Skip to main content
Urology Case Reports logoLink to Urology Case Reports
. 2021 Nov 9;40:101934. doi: 10.1016/j.eucr.2021.101934

Genital syringocystadenocarcinoma papilliferum: An unusual location and review of the literature

Mohammad Shahidi Dadras a,1, Moein Baghani a,1, Azadeh Rakhshan b, Anahita Ansari Djafari c, Fahimeh Abdollahimajd a,d,
PMCID: PMC8599099  PMID: 34820287

Abstract

Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm of the sweat glands. It is believed to arise from the malignant transformation of syringocystadenoma papilliferum (SCAP). The majority of cases present on the head and neck and up to 17% of cases show metastatic progression. These tumors seldom occur in the anogenital area and, to date, only one case has been reported on the penis. Here, we report a rare case of SCACP in a 65-year-old man who presented with an erythematous, non-healing, ulcerated lesion on the penis.

Keywords: Syringocystadenocarcinoma papilliferum, Adnexal neoplasm, Anogenital area

Abbreviations: FDG-PET, fluorodeoxyglucose-positron emission tomography; SCACP, Syringocystadenocarcinoma papilliferum

Highlights

  • Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm of the sweat glands.

  • The majority of SCACP cases present on the head and neck.

  • SCACP seldom occur in the anogenital area and, to date, only one case has been reported on the penis.

  • The present case is the second case of SCACP with involvement of the penis, the first involving the scrotum.

  • Clinicians should be aware of the possibility of SCACP in cases with chronic ulcerative nodular lesions in the genital area.

1. Introduction

Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant adnexal tumor that represents the malignant counterpart of syringocystadenoma papilliferum (SCAP).1 Mostly, it is believed to arise from SCAP, nevus sebaceous, or linear nevus verrucosus lesions.2 The majority of cases present on the head and neck and up to 17% of cases show metastatic progression.1 Only 52 cases of this malignancy have been reported to date, with limited data being available about its origin and etiology. Moreover, these tumors seldom occur in the anogenital area; to date, only one case has been reported to appear on the penis.2, 3, 4 Here, we report a case of SCACP in the anogenital area with penile involvement.

2. Case presentation

A 65-year-old Iranian man presented to the dermatology clinic with multiple papules, nodules, and exudative crust over the genitalia and an erythematous, non-healing, ulcerated lesion on the penis measuring 1 × 2 cm that had appeared two months earlier (Fig. 1). The patient complained of moderate irritating and obstructive urologic symptoms and had unintentionally lost approximately 10 kg of weight during the preceding 6 months. Severe lymphedema and subcutaneous edema were apparent in the scrotum. Skin biopsies were obtained from the skin ulcer and the adjacent papule. Sonography was performed, revealing irregularity in the bladder wall; the prostate volume was 45 ml, with bulging of 10 ml of the median lobe into the base of the bladder. No free fluid was detected in the abdominopelvic cavity. Testicular atrophy was prominent. The scrotal wall was thickened to about 19 mm with increased fat echogenicity in favor of soft tissue inflammation and edema. Bilateral complicated hydrocele was detected. Vascular flow was normal. Cystourethroscopy was performed under spinal anesthesia in the lithotomy position. There were some cotton-like, irregular, epithelial lesions in the distal part of the penile urethra, 2 cm proximal to the fossa navicularis; a cold cup biopsy was taken under vision. Other parts of the urethra had normal appearance and there was a prominent, enlarged prostate with kissing lobes. There was no visible abnormality in the bladder except for moderate trabeculation.

Fig. 1.

Fig. 1

Multiple papules, nodules, and exudative crust over the genitalia and an erythematous, non-healing, ulcerated lesion on the penis along with severe scrotal edema (a, b).

Histopathologic examination revealed hyperplastic epidermis and a crateriform lesion populated by papillary projections and lined by atypical epithelium, with the fibrovascular cores containing numerous plasma cells. Dermal invasion was seen, characterized by tubular structures, single cells, and small groups of cells that infiltrated the full thickness of the dermis and part of the subcutis. These structures were surrounded by desmoplastic stroma with lymphoplasmacytic infiltration and perineural invasion. Mild neutrophilic infiltration in dermal nests was identified along with isolated tumor cell necrosis. Overall, the manifestations were characterized as a malignant epithelial neoplasm with papillary and tubular structures in favor of SCACP (Fig. 2). Histologic evaluation of the urethral lesion was also consistent with adenocarcinoma. No distant metastasis was identified via a fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan.

Fig. 2.

Fig. 2

Hyperplastic epidermis and a crateriform lesion populated by papillary projections and lined by atypical epithelium, with the fibrovascular cores containing numerous plasma cells (a, H&E × 100; b, H&E × 400) (a, b). Dermal invasion was seen, characterized by tubular structures, single cells, and small groups of cells that infiltrated the full thickness of the dermis and part of the subcutis (c, H&E × 100; d, H&E × 200) (c, d).

3. Discussion

As an adnexal tumor, SCAP was first described by Stokes in 1917.1 Dissanayake and Salm, in 1980, were the first to discuss SCACP, which is considered as the malignant counterpart of SCAP.5 Since then, this is just the 53rd reported case of this malignancy, meaning that the clinical and pathological characteristics are not well defined.4 We performed a literature review of the Medline, EMBASE, and Cochrane databases to characterize the cases previously listed in the literature (Table 1). Our results show that SCACP predominantly involves the head and neck of patients over 60 years of age with no gender predilection, but can also occur on the chest, arm, anogenital region, and back (Table 1).

Table 1.

Reported cases of syringocystadenocarcinoma papilliferum.

# Reference Clinical presentation Sex/Age (year) Location Size (mm) Duration Diagnosis Association Follow-up Treatment
1 Dissanayake and Salm, 1980 Exophytic tumor with copious secretion F/74 Scalp 65 30 years SCACP in situ SCAP NED (6.75 years) Surgery
2 Dissanayake and Salm, 1980 A lump F/71 Back 30 N/A SCACP invasive N/A NED (7 years) Surgery
3 Seco Navedo et al., 1982 Tumor F/50 Scalp 65 Congenital SCACP invasive Nevus sebaceous 3 Local lymph node, lymph node metastasis Surgery + RTx + CTx (NED—2 years)
4 Numata et al., 1985 Nodular, partially cystic and solid tumor F/52 Chest 130 × 80 20 years SCACP invasive N/A 1 Local lymph node, lymph node metastasis Surgery NED (12 months)
5 Bondi and Urso, 1996 Ulcerated and crusted lesion. M/47 Scalp 25 N/A SCACP invasive N/A N/A Surgery
6 Ishida-Yamamoto et al., 2001 Enlarging nodule as a black exophytic tumor with a granular surface M/61 Perianal 60 10 years SCACP in situ N/A NED (11 months) Surgery
7 Arai et al., 2003 Enlarging tumor, bleed easily when pressed, surrounded by a bloody crust with macerated white papules on the surface M/64 Scalp 35 2 years SCACP in situ SCAP N/A Surgery
8 Chi et al., 2004 Two ulcerated verrucous plaques coated with yellow crust, painful, pruritic M/60 Auricle 40 × 10 Since childhood SCACP invasive SCAP NED (72 months) Surgery
9 Woestenborghs et al., 2006 A bleeding raised tumor F/81 Scalp 15 N/A SCACP in situ SCAP N/A Surgery
10 Park et al., 2007 Single erythematous dome-shaped and firm nodule surrounded by bloody crust M/65 Suprapubic region 35 2 years SCACP in situ N/A NED (24 months) Surgery
11 Langner and Ott, 2009 A nodule with partly cystic and partly solid appearance M/83 Perianal 15 N/A SCACP in situ SCAP N/A Surgery
12 Sroa et al., 2010 Flesh-colored exophytic nodule with a peripheral crusted hyperpigmented border M/77 Calf 25 9 years SCACP invasive N/A NED (15 months) Surgery
13 Kazakov et al., 2010 Verrucous ulcerated nodule F/56 Neck 20 10 years SCACP in situ SCAP NED (9 months) Surgery
14 Kazakov et al., 2010 Clinical impression of a ruptured cyst or carcinoma M/58 Forehead 25 25 years SCACP invasive SCAP NED (4 years) Surgery
15 Kazakov et al., 2010 Ulcerated smelling neoplasm F/46 Scalp 35 N/A SCACP invasive SCAP NED (6 years) Surgery
16 Kazakov et al., 2010 Ulcerated nodule M/67 Scalp 25 N/A SCACP in situ SCAP NED (2 years) Surgery
17 Kazakov et al., 2010 Ulcerated tumor with Recent rapid growth. F/60 Scalp 30 >30 years SCACP invasive SCAP N/A Surgery
18 Kazakov et al., 2010 Inflammatory plaque M/81 Scalp 20 N/A SCACP invasive SCAP NED (21 months) Surgery
19 Leeborg et al., 2010 Erythematous to violaceous asymmetric papule with lobulated contours F/86 Neck 45 4 months SCACP invasive Invasive squamous cell carcinoma Local recurrence (18 months) Surgery + RTx
20 Abrari and Mukherjee, 2011 As described by the patient, a bead like, 1.0 cm axillary swelling, had been self-discovered and this lesion had excoriated and attained a size of 3.5 cm in 6 months. M/62 Axilla 35 6 months SCACP invasive N/A N/A Surgery
21 Aydin et al., 2011 Ulcerative nodular lesion M/67 Scalp 40 Since childhood SCACP invasive SCAP NED (2 years) Surgery
22 Hoekzema et al., 2011 Exophytic nodule with a moist surface F/83 Arm 30 7 years SCACP invasive SCAP nevus verrucosus N/A Surgery
23 Hoguet et al., 2012 Erythematous, slightly elevated, centrally ulcerated and crusted nodule M/86 Eyelid 4 N/A SCACP invasive N/A NED (3 months) Surgery
24 Plant et al., 2012 A non-healing ulcerated lesion M/83 Penis 12 N/A SCACP in situ N/A N/A Surgery
25 Bakhshi et al., 2012 Hemispherical swelling was seen on the scalp which was firm in consistency with a granular surface and erosions and crustations F/45 Scalp 60 × 30 12 months N/A SCAP NED (12 months) Surgery
26 Zhang et al., 2012 Solitary tender erythematous ulcerated nodule within a background of red patch F/75 Arm 15 12 months SCACP invasive SCAP NED (6 months) Surgery
27 Peterson et al., 2013 Hairless flesh-colored exophytic tumor with serosanguinous exudate M/65 Scalp 30 × 30 12 months SCACP invasive SCAP NED Surgery
28 Arslan et al., 2013 Multinodular ulcerated lesions M/66 Scalp N/A 20 years SCACP invasive SCAP 3 Local lymph node, lymph node metastasis Surgery + RTx (NED—15 months)
29 Arslan et al., 2013 Well-defined erythematous ulcerated nodule F/66 Scalp 30 >12 months SCACP invasive N/A NED (2 years) Surgery
30 Castillo et al., 2014 Well-delimited oval dermal nontender nodule with a solid and cystic appearance. F/32 Scalp 22 N/A SCACP in situ N/A Local recurrence (8 years) Surgery
31 Paradiso et al., 2014 A single painful erythematous, dome-shaped, and firm nodule surrounded by normal skin M/88 Shoulder 15 × 15 N/A SCACP invasive N/A Died from other cause N/A
32 Shan et al., 2014 A pink ulcerated nodule without tenderness nor pruritis M/93 Popliteal fossa 20 >10 years N/A SCAP NED Surgery
33 Mohanty et al., 2014 Exophytic lobulated mass which was tan-pink to red in color with soft to firm consistency and was non-tender. F/80 Scalp 50 8 years SCACP in situ N/A NED (5 years) Surgery
34 Satter et al., 2014 Focally ulcerated exophytic nodule associated with a few small satellite papules which bled with minor trauma M/42 Scalp 45 × 40 >1 month SCACP invasive SCAP and Nevus sebaceous Lymph node metastasis Surgery
35 Parekh et al., 2016 A single well-demarcated red exophytic nodule with small foci of ulceration M/74 Scalp 20 Since childhood SCACP invasive SCAP, nevus sebaceous of Jadassohn, trichoblastoma Lymph node metastasis Surgery
36 Chen et al., 2016 A hairless, rough, ill-defined erythematous erosive warty plaque on the right parietal scalp with serosanguinous exudate F/60 Scalp 28 × 20 12 months SCACP in situ Nevus sebaceous N/A Surgery
37 Singh et al., 2017 Well-defined erythematous plaque with overlying ulceration on the midback, 1 cm left of the spine. Adjoining skin showed a horseshoe-shaped oval area of lichenification F/60 Back 15 × 10 >10 years SCACP in situ SCACP in situ with macular amyloidosis N/A Surgery
38 Zhang et al., 2017 Solitary red plaque M/26 Chest 50 22 years SCACP in situ Invasive adenocarcinoma subcutis Left axillary lymph node and bilateral lung metastases, DoD 2 months after diagnosis Surgery + CTx
39 Zhang et al., 2017 Solitary nodule with exudate M/47 Abdomen 15 23 years SCACP in situ N/A NED (9 years) Surgery
40 Zhang et al., 2017 Erythematous nodule M/67 Left Axilla 20 6 years SCACP in situ Invasive adenocarcinoma subcutis N/A Surgery + left axilla lymphadenectomy
41 Zhang et al., 2017 Flat verrucous neoplasm M/64 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 + RTx
42 Zhang et al., 2017 Exophytic ulcerated nodule with bleeding M/63 Chest 10 10 years SCACP in situ Invasive adenocarcinoma in dermis NED (36 months) Surgery
43 Zhang et al., 2017 Exophytic pinkish nodule M/74 Chest 20 6 years SCACP in situ Invasive adenocarcinoma subcutis NED (30 months) Surgery
44 Zhang et al., 2017 Flat mass with granular surface F/63 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
45 Zhang et al., 2017 Keloid plaque M/40 Chest 50 5 years SCACP in situ Invasive adenocarcinoma subcutis NED (14 months) Surgery + bilateral lymphadenectomy + CTx
46 Zhang et al., 2017 Subcutaneous nodule F/29 Forehead 15 2 years SCACP in situ Invasive squamous cell carcinoma NED (10 months) Surgery
47 Zhang et al., 2017 Verrucous ulcerated mass M/64 Axilla 22 10 years SCACP in situ Invasive adenocarcinoma subcutis NED (3 months) Surgery + right axilla lymphadenectomy + CTx
48 Muthusamy et al., 2017 Ulcerated nodule with mild pain F/78 Scalp 45 × 35 6 months SCACP invasive SCAP and Trichoblastoma N/A N/A
49* Yao et al., 2018 Erythematous friable mass which bleed intermittently F/73 Scalp 30 × 27 Since birth N/A N/A N/A N/A
50 Agulló Pérez et al., 2018 Pediculated erythematous-brownish papule which occasional bleeding caused by friction and exudative crust M/40 Chest 10 1 year SCACP invasive N/A NED (8 months) Surgery
51 Pagano Boza et al., 2019 Ulcerated, indurated, and erythematous nodule M/63 Eyelid 50 × 70 >6 years SCACP invasive SCAP Local recurrence Surgery
52 Alegría-Landa et al., 2019 Eroded easily-bleeding nodule F/90 Scalp N/A 10 months SCACP in situ with sarcomatoid appearance N/A Died from other cause 1 year after diagnosis N/A
53 Present case Non-healing erythematous ulcerated lesion, multiple papules and nodules, crustations and hyperpigmented patches. Scrotal Tense edema. M/65 Genitalia 10× 20 2 months SCACP invasive Invasive Adenocarcinoma Died from other cause 7 months after diagnosis CTx

Abbreviations: CTx, chemotherapy; DoD, died of disease; F, Female; M, Male; N/A, not available; NED, no evidence of disease; RTx, radiation therapy.

*Full text was not accessible online

These lesions clinically present as erythematous, skin-colored, brown or yellowish nodules, papules, and/or ulcerated lesions that may be associated with mild pain, pruritus, or easily induced bleedings (Table 1). In the present case, the initial differential diagnoses were angiosarcoma, Kaposi sarcoma, and squamous cell carcinoma; however, the histopathology findings were consistent with SCACP.

Currently, no treatment protocols for SCACP are available but surgery with wide margins is the most favorable option and Mohs surgery has been suggested.1,4 The application of chemotherapy and radiotherapy is controversial.4 Our therapeutic plan consisted of chemotherapy. Unfortunately, despite receiving five courses of chemotherapy, the patient died seven months after the diagnosis of SCACP.

There have been only eight cases reported with metastasis (Table 1); our patient did not show any signs of metastasis in the PET-FDG scan. On the other hand, there have been limited cases of SCACP associated with invasive adenocarcinoma (Table 1), as was the case for our patient.

To the best of our knowledge, the present case is the second case of SCACP with involvement of the penis, the first involving the scrotum, and one of the limited cases associated with invasive behavior of SCACP.

In conclusion, regarding the variable course of SCACP, in case of encountering chronic nodular and ulcerative lesions in the genital area, despite the rarity, clinicians should be aware of its possibility.

Ethics approval and consent to participate

Ethical approval was not required for this study in accordance with national guidelines.

Consent for publication

Written informed consent was obtained from the patient's family for publication of this case report and any accompanying images.

Declaration of competing interest

The authors have no conflicts of interest to declare.

Funding sources

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contributions

MSD, FA and AAِ were involved in the diagnosis and management of the patients and have been responsible for the clinical part of the manuscript. AR reported the result of histopathological evaluation. FA and MB did literature review and drafted the manuscript. MSD, FA, AR and AA were responsible for final editing of the manuscript, and coordinated the study. All authors have read and approved the final manuscript.

Availability of data and material

Not applicable.

References

  • 1.Pagano Boza C., Gonzalez-Barlatay J., Ugradar S., Pol M., Premoli E.J. Syringocystadenocarcinoma papilliferum with orbital invasion: a case report with literature review. Ther Adv Ophthalmol. 2019;11 doi: 10.1177/2515841419844087. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 2.Hoekzema R., Leenarts M.F., Nijhuis E.W. Syringocystadenocarcinoma papilliferum in a linear nevus verrucosus. J Cutan Pathol. 2011;38(2):246–250. doi: 10.1111/j.1600-0560.2009.01419.x. [DOI] [PubMed] [Google Scholar]
  • 3.Plant M.A., Sade S., Hong C., Ghazarian D.M. Syringocystadenocarcinoma papilliferum in situ of the penis. Eur J Dermatol. 2012;22(3):405–406. doi: 10.1684/ejd.2012.1723. [DOI] [PubMed] [Google Scholar]
  • 4.Arslan H., Diyarbakrl M., Batur S., Demirkesen C. Syringocystadenocarcinoma papilliferum with squamous cell carcinoma differentiation and with locoregional metastasis. J Craniofac Surg. 2013;24(1):e38–e40. doi: 10.1097/SCS.0b013e31826cffc6. [DOI] [PubMed] [Google Scholar]
  • 5.Dissanayake R., Salm R. Sweat‐gland carcinomas: prognosis related to histological type. Histopathology. 1980;4(4):445–466. doi: 10.1111/j.1365-2559.1980.tb02939.x. [DOI] [PubMed] [Google Scholar]

Associated Data

This section collects any data citations, data availability statements, or supplementary materials included in this article.

Data Availability Statement

Not applicable.


Articles from Urology Case Reports are provided here courtesy of Elsevier

RESOURCES