Multiphenotypic sinonasal carcinoma with adenoid cystic features: reporting a rare case.

doi:10.21203/rs.3.rs-3898258/v6

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Case Report

Multiphenotypic sinonasal carcinoma with adenoid cystic features: reporting a rare case.

https://doi.org/10.21203/rs.3.rs-3898258/v6

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Multiphenotypic sinonasal carcinoma with adenoid cystic features (MSCAC) is an uncommon and aggressive malignancy that primarily affects the sinonasal tract. It is characterized by a varied histological appearance, exhibiting similarities to both adenoid cystic carcinoma (ACC) and other sinonasal malignancies. MSCAC presents a diagnostic challenge due to its histological features that overlap with other tumors, emphasizing the importance of accurate diagnosis for appropriate management and prognosis.

Here, we present the case of a 49-year-old male with no notable medical history, who presented to the Emergency department with nasal bleeding. A biopsy revealed a tumor displaying a prominent basaloid component, accompanied by high mitotic activity and necrosis, an adenoid cystic-like component, and a non-keratinizing squamous cell component. Immunohistochemistry was performed and supported the diagnosis of multiphenotypic sinonasal carcinoma with adenoid cystic features.

In conclusion, MSCAC is a rare and aggressive malignancy of the sinonasal tract. Its diverse histological appearance and overlapping features with other tumors present diagnostic challenges. This abstract provides an overview of MSCAC, highlighting its clinical presentation, histopathological characteristics, diagnostic challenges, and treatment options. Further research and collaboration among experts are necessary to enhance our understanding of this uncommon malignancy and develop standardized diagnostic and therapeutic approaches.

Head & Neck Surgery

sinonasal tumor

adenoid cystic carcinoma

Multiphenotypic sinonasal carcinoma with adenoid cystic features (MSCAC) is an infrequent and aggressive malignancy primarily impacting the sinonasal tract. It is distinguished by a blend of histological characteristics observed in both sinonasal carcinoma and adenoid cystic carcinoma. MSCAC was initially documented in 2013 and has subsequently been acknowledged as a distinct entity by the World Health Organization. It is an unusual neoplasm restricted to the sinonasal tract, exhibiting features of both a surface-derived and salivary gland carcinoma (notably adenoid cystic carcinoma), and is associated with high-risk HPV. Sinonasal carcinoma designates a collection of malignant tumors originating from the epithelial lining of the sinonasal tract. Conversely, adenoid cystic carcinoma is a specific form of salivary gland tumor distinguished by its biphasic growth pattern and perineural invasion.

39-year-old female with a history of allergic rhinitis and sinusitis presented to the emergency room due to nasal bleeding. A computed tomography (CT) scan was performed, revealing a 2 cm mass on the right side at the level of the right middle turbinate (Figure 1). The patient underwent a biopsy, which showed the following histological findings: the tumor exhibited mixed components characteristic of a multiphenotypic carcinoma, including a prominent basaloid component with high mitotic activity and necrosis, an adenoid cystic-like component, and a non-keratinizing squamous cell component. Immunohistochemistry demonstrated that the basaloid nests are diffusely positive for CK7 and are rimmed peripherally by a single layer of p63 and p40-positive myoepithelial cells. CD117 staining was negative, while p16 staining was diffuse and strong. Ki67 staining showed an elevated level (>80%) (Figure 2). Moreover, the tumor tested positive for human papillomavirus (HPV) by in situ hybridization, and MYB rearrangement was negative.

In MSCAC, the tumor exhibits a biphasic growth pattern, with areas resembling both sinonasal carcinoma and adenoid cystic carcinoma. The sinonasal carcinoma component typically shows features of squamous cell carcinoma or undifferentiated carcinoma, while the adenoid cystic carcinoma component displays the characteristic cribriform or tubular growth pattern. This unique combination of histological features distinguishes MSCAC from other sinonasal malignancies.

The etiology of MSCAC remains unclear, and no specific risk factors have been identified. However, some studies suggest a possible association with occupational exposure to wood dust or certain chemicals. Further research is needed to establish a definitive link. Clinically, MSCAC presents with nonspecific symptoms such as nasal obstruction, epistaxis (nosebleeds), facial pain, and swelling. Due to its rarity and overlapping histological features with other sinonasal malignancies, MSCAC is often misdiagnosed or underdiagnosed. Accurate diagnosis requires a comprehensive evaluation, including imaging studies (such as CT or MRI scans), endoscopic examination, and histopathological analysis of biopsy samples.

The differential diagnosis of multiphenotypic sinonasal carcinoma with adenoid cystic features (MSCAC) includes several other types of sinonasal tumors that may have similar clinical and histological features. It is important to differentiate MSCAC from these other tumors to ensure accurate diagnosis and appropriate treatment. However, ACC typically lacks the multiphenotypic differentiation seen in MSCAC, which includes features of both adenoid cystic carcinoma and other types of sinonasal tumors.

Another important differential diagnosis is sinonasal undifferentiated carcinoma (SNUC), which is a highly aggressive and poorly differentiated carcinoma. SNUC can have overlapping histological features with MSCAC, such as solid growth patterns and necrosis. However, SNUC lacks the characteristic cribriform and tubular growth patterns seen in MSCAC. Other tumors that may be considered in the differential diagnosis of MSCAC include sinonasal adenocarcinoma, sinonasal neuroendocrine carcinoma, and sinonasal squamous cell carcinoma. These tumors can have overlapping histological features with MSCAC, but they typically lack the specific features of adenoid cystic carcinoma seen in MSCAC.

To differentiate MSCAC from these other tumors, a combination of clinical, radiological, and histological findings is necessary. Immunohistochemical staining can also be helpful in distinguishing MSCAC from other tumors, as it can demonstrate the presence of specific markers associated with adenoid cystic carcinoma and other types of sinonasal tumors.

Treatment of MSCAC typically involves a multidisciplinary approach, including surgery, radiation therapy, and chemotherapy. The extent of surgical resection depends on the tumor size, location, and involvement of adjacent structures. Adjuvant radiation therapy is commonly employed to target any residual tumor cells and reduce the risk of local recurrence. Chemotherapy may be considered in advanced or metastatic cases, although its efficacy in MSCAC is not well-established.

The prognosis for MSCAC is generally poor, with a high risk of local recurrence and distant metastasis. The 5-year survival rate is estimated to be around 30-40%. Factors associated with a worse prognosis include advanced stage at diagnosis, positive surgical margins, lymph node involvement, and distant metastasis.

In conclusion, multiphenotypic sinonasal carcinoma with adenoid cystic features is a rare and aggressive malignancy of the sinonasal tract. Its unique histological features and clinical presentation pose diagnostic challenges. Further research is needed to better understand its etiology, improve diagnostic accuracy, and develop more effective treatment strategies.

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The authors declare no competing interests.

Consent for publication: A written consent was obtained from the patient to publish his data anonymously.

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Multiphenotypic sinonasal carcinoma with adenoid cystic features: reporting a rare case.

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