2024 May 9 doi: 10.1097/SAP.0000000000003879. PMID: 38725109
Alegría-Landa V, Jo-Velasco M, Santonja C, Eraña I, Vergara-Sanchez A, Kutzner H, Requena L
J Cutan Pathol 2020 Jan;47(1):12-16. Epub 2019 Sep 4 doi: 10.1111/cup.13572. PMID: 31449665
Cassarino DS, Su A, Robbins BA, Altree-Tacha D, Ra S
J Cutan Pathol 2017 Jun;44(6):544-547. Epub 2017 Apr 20 doi: 10.1111/cup.12939. PMID: 28343365
Watanabe Y, Shido K, Niihori T, Niizuma H, Katata Y, Iizuka C, Oba D, Moriya K, Saito-Nanjo Y, Onuma M, Rikiishi T, Sasahara Y, Watanabe M, Aiba S, Saito R, Sonoda Y, Tominaga T, Aoki Y, Kure S
Am J Med Genet A 2016 Jan;170A(1):189-94. Epub 2015 Sep 11 doi: 10.1002/ajmg.a.37376. PMID: 26360803

Prognosis

Zwierz A, Masna K, Burduk P
J Int Adv Otol 2022 Nov;18(6):544-547. doi: 10.5152/iao.2022.21594. PMID: 36349679Free PMC Article
Hsieh MS, Bishop JA, Yu Fong Chang J
Surg Pathol Clin 2021 Mar;14(1):43-51. Epub 2021 Jan 5 doi: 10.1016/j.path.2020.09.006. PMID: 33526222
Zhang Y, Kong YY, Cai X, Shen XX, Kong JC
J Cutan Pathol 2017 Jun;44(6):538-543. Epub 2017 Apr 17 doi: 10.1111/cup.12934. PMID: 28337777
Satter E, Grady D, Schlocker CT
Dermatol Online J 2014 Apr 16;20(4):22335. PMID: 24746298
Karg E, Korom I, Varga E, Ban G, Turi S
Pediatr Dermatol 2008 Jan-Feb;25(1):132-3. doi: 10.1111/j.1525-1470.2007.00607.x. PMID: 18304179

Clinical prediction guides

Zwierz A, Masna K, Burduk P
J Int Adv Otol 2022 Nov;18(6):544-547. doi: 10.5152/iao.2022.21594. PMID: 36349679Free PMC Article
Kazakov DV, Bisceglia M, Calonje E, Hantschke M, Kutzner H, Mentzel T, Michal M, Mukensnabl P, Spagnolo DV, Rütten A, Rose C, Urso C, Vazmitel M, Zelger B
Am J Dermatopathol 2007 Jun;29(3):256-63. doi: 10.1097/DAD.0b013e3180576fd9. PMID: 17519623
Rammeh-Rommani S, Fezaa B, Chelbi E, Kammoun MR, Ben Jilani SB, Zermani R
Pathologica 2006 Jun;98(3):178-80. PMID: 17036946
Yamamoto O, Doi Y, Hamada T, Hisaoka M, Sasaguri Y
Br J Dermatol 2002 Nov;147(5):936-45. doi: 10.1046/j.1365-2133.2002.05027.x. PMID: 12410704
Umbert P, Winkelmann RK
J Cutan Pathol 1976;3(2):75-87. doi: 10.1111/j.1600-0560.1976.tb00850.x. PMID: 993400

Recent systematic reviews

Wan L, Park A, Khachemoune A
Arch Dermatol Res 2024 Jun 21;316(7):421. doi: 10.1007/s00403-024-03176-w. PMID: 38904691
Pang S, Cevik J, Sreedharan S, Wilks DJ
Ann Plast Surg 2024 Jun 1;92(6):711-719. Epub 2024 May 9 doi: 10.1097/SAP.0000000000003879. PMID: 38725109

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