CASE REPORT |
https://doi.org/10.5005/jp-journals-10001-1563 |
Sarcomatoid Squamous Cell Carcinoma of the Scalp: Case Report and Review of the Literature
1Department of Otolaryngology, Stritch School of Medicine, Loyola University Chicago, Illinois, United States
2,5Department of Otolaryngology, Loyola University Medical Center, Illinois, United States
3,4Department of Pathology, Loyola University Medical Center, Illinois, United States
Corresponding Author: Brian T Yuhan, Department of Otolaryngology, Loyola University Medical Center, Illinois, United States, Phone: +1 708 216 5120, e-mail: btyuhan@gmail.com
Received on: 30 November 2022; Accepted on: 07 June 2024; Published on: 17 July 2024
ABSTRACT
Aim and background: Sarcomatoid squamous cell carcinoma (SSCC) is a rare malignant variant of SCC. Here, we present a rare case of primary cutaneous SSCC of the scalp.
Case description: We present a case of a 68-year-old male with a very large pedunculated left scalp mass, first noted several years ago, with rapid expansion of the mass 6 months prior to presentation. The mass was mobile of the cranium and without calvarial involvement. He successfully underwent a wide local excision of the mass with no evidence of recurrence at his 6-month follow-up visit.
Conclusion: This case of primary scalp cutaneous SSCC represents a rare head and neck site of involvement. Due to the rarity of primary cutaneous SSCC and very few reports of primary cutaneous SSCC arising in the head and neck, management is guided by case series of SSCC arising in other sites. Surgical resection is the recommended primary modality of treatment, with or without regional lymphadenectomy. Adjuvant radiation therapy may be considered for larger tumors, positive lymph nodes, older age, and high-risk features. Due to the concern for a potentially high rate of recurrence, close follow-up is essential.
Clinical significance: Sarcomatoid squamous cell carcinoma is a rare malignant variant of SCC that presents a significant diagnostic challenge, as it shares morphologic and immunohistochemical overlap with other spindle cell tumors. While the majority of SSCC of the head and neck occurs in mucosal sites such as the larynx, the scalp remains an extremely rare presentation, with only a few reported cases in the literature. This review of the literature and case report presents an additional case of primary cutaneous SSCC involving the scalp.
How to cite this article: Rewis K, Yuhan BT, Borys D, et al. Sarcomatoid Squamous Cell Carcinoma of the Scalp: Case Report and Review of the Literature. Int J Head Neck Surg 2024;15(1):17-20.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.
Keywords: Case report, Head and neck, Sarcomatoid, Scalp.
INTRODUCTION
Sarcomatoid squamous cell carcinoma (SSCC) is a rare malignant variant of SCC, reported to account for 3% of all squamous carcinomas in the head and neck.1-3 Having evolved from conventional SCC, dedifferentiation in these tumors is associated with sarcomatoid transformation. While the majority of SSCCs occur in the larynx, they may also be found in other mucosal sites within the head and neck, including the oral tongue, gingiva, nasal cavity, and hypopharynx.1 They pose a significant diagnostic challenge to the pathologist, as the tumor shares morphologic and immunohistochemical overlap with other spindle cell tumors.2 Primary cutaneous SSCC of any site is exquisitely rare, with approximately 130 reported cases in the literature.4,5 The scalp is even more rarely involved, with only seven cases reported.4,6 Here, we present a case of primary cutaneous SSCC involving the scalp, with the goal of reviewing the initial presentation, physical and histologic findings, and approach to management for this rare tumor.
CASE DESCRIPTION
A 68-year-old male with a past medical history significant for alcoholism presented to our head and neck clinic with a large lobulated and pedunculated left parietal scalp mass present for several years (Fig. 1). The mass had been rapidly growing in size over the prior 6 months. On exam, the mass bled easily, was mobile of the cranium, and was nontender. Computed tomography (CT) imaging with contrast revealed an expansile, partially necrotic, heterogeneous-appearing 14 × 8 cm mass centered at the left vertex subcutaneous soft tissue with no calvarial erosion or intracranial extension (Fig. 2). No lymphadenopathy was noted. A biopsy of the mass in the clinic revealed poorly differentiated SCC with sarcomatoid features. A chest X-ray showed no evidence of pulmonary metastasis.
Fig. 1: Preoperative photo of the large lobulated and pedunculated left parietal scalp mass
Figs 2A and B: (A) Sagittal view of soft tissue CT revealing a partially necrotic, heterogeneously appearing 14 × 8 cm mass centered at the left parietal scalp; (B) Sagittal view of bone window CT demonstrating no obvious calvarial involvement
The patient underwent successful wide local excision of the scalp mass, calvarial drilling, and reconstruction with Integra™ Meshed Bilayer Wound Matrix. The patient developed a wound infection with loss of the Integra™. He returned to the operating room, and the Integra™ was replaced with a wound vac. This also failed, and due to patient compliance issues, the decision was made to allow the wound to heal by secondary intention. Final pathology confirmed a biphasic SSCC with liposarcoma-like and pleomorphic sarcoma-like heterologous components (Fig. 3). The epithelial component was positive for AE1/AE3, CK5/6, p40, p63, and p16, and the spindle cell component was positive for CK5/6 and p16, confirming the final interpretation of sarcomatoid carcinoma.
Figs 3A and B: (A) A representative routine hematoxylin and eosin section at 20× revealing a biphasic tumor composed of SCC and sarcoma-like dedifferentiation with liposarcoma-like and pleomorphic sarcoma-like features; (B) A immunohistochemical stain at 10× showing CK positivity
Follow-up has been complicated by the patient’s active alcohol use and multiple admissions for intoxication and withdrawal, but at 6 months, there was no evidence of residual or recurrent locoregional disease.
DISCUSSION
Historically, the histologic classification of these biphasic sarcomatoid carcinomas has been the focus of a great deal of discussion. Classically, these ”biphasic” tumors originate from a monoclonal epithelial tumor, with an additional spindle cell component thought to be derived from squamous epithelium that subsequently undergoes mesenchymal differentiation.7 It is this homologous (organ-appropriate) and heterologous (organ-inappropriate) differentiation that makes histologic diagnosis challenging. Differing rates of epithelial and mesenchymal components may exhibit varying levels of differentiation despite originating from the same epithelioid clone.5 At times, malignant mesenchymal components are undifferentiated or may closely resemble spindle cell carcinoma, fibrosarcoma, chondrosarcoma, leiomyosarcoma, rhabdomyosarcoma, or angiosarcoma.5 A better understanding of these histologic nuances may lead to improved recognition and help prevent mislabeling or misdiagnosis of this rare malignancy.
Clinically, SSCC characteristically grows as a grossly exophytic and ulcerated mass with remnants of dysplastic squamous epithelium and frequently with areas transitioning to malignant spindled tumor cells.8 Accurate diagnosis typically relies on demonstrating epithelial differentiation, either by morphology or by immunohistochemistry for cytokeratins (CKs) and other markers. Specific markers used to identify epithelial differentiation include pancytokeratin (AE1/AE3), CK5/6, epithelial membrane antigen (EMA), MOC-31, and p63. However, currently, there is no clear consensus on which markers are most helpful in diagnosing SSCC.9,10 Keratin reactivity may vary from focal to diffuse, and the absence of CK reactivity does not exclude a diagnosis of SSCC.11 p63 staining is more consistently positive than p40, but it is often limited and present only in scattered malignant cells. Vimentin reactivity is consistently identified in the literature and is often diffusely and strongly reactive.11 Zheng et al. concluded that CK was the most useful and reliable marker for demonstrating epithelial phenotype, while Thompson et al. suggested that keratin AE1/AE3, EMA, and CK18 were the most useful markers.3,10 In addition, there appears to be no association with transcriptionally active human papillomavirus.11 A positive epithelial marker may aid in diagnosing SSCC, and although approximately 70% of cases may exhibit positive epithelial immunoreactivity, a negative or nonreactive result cannot definitively rule out SSCC.11
An understanding of disease-specific clinicopathological features is important to ensure appropriate diagnosis and management of SSCC. However, SSCC appears to have different clinical characteristics based on the primary site, making a determination of appropriate management challenging. Risk factors for SSCC of the head and neck are thought to be excessive alcohol and tobacco consumption, as well as previous local irradiation.3,9,10 Other risk factors may include recent injury and a possible genetic predisposition hypothesized to involve functional loss of genes in the development of the spindle cell phenotype.10 In one review of 187 patients with laryngeal SSCC, the male-to-female predilection was found to approach 13:1, with the mean age of presentation at 65 years.3 They found an overall 5-year survival of 64.4%, improving to 78.7% when excluding patients who died from other causes.3 However, another study of 55 patients evaluating all mucosal locations of head and neck SSCC reported a 3-year survival of 20% and a 5-year survival of 6.7%.12 Recurrence is a concern, with studies reporting anywhere between 49 and 71% rate of locoregional recurrence and a distant metastasis rate of 21%, most commonly involving the lungs, liver, and kidneys.10,12 As primary cutaneous SSCC is exceptionally rare, disease-free survival and rates of recurrence are difficult to predict.
Surgical excision with or without local lymphadenectomy remains the first-line modality of treatment for SSCC, with radiation therapy often offered as optional or mandatory adjunctive therapy.3,9,10 High-risk factors for SSCC are considered similar to those for conventional advanced-stage SCC and include lymphovascular invasion (LVI), perineural invasion (PNI), multiple disease-positive lymph nodes, extracapsular extension, or involved surgical margins. With the presence of any of these factors, multimodal therapy is typically recommended. Several studies in the literature have sought to identify prognostic factors in SSCC that may indicate the need for more aggressive treatment.12-14 Su et al. reported that a high T classification (T3/T4), advanced clinical stage (III/IV), nodal disease, LVI, and distant metastatic recurrence led to poorer prognosis in SSCC of the oral cavity and oropharynx.13 A study of 118 head and neck SSCC patients by Bice et al. also identified tumor size, M-stage, and older age as statistically significant poor prognostic indicators.14 Mingo et al. found that multimodal therapy for SSCC of the head and neck was associated with a decreased incidence of locoregional recurrence, but interestingly, it did not appear to improve overall survival.12 In one study considering the role of adjuvant radiation, 559 patients with head and neck SSCC identified from the surveillance, epidemiology, and end results database (National Cancer Institute) were used to establish a prediction model for survival.15 Overall, postoperative adjuvant radiation was positively associated with improved survival for patients with advanced stage disease (T3/T4, N1-3). Only 5% of patients with early stage disease (T1-2, N0) underwent adjuvant radiation, and no survival benefit was found. Chemotherapy was also found to improve survival for patients with advanced stage disease, with no difference in survival for patients with early stage disease treated with chemotherapy. For patients with advanced stage disease who did not undergo surgery, radiation alone was found to improve survival; however, the difference was not significant.
CONCLUSION
This case of primary scalp cutaneous SSCC represents a rare head and neck site of this SSCC with liposarcoma-like and pleomorphic sarcoma-like heterologous differentiation. Due to the rarity of primary cutaneous SSCC and very few reports of primary cutaneous SSCC arising in the head and neck, management is guided by case series of SSCC arising in other sites. Surgical resection is the recommended primary modality of treatment, with or without regional lymphadenectomy. Adjuvant radiation therapy may be considered for larger tumors, positive lymph nodes, older age, and high-risk features. Due to the concern for a potential high rate of recurrence, close follow-up is essential.
Clinical Significance
Sarcomatoid squamous cell carcinoma is a rare malignant variant of SSC that presents a significant diagnostic challenge, as it shares morphologic and immunohistochemical overlap with other spindle cell tumors. While the majority of SSCCs of the head and neck occur in mucosal sites such as the larynx, the scalp remains an extremely rare presentation, with only a few reported cases in the literature. This review of the literature and case report presents an additional case of primary cutaneous SSCC involving the scalp.
ORCID
Brian T Yuhan https://orcid.org/0000-0001-7755-8131
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