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. 2019 Apr 26;11:2515841419844087. doi: 10.1177/2515841419844087

Syringocystadenocarcinoma papilliferum with orbital invasion: a case report with literature review

Carla Pagano Boza 1,, Joaquin Gonzalez-Barlatay 2, Shoaib Ugradar 3, Melina Pol 4, Eduardo Jorge Premoli 5
PMCID: PMC6487760  PMID: 31065626

Abstract

We present a case of Syringocystadenocarcinoma papilliferum that originated in the eyelid and extended into the orbit. These tumors are very rare and have the potential to metastasize. A literature review of all the previous cases has been compiled from the Medline, EMBASE, and PubMed databases. We found that the majority of cases present on the head and neck and up to 17% of cases showed metastatic progression. This is the first case to show orbital involvement and highlights the need to remain vigilant with such lesions, as they have a tendency to become aggressive.

Keywords: eyelid, orbit, Syringocystadenocarcinoma papilliferum

Introduction

Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant sudoriferous gland tumor that is related to its more common, benign counterpart, syringocystadenoma papilliferum (SCAP). Since the original description of SCAP in 1917,1 only 43 cases of SCACP have been described in the literature. To date, only one has appeared in the eyelid.2 SCACP is thought to develop from SCAP, nevus sebaceous, and linear nevus verrucosus lesions.3 However, due to the rarity of this tumor, little is known regarding its etiology and origin.3

In this study, we report the first case of SCACP with orbital involvement. Interestingly, it recurred following exenteration. An informed written consent was obtained from the patient for the publication of medical data and images.

Case report

A 63-year-old man presented with a lesion on the right upper eyelid that had been present for 7 years. The lesion was nodular, measuring 5.0 cm × 7.0 cm, ulcerated, indurated, and erythematous. It involved the lower eyelid (Figure 1). The patient had no light perception with the right eye and had a visual acuity of 20/20 on the left. Due to the presence of the tumor over the right eye, his intraocular pressure could not be measured, and it was found to be 18 mmHg on the left.

Figure 1.

Figure 1.

Lesion on presentation.

The left orbital examination did not reveal any abnormalities. A full examination of his local lymph nodes and lacrimal duct did not reveal any abnormalities. He explained that he did not have any previous therapy for this lesion. He was otherwise systemically well with no relevant family history. He did not have any history of trauma and informed us that he was a farmer by occupation.

A computed tomography (CT) scan of the orbit revealed right anterior orbital invasion with no bony or lacrimal gland involvement (Figure 2). A subsequent incisional biopsy revealed squamous cell invaginations extending from the epidermis into the dermis. The invaginations and papillary projections were lined with a bilayer epithelium: the luminal layer was composed of columnar cells with decapitation secretion and the outer layer was composed of small cuboidal cells. These cells had significant nuclear pleomorphism, prominent nucleoli, and increased mitotic activity (Figure 3). Immunohistochemical staining demonstrated positivity for epithelial membrane antigen (EMA), Cytokeratin 8/18, and a Cytokeratin cocktail of high and low density (Figure 3). It was negative for GCDFP-15 (protein 15 of the fibrocystic disease of the breast), which excluded a lesion of breast origin and carcinoembryonic antigen (CEA). The diagnosis of SCACP was therefore confirmed. A positron emission tomography (PET) scan did not reveal any metastatic spread.

Figure 2.

Figure 2.

CT imaging of the lesion at presentation.

Figure 3.

Figure 3.

Hematoxylin and eosin staining (H&E): (a) the transition between squamous and glandular epithelium (100×). (b) Large areas of superficial epithelium were sphacelated. Glandular invaginations showed a characteristic funnel shape. Papillary structures can be identified inside a dermal cyst (100×). (c) The papillary structures are lined with a stratified atypical epithelium. Micropapillae and secretion by decapitation can be seen (100×). (d) At high power magnification, atypical nuclei are evident. Large atypical nuclear shapes are seen and increased mitotic activity is observed (*).

The patient was treated with exenteration of the right orbit to remove the tumor. After 11 months of follow-up, we noted local recurrence of the original tumor (confirmed with biopsy) in the anophthalmic orbit. There was no associated lymph node enlargement on examination, though the patient refused any further imaging. Radical exenteration with adjuvant radiotherapy has been planned for the patient.

Discussion

SCACP is an extremely rare adnexal neoplasm of the sweat glands and has only been documented 43 times in the literature. It is believed to arise from a malignant transformation of SCAP lesions.4 Clinically, it may present as an asymptomatic long-standing lesion, which may be flat or nodular, cystic, or ulcerated. We performed a literature review of the Medline, EMBASE, and Cochrane databases to characterize the cases previously listed in the literature (Table 1).

Table 1.

Previous case reports on SCACP.

Reference Age Sex Location Size (mm) Duration Diagnosis Association Follow-up Treatment
Dissanayake and Salm5 74 F Scalp 65 30 years SCACP in situ SCAP NED (6.75 years) Surgery
71 F Back 30 N/A SCACP invasive N/A NED (7 years) Surgery
Seco Navedo and colleagues6 50 F Scalp 65 Congenital SCACP invasive Nevus sebaceous 3 Local lymph node, lymph node metastasis Surgery + Rt + Ct (NED—2 years)
Numata and colleagues7 52 F Chest 130 × 80 20 years SCACP invasive N/A 1 Local lymph node, lymph node metastasis Surgery NED (12 months)
Bondi and Urso8 47 M Scalp 25 N/A SCACP invasive N/A N/A Surgery
Ishida-Yamamoto and colleagues9 61 M Perianal 60 10 years SCACP in situ N/A NED (11 months) Surgery
Arai and colleagues10 64 M Scalp 35 2 years SCACP in situ SCAP N/A Surgery
Chi and colleagues11 60 M Auricle 40 × 10 Since childhood SCACP invasive SCAP NED (72 months) Surgery
Woestenborghs and colleagues12 81 F Scalp 15 N/A SCACP in situ SCAP N/A Surgery
Park and colleagues13 65 M Suprapubic region 35 2 years SCACP in situ N/A NED (24 months) Surgery
Langner and Ott14 83 M Perianal 15 N/A SCACP in situ SCAP N/A Surgery
Sroa and colleagues15 77 M Calf 25 9 years SCACP invasive N/A NED (15 months) Surgery
Kazakov and colleagues16 56 F Neck 20 10 years SCACP in situ SCAP NED (9 months) Surgery
58 M Forehead 25 25 years SCACP invasive SCAP NED (4 years) Surgery
46 F Scalp 35 N/A SCACP invasive SCAP NED (6 years) Surgery
67 M Scalp 25 N/A SCACP in situ SCAP NED (2 years) Surgery
60 F Scalp 30 >30 years SCACP invasive SCAP N/A Surgery
81 M Scalp 20 N/A SCACP invasive SCAP NED (21 months) Surgery
Leeborg and colleagues17 86 F Neck 45 4 months SCACP invasive Invasive squamous cell carcinoma Local recurrence (18 months) Surgery + Rt
Abrari and Mukherjee18 62 M Axilla 35 6 months SCACP invasive N/A N/A Surgery
Aydin and colleagues19 67 M Scalp 40 Since childhood SCACP invasive SCAP NED (2 years) Surgery
Hoekzema and colleagues3 83 F Arm 30 7 years SCACP invasive SCAP nevus verrucosus N/A Surgery
Hoguet and colleagues20 86 M Eyelid 4 N/A SCACP invasive N/A NED (3 months) Surgery
Plant and colleagues21 83 M Penis 12 N/A SCACP in situ N/A N/A Surgery
Bakhshi and colleagues22 45 F Scalp 60 × 30 12 months N/A SCAP NED (12 months) Surgery in situ
Zhang and colleagues23 75 F Arm 15 12 months SCACP invasive SCAP NED (6 months) Surgery
Peterson and colleagues24 65 M Scalp 30x30 12 months SCACP invasive SCAP NED Surgery
Arslan and colleagues2 66 M Scalp N/A 20 years SCACP invasive SCAP 3 Local lymph node, lymph node metastasis Surgery + Rt (NED—15 months)
66 F Scalp 30 >12 months SCACP invasive N/A NED (2 years) Surgery
Castillo and colleagues25 32 F Scalp 22 N/A SCACP in situ N/A Local recurrence (8 years) Surgery
Paradiso and colleagues26 88 M Shoulder 15 × 15 N/A SCACP invasive N/A Died from other cause N/A
Shan and colleagues27 93 M Popliteal fossa 20 >10 years N/A SCAP NED Surgery
Mohanty and colleagues28 80 F Scalp 50 8 years SCACP in situ N/A NED (5 years) Surgery
Satter and colleagues29 42 M Scalp 45 × 40 >1 month SCACP invasive SCAP and Nevus sebaceous Lymph node metastasis Surgery
Parekh and colleagues4 74 M Scalp 20 Since childhood SCACP invasive SCAP, nevus sebaceous of Jadassohn, trichoblastoma Lymph node metastasis Surgery
Chen and colleagues30 60 F Scalp 28 × 20 12 months SCACP in situ Nevus sebaceous N/A Surgery
Singh and colleagues31 60 F Back 15 × 10 >10 years SCACP in situ SCACP in situ with macular amyloidosis N/A Surgery
Zhang and colleagues32 26 M Chest 50 22 years SCACP in situ Invasive adenocarcinoma subcutis Left axillary lymph node and bilateral lung metastases, DOD 2 months after diagnosis Surgery + Ct
47 M Abdomen 15 23 years SCACP in situ N/A NED (9 years) Surgery
67 M Left Axilla 20 6 years SCACP in situ Invasive adenocarcinoma subcutis N/A Surgery + left axilla lymphadenectomy
64 M Scalp 20 1 years SCACP in situ Invasive adenocarcinoma in dermis + mucinous metaplasia Metastases to multiple distant lymph nodes and lung metastases, DOD 34 months after diagnosis Surgery + Rt
63 M Chest 10 10 years SCACP in situ Invasive adenocarcinoma in dermis NED (36 months) Surgery
74 M Chest 20 6 years SCACP in situ Invasive adenocarcinoma subcutis NED (30 months) Surgery
63 F Axilla 50 3 months SCACP in situ Invasive adenocarcinoma + invasive squamous cell carcinoma Widespread subcutaneous metastases, DOD 20 months after diagnosis Surgery + right axilla lymphadenectomy
40 M Chest 50 5 years SCACP in situ Invasive adenocarcinoma subcutis NED (14 months) Surgery + bilateral lymphadenectomy + Ct
29 F Forehead 15 2 years SCACP in situ Invasive squamous cell carcinoma NED (10 months) Surgery
64 M Axilla 22 10 years SCACP in situ Invasive adenocarcinoma subcutis NED (3 months) Surgery + right axilla lymphadenectomy + Ct
Present case 63 M Eyelid 50 × 70 >6 years SCACP invasive SCAP Local recurrence Surgery

Ct, chemotherapy; Rt, radiation therapy; N/A, not available; NED, no evidence of disease; DOD, died of disease.

The tumor appears to affect middle-aged or elderly individuals15 and does not seem to have a gender bias. The most frequent location is the head and neck (53%), with only one case in the eyelid. Other locations where these lesions occur frequently are the back, chest, suprapubic, and perianal regions.

Treatment is based on a complete tumor resection with oncological margins, which is essential for a better prognosis. Mohs surgery has also been successfully used for this purpose.11 Sentinel lymph node biopsy may be feasible in some cases when there is suspicion of lymph spread, although lymphatic spread has been shown to be rare with this tumor (6 of the 42 documented cases; Table 1). Radiotherapy and chemotherapy have also been used rarely, but the experience with these treatments is scarce due to the rarity of the lesion.25

SCACP characteristically presents with squamous cell invaginations extending from the epidermis into the dermis. The invaginations and papillary projections are lined by two-layer epithelium: the luminal layer composed of columnar cells with decapitation secretions and the outer layer composed of small cuboidal cells. The immunohistochemical features of SCACP are still under study, but the most frequently reported markers are CEA,15,20,28 followed by EMA,9,28 GDFP-15,20,28,32 and cytokeratin.11,28,32

Due to its appearance, the differential diagnosis includes other skin tumors such as basal cell carcinoma, squamous cell carcinoma, sebaceous carcinoma, metastatic breast or gastrointestinal adenocarcinomas, and other sweat gland neoplasms.2,20

Of the cases that reported head and neck involvement, 16 (72.72%) were in remission following therapy, 2 (9.09%) had local recurrence, 3 (13.63%) had regional lymphatic invasion, and 1 (4.54%) had distant metastases. Of the reports describing involvement of the thorax, abdomen, and pelvis, 17 (85%) went into remission following therapy, none had local recurrence, 1 (5%) had regional lymphatic invasion and 2 (10%) had distant metastases.

This is the first reported case of SCACP with extension into the anterior orbit. While SCACP is an exceedingly rare tumor, we found that of the reported cases, 16% showed signs of metastasis. It is therefore an important diagnosis to consider when reviewing skin lesions around the orbit. It also encourages us to monitor patients with SCAP more closely as our literature review suggests that SCACP may be more aggressive than previously considered.

Footnotes

Funding: The authors received no financial support for the research, authorship, and/or publication of this article.

Conflict of interest statement: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

ORCID iD: Shoaib Ugradar Inline graphic https://orcid.org/0000-0003-4479-3033

Contributor Information

Carla Pagano Boza, Oculoplastics Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

Joaquin Gonzalez-Barlatay, Oculoplastics Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

Shoaib Ugradar, Division of Orbital and Ophthalmic Plastic Surgery, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA, USA.

Melina Pol, Pathology Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

Eduardo Jorge Premoli, Oculoplastics Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

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