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Syringocystadenoma papilliferum

SCAP


Presentation

  • A major review of the literature of syringocystadenoma papilliferum's (SCAP's) presentation and management is presented. In addition, a case report of this unique diagnosis presenting as a corneal abrasion in a 66-year-old-male is included.[ncbi.nlm.nih.gov]
Soft Tissue Mass
  • A gross examination showed two gray brown hairy skin covered soft tissue masses altogether measuring 3 cm 3 cm, with areas of hemorrhage.[jscisociety.com]
  • High resolution computed tomography showed an approximately 0.8 0.6 cm soft tissue mass occupying within the left external ear canal near the opnening, without eroding canal wall.[headandneckoncology.biomedcentral.com]
Accelerated Growth
  • The lesion has been gradually increasing in size showing a recent accelerated growth. Excisional biopsy demonstrated the histological picture of syringocystadenoma papilliferum (SCAP).[ncbi.nlm.nih.gov]
Weight Loss
  • There was no history of itching, fever, weight loss, or other constitutional symptoms. Physical examination revealed a solitary, well circumscribed swelling of 1 cm diameter. The swelling was firm in consistency, mobile and nontender.[jcytol.org]
Constitutional Symptom
  • There was no history of itching, fever, weight loss, or other constitutional symptoms. Physical examination revealed a solitary, well circumscribed swelling of 1 cm diameter. The swelling was firm in consistency, mobile and nontender.[jcytol.org]
Diplopia
  • No history of diplopia was there. Systemic history was unremarkable. On examination, best-corrected visual acuity was 20/20 and 20/120 for oculus dexter and oculus sinister, respectively.[meajo.org]
Irritability
  • Scratching to relieve itching causes irritation and bleeding. The ulcers that form in such irritated lesions tend to crust and breakdown repeatedly as occurred in our case.[ijo.in]
  • During adolescence the lesion may become raised with verrucous or papillomatous surface. [2] Usually it is asymptomatic but patients having lesions over the scalp may complain of mild irritation or bleeding while combing the hair or may be tender to even[ijdvl.com]
  • Examination revealed a noduloulcerative growth. measuring 6 cm 3.5 cm 2.5 cm over the left deltoid region; it bled on manipulation surrounded by small warty lesions and partly excoriated skin due to continued irritation [Figure 1] .[ijdpdd.com]

Workup

  • In such cases, historic features must be combined with complete systemic workup to exclude primary extra cutaneous tumors. Seven such tumors were present in our series.[jpma.org.pk]
Lymphocytic Infiltrate
  • Microscopically, it showed a hyperkeratotic epidermis with foci of papillomatosis, focal absence of hair follicles, focal primitive hair germ-like proliferations with patchy perivascular lymphocytic infiltrate.[ncbi.nlm.nih.gov]

Treatment

  • Treatment is complete excision of the lesion and this has a low recurrence rate. While predominantly benign, there have been cases of basal cell carcinoma development or other malignant transformations.[ncbi.nlm.nih.gov]
  • Management and treatment Treatment involves complete surgical excision. Carbon dioxide laser treatment can be useful for lesions in anatomic areas not favorable for surgery.[orpha.net]
  • The treatment of Syringocystadenoma Papilliferum may involve the following: The treatment is a complete surgical excision, which can result in a cure.[dovemed.com]

Prognosis

  • Regardless of the type, the histopathology of the lesion is usually the same and renders the same prognosis.[ncbi.nlm.nih.gov]

Etiology

  • Etiology The etiology of SCAP has not been elucidated. It is not known whether SCAP derives from eccrine or apocrine glands. The role of pluripotent stem cells has also been suggested.[orpha.net]
  • TERMINOLOGY Abbreviations Syringocystadenoma papilliferum (SCP) Definitions Benign adnexal tumor with endophytic/exophytic growth pattern ETIOLOGY/PATHOGENESIS Cytogenetics Subset of tumors have loss of heterozygosity for PTCH &/or P16, suggesting a role[basicmedicalkey.com]
  • (Etiology) The cause of development of Syringocystadenoma Papilliferum is unknown Genetic mutations have been suspected by researchers in some cases Some researchers believe that these tumors arise from the eccrine or apocrine sweat gland What are the[dovemed.com]
  • Available from: Syringocystadenoma papilliferum (SCAP) is a hamartomatous adnexal tumor with unknown etiology. [1] It is classified as a benign tumor of apocrine differentiation. [2] It either develops independently or is commonly seen in association[jcytol.org]

Epidemiology

  • Her main area of interest is cancer epidemiology, with a particular focus on melanoma and other skin cancers. Nagwa M. Elwan is Professor of Dermatology at the Faculty of Medicine, Tanta University, Egypt.[books.google.es]
  • Benign adnexal tumor with endophytic/exophytic growth pattern ETIOLOGY/PATHOGENESIS Cytogenetics Subset of tumors have loss of heterozygosity for PTCH &/or P16, suggesting a role for loss of these tumor suppressor genes in some cases CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Summary Epidemiology The prevalence of SCAP is not known. About 300 cases have been reported to date. The condition has been reported in various ethnic groups.[orpha.net]
  • Epidemiology of benign eyelid lesions in patients presenting to a teaching hospital. Saudi J Ophthalmol 2012;26:211-6. 6. Rook A, Wilkinson DS, Ebling FJ. Textbook of Dermatology. Pondicherry: Blackwell; 1986. p. 177. 7. Helwig EB, Hackney VC.[ijo.in]
  • Material Type: Journal Article Impact Factor: 2,227 Lingua: Inglese Ricevuto in biblioteca: 01/12/2008 Gallus S, Naldi L; Oncology Study Group of the Italian Group for Epidemiologic Research in Dermatology.[asmn.netribe.it]
Sex distribution
Age distribution

Pathophysiology

  • […] tumors, such as ceruminous adenoma, ceruminous pleomorphic adenoma, and ceruminous syringocystadenoma papilliferum, as well as malignant tumors, such as ceruminous adenocarcinoma, adenoid cystic carcinoma, and, mucoepidermoid carcinoma. [4, 5, 6, 7] Pathophysiology[emedicine.medscape.com]

Prevention

  • Natural history of SCAP is yet to be determined, thus prevention is not yet an option. Excision of the lesion is the mainstay of treatment, but wide margins are not necessary. Financial support and sponsorship Nil.[ncbi.nlm.nih.gov]

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