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Year : 2022  |  Volume : 67  |  Issue : 6  |  Page : 836
Ectopic syringocystadenoma papilliferum on the arm in a patient with noonan syndrome

Department of Dermatology, Fukushima Medical University, Fukushima, Japan

Date of Web Publication23-Feb-2023

Correspondence Address:
Nobuyuki Kikuchi
Department of Dermatology, Fukushima Medical University, Fukushima
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.ijd_437_21

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How to cite this article:
Kikuchi N, Yamamoto T. Ectopic syringocystadenoma papilliferum on the arm in a patient with noonan syndrome. Indian J Dermatol 2022;67:836

How to cite this URL:
Kikuchi N, Yamamoto T. Ectopic syringocystadenoma papilliferum on the arm in a patient with noonan syndrome. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 29];67:836. Available from:


Syringocystadenoma papilliferum (SCAP) is a benign adnexal neoplasm of apocrine histogenesis. SCAP most commonly occurs on the scalp, in association with an organoid nevus or as a solitary lesion. We present here a case of ectopic SCAP on the arm, without pre-existing nevus sebaceous.

A 31-year-old male with Noonan syndrome and tetralogy of Fallot was referred to our department, complaining of a solitary asymptomatic nodule which was recognized 6 months previously. He had a characteristic facial feature, diffuse alopecia, vision problem, and mental retardation. A physical examination showed a well-circumscribed, firm, sized 1 × 1 cm, reddish nodule with a keratotic surface on the flexor aspect of the left upper arm [Figure 1]a and [Figure 1]b. The tumor was totally removed under local anesthesia. Histopathological examination showed an exophytic tumor in the upper dermis, with cystic nests [Figure 1]c. The overlying epidermis showed irregular acanthosis with hyperkeratosis and in the center, tumor nests showed continuity with the epidermis [Figure 1]d. The tumor consisted of two rows of cells, with cuboidal cells, round nuclei, and scarce cytoplasm in the external row, and cylindrical cells with decapitation secretion in the internal row [Figure 1]e. Higher magnification revealed numerous papillary folds projected into the cystic spaces. There were a number of infiltrating mononuclear cells and plasma cells in the surrounding stroma. In addition, the proliferation of sweat glands was prominent in the mid-dermis [Figure 1]f. The result of immunohistochemistry showed that carcinoembryonic antigen (CEA) was positive in the inner layer of the ductal structure of the tumor nests, luminal layer cells reacted with epithelial membrane antigen (EMA), and the luminal columnar cells were strongly positive for CK7, and partially reactive for gross cystic disease fluid protein 15 (GCDFP-15) [Figure 2]. Based on those histopathological findings, a diagnosis of SCAP was made.
Figure 1: (a) A dome-shaped, red-brownish nodule with surface scales on the upper arm. (b) Close-up view. (c) Histologic view showing villous papillary invaginations in continuity with the epidermis and cystic cavities in the upper dermis. (d) Higher magnification showing glandular structures with dilated papillary vessels. (e) The luminal layers were lined by tall columnar cells that showed active decapitation secretion. (f) Proliferation of sweat gland structures beneath the tumor

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Figure 2: The result of immunohistochemistry showed that CEA was reactive for the inner layer cells of the ductal structure of the tumor nests (a). The luminal layers were reactive for EMA (b), and CK7 was strongly detected in the luminal columnar cells (c), whereas GCDFP-15 was partially positive (d)

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SCAP is known to occur usually on the scalp, and in unusual locations such as the trunk and extremities. To our knowledge, there have been only three reported cases of ectopic SCAP developed on the upper extremities.[1],[2],[3] The patients were female, and the age ranged between 10 and 59. Two cases showed multiple tumors in a linear fashion.[1],[3] By contrast, a solitary nodule occurred on the[2] upper arm in the present case. All of the previously reported cases did not have accompanying diseases. Noonan syndrome is an autosomal dominant disorder belonging to RASopathies, caused by gene mutations of the Ras/mitogen-activated protein (MAP) kinase pathway. Noonan syndrome is characterized by distinctive facial dysmorphism, short stature, congenital heart defects, as well as other anomalies including skin manifestations such as hair abnormalities and keratinizing disorders;[4] however, there are no reports of sweat gland neoplasms. Recent studies have suggested the loss of suppressor genes or mutations in the Ras-Raf-MAPK pathway in SCAP in either sebaceous nevus–associated or sporadic cases.[5] The similar RAS-MAPK signaling pathway alteration may be relevant to the development of SCAP in the present case, but further studies are necessary.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Rostan SE, Waller JD. Syringocystadenoma papilliferum in an unusual location. Arch Dermatol 1976;112:835-6.  Back to cited text no. 1
Mammino JJ, Vidmar DA. Syringocystadenoma papilliferum: Report of an unusual case. Int J Dermatol 1991;30:828.  Back to cited text no. 2
Gönül M, Soylu S, Gül U, Kaya I, Albayrak L, Unal T. Linear syringocystadenoma papilliferum of the arm: A rare localization of an uncommon tumour. Acta Derm Venereol 2008;88:528-9.  Back to cited text no. 3
Bessis D, Miquel J, Bourrat E, Chiaverini C, Morice-Picard F, Abadie C, et al. Dermatological manifestations in Noonan syndrome: A prospective multicentric study of 129 patients positive for mutation. Br J Dermatol 2019;180:1438-48.  Back to cited text no. 4
Shen AS, Peterhof E, Kind P, Rütten A, Zelger B, Landthaler M, et al. Activating mutations in the RAS/mitogen-activated protein kinase signaling pathway in sporadic trichoblastoma and syringocystadenoma papilliferum. Hum Pathol 2015;46:272-6.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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