
==== Front
Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)01052-6
10.1016/j.ijscr.2024.110271
110271
Case Report
Squamous cell carcinoma arising in chronic hidradenitis suppurativa: A case report and comprehensive literature review
Filho Andrey Salgado Moraes
Pazin Giovanna Savoy
Genaro Lívia Moreira
Oliveira Priscila de Sene Portel
Ayrizono Maria de Lourdes Setsuko
Leal Raquel Franco rafranco@unicamp.br
⁎
Colorectal Surgery Unit, School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil
⁎ Corresponding author at: Colorectal Surgery Unit, Department of Surgery, School of Medical Sciences, University of Campinas, Carlos Chagas Street, 420, Cidade Universitária Zeferino Vaz, Campinas 13083-878, Sãoo Paulo, Brazil. rafranco@unicamp.br
10 9 2024
10 2024
10 9 2024
123 11027129 7 2024
3 9 2024
6 9 2024
© 2024 The Authors
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction

Hidradenitis Suppurativa (HS) is a chronic inflammatory disorder that affects the pilosebaceous unit. Squamous Cell Carcinoma (SCC) can emerge as a complication.

Presentation of case

A 58-year-old male patient with a history of smoking, obesity, and type 2 diabetes was initially managed by a dermatology team for Follicular Occlusion Syndrome manifesting as HS. Despite clinical treatment, the patient was referred to the Coloproctology Unit because of the development of a lesion in the perianal region near the HS lesions. Physical examination revealed an ulcerated, vegetative, painful, and friable lesion in the right perianal region consistent with SCC of the HS scar. The patient underwent chemotherapy and radiotherapy, but the lesions recurred, necessitating abdominoperineal amputation of the rectum.

Discussion

Although rare, patients with chronic HS are at an increased risk of developing SCC, particularly in the perineal and gluteal regions. The standard treatment protocol for SCC in HS involves chemoradiotherapy with the aim of preserving the anal sphincter and avoiding surgery. Surgical intervention is reserved for patients that are unresponsive to chemoradiotherapy or for advanced cases in which local resection is insufficient.

Conclusion

This disease course aligns with the epidemiology of HS, which predominantly affects male individuals with chronic lesions in the perianal, gluteal, and perineal regions. Such lesions can progress severely, often resisting non-invasive treatments and requiring more aggressive surgical interventions.

Highlights

• Chronic Hidradenitis Suppurativa has an increased risk of developing Squamous Cell Carcinoma, though rare.

• Squamous cell carcinoma with hidradenitis suppurativa is severe, with high early metastasis and mortality.

• Standard treatment uses chemoradiotherapy to preserve the anal sphincter; surgery is recommended for advanced cases.

Keywords

Squamous cell carcinoma
Colorectal surgery
Hidradenitis suppurativa
Case report
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pmc1 Introduction

Hidradenitis Suppurativa (HS) is a chronic inflammatory disorder affecting the pilosebaceous unit, and patients typically present with painful, deep subcutaneous nodular lesions and sinus tracts located in intertriginous areas, such as the axillary, inguinal, and gluteal regions. The exact etiology of HS remains unknown; however, it tends to be associated with factors such as poor hygiene, diabetes, smoking habits, and immunosuppression. The primary pathogenic mechanism involves follicular occlusion, which leads to secondary inflammation and destruction of the pilosebaceous apocrine apparatus. This process extends to the adjacent subcutaneous tissue, resulting in an inflammatory response, often accompanied by glandular bacterial infections [1].

Squamous Cell Carcinoma (SCC) can arise as a complication of HS, with a prevalence of 4.6 % [2]. Although infrequent, SCC is the most severe complication of HS [3]. It is more prevalent in females, but males have more significant involvement in the perineal region [4]. Most cases of SCC arising from HS are well-differentiated. Despite this favorable histological prognosis, SCC associated with HS generally manifests as a severe disease with the potential for early metastasis and high mortality rates [2]. Therefore, this case report aims to present a rare case of HS lesions that degenerated into malignant neoplasia (SCC) and were treated surgically.

2 Methodology

This study adhered to the SCARE (Surgical CAse REport) Criteria 2023 for reporting surgical case reports [5]. The patient provided written informed consent to publish this case report and accompanying images.

3 Case report

A 58-year-old male with a history of smoking, obesity, and type 2 diabetes was initially monitored by a dermatology team for Follicular Occlusion Syndrome manifested by HS and was treated with acitretin 10 mg. He was referred to the Coloproctology Unit at the Hospital das Clínicas of the University of Campinas (Unicamp) for the development of a sinus tract near the perianal region secondary to HS. The patient underwent multiple resections of the fistula and sinus tracts, and abscess drainage. His treatment history included isotretinoin, methotrexate, dapsone, and infliximab for four years.

In March 2020, the patient presented with an ulcerated, vegetative, painful, and friable lesion in the right perianal area. A digital rectal examination revealed no abnormalities or inguinal lymph node enlargement. Pelvic magnetic resonance imaging (MRI) demonstrated neoplastic tissue proliferation on the right anal verge, measuring 5.2 × 1.2 × 3.0 cm, with neoplastic involvement of the internal and external sphincters. A biopsy revealed SCC (T3N1M0) within the HS scar. Between August and October 2020, the patient underwent three cycles of chemotherapy (Cisplatin + Capecitabine) and radiotherapy as a curative attempt.

In June 2021, the patient developed a new lesion, and a subsequent biopsy confirmed recurrence of the neoplasia. In July 2021, he contracted SARS-CoV-2, leading to severe acute respiratory syndrome, necessitating invasive mechanical ventilation (orotracheal intubation and tracheostomy). He remained hospitalized for an extended period and was discharged in September 2021. The prolonged hospitalization, complications related to COVID-19, and immunosuppression compromised his nutritional status.

In March 2022, follow-up pelvic MRI revealed an ulcerated lesion with a cranial margin in the distal intersphincteric plane of the anal canal, near the anal verge, located in the right posterolateral perianal region. The lesion extended inferiorly into the cutaneous and subcutaneous planes to the right of the intergluteal groove, with an estimated extension of 4.6 cm and a thickness of up to 1.1 cm. Additionally, a lymph node in the right pelvic lateral chain (internal iliac chain) was observed, measuring 1.2 × 1.0 cm, with regular contours, rounded and heterogeneous, presenting an indeterminate signal (Fig. 1).Fig. 1 Magnetic Resonance Imaging of the Pelvis. The lesion extends inferiorly into the cutaneous and subcutaneous plane to the right of the intergluteal groove, measuring approximately 4.6 cm in extension and up to 1.1 cm in thickness. (A) Transverse section – ulcerated lesion on the right anal verge corresponding to squamous cell carcinoma (SCC). (B) Sagittal section – lymph node in the right internal iliac chain.

Fig. 1

To improve the patient's pre-operative nutritional status, a plan was implemented to prepare the patient for abdominoperineal amputation of the rectum with terminal colostomy. A two-team approach was used with the patient in the lithotomy position. This procedure completely removed the sigmoid, rectal, and anal sphincters using both abdominal laparotomic and perineal incisions, resulting in permanent colostomy. The proximal sigmoid region was then sectioned. The mesorectum was entirely removed and the dissection reached the level of the levator muscles. The surgeon in the perineum delimited the skin resection area (Fig. 2A) and dissected the subcutaneous tissue into the ischiorectal fat to the level of the levator muscles to remove the surgical specimen from the perineum. The intraoperative findings were in accordance with the pre-operative radiological examinations. Therefore, this procedure aimed to remove the entire lesion (Fig. 2) and reconstruct the pelvic floor (Fig. 3) using a V—Y flap on the right side of the perineal region and a rotation flap on the left side. The surgical specimen was sent to the Pathology Unit where the presence of SCS was confirmed, demonstrating the characteristic pattern of cell blocks (Fig. 4). The procedure was performed in April 2022, followed by 24 sessions of hyperbaric therapy to enhance healing of the surgical wound.Fig. 2 Squamous Cell Carcinoma. Well to moderately differentiated squamous cell carcinoma in the anal canal with extensive areas of chorion fibrosis. Proximal, distal, and radial surgical margins free of neoplasia. (A) Delimitation of surgical margins. (B) Perineal area after resection of the neoplasia. (C) Surgical specimen.

Fig. 2

Fig. 3 Immediate postoperative appearance. Complete removal of the lesion and reconstruction of the perineum using a V—Y flap on the right and a rotation flap on the left side.

Fig. 3

Fig. 4 Histopathological Analysis. Infiltrative pattern squamous cell carcinoma confirmed the presence of squamous cell carcinoma (SCC) with the cell blocks pattern (shown in the arrows). (A) 40× magnification. (B) 400× magnification.

Fig. 4

The patient remains monitored in the outpatient clinics of the coloproctology, clinical oncology, and stomatotherapy teams. A Positron emission tomography/computed tomography (PET/CT) scan with fluorodeoxyglucose (FDG) performed in September 2023 did not reveal any hypermetabolic lesions suggestive of neoplasia.

4 Discussion

The incidence of malignant neoplasia in the perineal region is generally low; however, SCC is the second most commonly diagnosed malignancy [6]. The primary sites of involvement are the genital, perineal, and gluteal regions [7]. Few cases have been described in the literature, as highlighted by Chapman et al. (2018) who found only 85 cases [8], which indicates the importance of new studies involving this complication of HS. Studies have already demonstrated that patients with chronic HS are at a greater risk of developing SCC, especially when the perineal and gluteal regions are involved, despite being rare event [[8], [9], [10]]. The interval between the onset of HS and the diagnosis of SCC varied significantly and ranged from 10 [11] to 53 years [9]. In our case, this interval spanned 45 years.

Our case report describes a male patient who had an HS lesion progressing to SCC, corroborating the findings of Kurayev et al. (2016) who demonstrated greater involvement of HS lesions in the perineal region in males [4]. Smoking is also associated with the development and worsening of HS, the mechanisms of which include the pro-inflammatory action of tobacco, which can trigger and perpetuate the inflammatory response characteristic of the disease. Furthermore, smoking compromises the microcirculation of the skin, making it difficult for wounds to heal and favoring the formation of abscesses. This was observed in our patients as well as in the cases reported by Jourabchi et al. (2017) [9], Artri et al. (2021) [11], and Zhang and Tan. (2017) [12], Roy et al. (2019) [13], and Juliver et al. (2019) [14]. This association was not seen [15]. In general, sex, location of lesions, and chronicity of HS, as described in the literature [16] as risk factors for SCC progression, were similar to those found in our patient.

The pathophysiology of HS involves the occlusion of hair follicles, followed by dilation, hyperkeratosis, and hyperplasia, which results in the rupture of follicles and the release of keratin and bacteria into the dermis. This process is accompanied by the recruitment of several pro-inflammatory components [8,17]. The literature indicates that obesity is a significant risk factor due to the ability of adipocytes to secrete pro-inflammatory cytokines, contributing to the chronic inflammation observed in HS [18]. Obesity was also a comorbidity found in our patient.

Treatment of perianal SCC involves a multidisciplinary approach involving radiotherapy, chemotherapy, and rescue surgery, if necessary. Therapeutic decisions must be individualized, considering the extent of the disease, the patient's general health, and the preservation of anorectal function [3]. The standard conventional treatment for SCC is chemoradiotherapy, which preserves the anal sphincter while avoiding the need for surgery. However, there is a margin of failure of 20–30 %, which can lead to disease recurrence in approximately 10–15 % of cases [19,20]. In such cases, abdominoperineal amputation of the rectum must be performed to control the disease and reduce the risk of mortality [21].

Studies have shown that this approach provides effective local control of the disease, especially in large tumors or cases with deep infiltration [22]. Nigro et al. (2019) [23] reported that despite the high rate of complications, abdominoperineal amputation of the rectum is a viable option to achieve tumor-free surgical margins in cases where other therapeutic modalities fail to treat SCC. In their case study, Lahhan et al. (2023) [24] reported an alternative for the treatment of SCC in patients with advanced disease and nonsurgical viability through PD-1-targeted immunotherapy (pembrolizumab), which has proven to be innovative and promising as it has greater tolerability and lower rates of side effects. Ruggiero et al. (2023) [25] demonstrated the use of Cemiplumab, also a PD-1-targeted immunotherapy for unresectable lesions. However, after 18 weeks, the lesion regressed and surgery became feasible. The challenge in managing SCC that evolves as a complication of HS is its early diagnosis, as many patients have skin aggression for a long time and are already at an advanced stage at diagnosis [26].

Table 1 presents a compilation of published case reports. Our patient first received chemoradiotherapy using cisplatin and capecitabine; however, because of the recurrence of the lesion after eight months of treatment, abdominoperineal amputation was indicated. The patient remained disease-free after two years of follow-up until the present report.Table 1 Cases reported in the literature about the occurrence of squamous cell carcinoma in patients with hidradenitis suppurativa.

Table 1Author	Number of cases	Sex	Age	Smoking habit	Duration until diagnosis	Hidradenitis site	Anatomo-pathological exam	Metastasis	Treatment	Follow-up	
Jourabchi et al. 2017 (9)	1	Male	64	Yes	53y	Glutes	Moderately differentiated invasive SCC	No	Lesion resection + loop colostomy	Death after 48 months of diagnosis	
Artri et al. 2021 (11)	3	Male	56a	Yes	20y
15y
10y	Glutes;
Glutes and perianal;
Glutes	Well differentiated SCC;
poorly differentiated SCC;
Moderately differentiated SCC	Yes	Lesion resection + loop colostomy + adjuvant RT/CT;
Loop colostomy;
Resection of the lesion	Death after 60 months of diagnosis;
Death after 3 weeks of diagnosis;
No recurrence	
Scheinfeld. 2017 (15)	1	Male	47	No		Glutes, groin, and perianal	SCC	Yes	Resection of the lesion	Death after 11 months of diagnosis	
Zhang and Tan. 2017 (12)	1	Male	59	Yes	29y	Glutes	Moderately differentiated SCC	Yes	Palliative CT	Death after 42 months of diagnosis	
Roy et at. 2019 (13)	1	Male	64	Yes	28y	Glutes	Well differentiated SCC	Yes	Resection of the lesion	Death after 24 months of diagnosis	
Juliver et al. 2019 (22)	1	Male	63	Yes	30y	Glutes and scrotal region	Poorly differentiated SCC	Yes	ATB + drainage	Death after 6 months of diagnosis	
Lahham et al. 2023
(24)	1	Male	50	Yes	20y	Glutes and groin	–	No	Pembrolizumab	Follow-up with dermatologist	
Ruggiero et al. 2023
(25)	1	Male	56	Yes	6y	Scrotum	Well differentiated SCC	No	Cemiplimab
+
Resection of the lesion	Follow-up with dermatologist	
Fekete et al. 2024
(26)	1	Male	72	No	40	Glutes	Poorly differentiated SCC	Yes	Resection of the lesion	Death after 3 months of diagnosis	
a Average age. SCC, squamous cell carcinoma; CT, chemotherapy; RT, radiotherapy; ATB, antibiotics.

5 Conclusion

This case report demonstrates that although rare, the degeneration of HS lesions into malignant neoplasia is a possible outcome of progression to SCC. This case follows the epidemiology, mainly affecting males with chronic lesions in the perianal, gluteal, and perineal area. These lesions can progress seriously and are refractory to noninvasive measures, such as chemotherapy associated with radiotherapy, as proposed in the case reported. Given this refractoriness, a more aggressive surgical approach was necessary, resulting in abdominoperineal rectal amputation.

Informed consent

The patient provided written informed consent for the publication of this case report and accompanying images. The editor-in-chief of this journal will review a copy of the manuscript upon request.

Provenance and peer review

Not commissioned - externally peer-reviewed.

Ethical approval

This case report was approved by the Ethics Committee of the University of Campinas (CAAE number 80560024.5.0000.5404).

Funding

No funding was received for this research.

Author contribution

Andrey Salgado Moraes Filho contributed to data collection and authored the paper.

Giovanna Savoy Pazin contributed to data collection and assisted in manuscript preparation.

Lívia Moreira Genaro contributed to manuscript preparation.

Priscila de Sene Portel Oliveira contributed to manuscript preparation.

Maria de Lourdes Setsuko Ayrizono participated in the colorectal surgery and contributed to the final revision of the manuscript.

Raquel Franco Leal supervised and conceptualized the study, participated in the colorectal surgery, and contributed to the final manuscript revision.

Guarantor

Raquel Franco Leal, MD, PhD.

Research registration number

Not applicable. This study is a case report.

Conflict of interest statement

The authors declare no competing interests or personal relationships that could have influenced the work reported in this study.

Acknowledgements

We thank Dr. Morgana Danubia Gomes de Souza Bonfitto for histopathological assistance and Prof. Renata Ferreira Magalhães for dermatological consultations. We thank Professor Tristan Guillermo Torriani and Editage (www.editage.com.br) for reviewing the English version of the manuscript.
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