HPV-Related Multi-Phenotypic Sinonasal Carcinoma with Aggressive Clinical Behavior; A Rare Case

Abstract Introduction/Objective Human Papilloma Virus (HPV)-related multiphenotypic sinonasal carcinoma, previously known as HPV-related sinonasal carcinoma with adenoid cystic carcinoma-like features, is a rare type of sinonasal carcinoma with both epithelial-derived and salivary gland-type morphologic features. It is associated with high-risk HPV, but lacks MYB gene rearrangements. Methods/Case Report We report a case of a 59-year-old male who presented with a rapidly growing sinonasal mass. On MRI, a left nasal cavity lesion was identified growing laterally along the frontal process of the maxilla, extending into the middle meatus and into the maxillary sinus. Patient underwent a complex left medial maxillectomy, spheno- ethmoidectomy, and sinusotomy. On gross evaluation, the left inferior turbinate and sidewall demonstrated a 4 cm unremarkable turbinate with attached friable soft tissue. Microscopic examination revealed sections of carcinoma with various architectural patterns comprised of foci with adenoid cystic carcinoma-like morphology, basaloid squamous cell carcinoma and adenocarcinoma. The tumor showed positive immunostaining for P40, but focal reactivity to S100 and rare scattered reactivity with CD117. INI-1 immunostain was retained in tumor cells. P16 immunostain was strong and diffuse and high-risk cocktail HPV RNA ISH was positive. However, MYB FISH testing was equivocal. Morphologic and immunophenotypic findings were consistent with HPV-related multiphenotypic sinonasal carcinoma. The tumor involved the olfactory nerve fibers requiring a skull base resection and showed extension into the dura mater. Results (if a Case Study enter NA) NA Conclusion HPV related multiphenotypic sinonasal carcinoma is a recently described entity that can pose significant diagnostic challenge. It typically has an indolent clinical course with potential for late recurrences. This case study highlights the potential aggressive nature of this type of sinonasal carcinoma, despite association with high-risk HPV, and use of ancillary testing in aiding diagnosis.

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HPV-Related Multiphenotypic Sinonasal Carcinoma: A Unique Case

International Journal of Surgical Pathology ◽

10.1177/1066896919866508 ◽

2019 ◽

Vol 27 (8) ◽

pp. 888-892 ◽

Cited By ~ 3

Author(s):

Daniel Ching ◽

Shadi Pirasteh ◽

Chanh Ly

Keyword(s):

Salivary Gland ◽

Human Papillomavirus ◽

High Grade ◽

Salivary Gland Carcinoma ◽

Unique Case ◽

Sinonasal Carcinoma ◽

Adenoid Cystic ◽

Gland Carcinoma ◽

Multiphenotypic Sinonasal Carcinoma ◽

High Risk Hpv

Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC), originally known as HPV-related carcinoma with adenoid cystic carcinoma-like features, is a recently described neoplasm that presents only in the sinonasal tract, displays features of both a surface-derived carcinoma and a salivary gland carcinoma, and is associated with high-risk HPV, specifically HPV type 33. Majority of the cases display high-grade histologic features, but HMSC paradoxically behaves in a relatively indolent fashion. Distinguishing HMSC from other histologic mimickers is essential as the management and prognosis are significantly different. In this article, we present a unique case of HMSC and review the literature.

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Human Papillomavirus–Related Carcinoma With Adenoid Cystic–like Features of the Sinonasal Tract (Also Known as Human Papillomavirus–Related Multiphenotypic Sinonasal Carcinoma)

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2018-0027-rs ◽

2019 ◽

Vol 143 (11) ◽

pp. 1420-1424 ◽

Cited By ~ 7

Author(s):

Chia-Chi Chen ◽

Sheau-Fang Yang

Keyword(s):

Human Papillomavirus ◽

High Risk ◽

Hpv Infection ◽

Myoepithelial Cells ◽

Sinonasal Tract ◽

Surface Epithelium ◽

Ductal Cells ◽

Adenoid Cystic ◽

Multiphenotypic Sinonasal Carcinoma ◽

High Risk Hpv

Human papillomavirus (HPV)–related carcinoma with adenoid cystic–like features is a rare, recently recognized entity restricted to the sinonasal tract. By definition, it is associated with high-risk HPV infection, particularly with HPV type 33. In most cases, tumors are composed of dual cell populations, including predominant basaloid myoepithelial cells and usually inconspicuous ductal cells. Solid components with focal cribriform or tubular patterns, abrupt keratinization within tumor nests, and squamous dysplasia of the surface epithelium are characteristics of HPV-related carcinoma with adenoid cystic–like features. The immunohistochemistry of p16 followed by high-risk HPV testing may help in the differential diagnosis. Recent studies have demonstrated that the morphologic features of this entity are more diverse than initially believed. Surgical resection is the prime alternative for treatment. According to the limited data, the prognosis of this disease may be better than that of other sinonasal carcinomas.

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Human Papillomavirus Related Multiphenotypic Sinonasal Carcinoma: Report of a Case with Early and Progressive Metastatic Disease

Journal of Neurological Surgery Reports ◽

10.1055/s-0039-3399571 ◽

2019 ◽

Vol 80 (04) ◽

pp. e41-e43 ◽

Cited By ~ 1

Author(s):

Alejandro I. Rodarte ◽

Anuraag S. Parikh ◽

Shekhar K. Gadkaree ◽

Ashton E. Lehmann ◽

William C. Faquin ◽

...

Keyword(s):

Human Papillomavirus ◽

High Risk ◽

Nasal Congestion ◽

Three Dimensional ◽

Pulmonary Nodules ◽

Sinonasal Carcinoma ◽

Medial Maxillectomy ◽

Multiphenotypic Sinonasal Carcinoma ◽

High Risk Hpv

Introduction Human papillomavirus (HPV) related multiphenotypic sinonasal carcinoma (HMSC) is a newly described entity. The prognosis of this neoplasm is not well understood, but cases often demonstrate high-grade features and paradoxically indolent behavior. Case Report We present the case of a 65-year-old man referred with unilateral nasal congestion and epistaxis. Nasal endoscopy and imaging revealed an expansile mass filling the left nasal cavity. The patient underwent endoscopic-assisted medial maxillectomy, with complete resection and negative margins. Pathology revealed a basaloid tumor consisting of solid nests with multiple foci of cribriform architecture, with positivity for high-risk HPV, thus supporting the diagnosis of HMSC. Postoperatively, the patient received 66.6-Gy adjuvant three-dimensional proton therapy. Twenty-three months after surgical resection, he developed enlarging pulmonary nodules with biopsy features consistent with the primary sinonasal tumor. He was treated with three cycles of chemotherapy and eight cycles of immunotherapy, progressing on both therapies. He remains on palliative chemotherapy. Conclusion Here, we present a case of HMSC with early and progressive distant metastasis. We aim to add to an understanding of the behavior of this entity. Although this neoplasm may typically be indolent, further classification of high-risk features is necessary to identify rare aggressive cases.

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Prevalence and associated survival of high-risk HPV-related adenoid cystic carcinoma of the salivary glands

International Journal of Oncology ◽

10.3892/ijo.2016.3563 ◽

2016 ◽

Vol 49 (2) ◽

pp. 803-811 ◽

Cited By ~ 9

Author(s):

Xu Qian ◽

Andreas M. Kaufmann ◽

Chao Chen ◽

Georgios Tzamalis ◽

Veit M. Hofmann ◽

...

Keyword(s):

High Risk ◽

Adenoid Cystic Carcinoma ◽

Salivary Glands ◽

Adenoid Cystic ◽

High Risk Hpv

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Adenoid cystic carcinoma of the female genital tract – Case series with review of literature

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqab191.157 ◽

2021 ◽

Vol 156 (Supplement_1) ◽

pp. S75-S75

Author(s):

S Bhele ◽

G Crisi ◽

J Hunt

Keyword(s):

High Risk ◽

Adenoid Cystic Carcinoma ◽

Genital Tract ◽

Female Genital Tract ◽

Case Series ◽

Review Of Literature ◽

Adenoid Cystic ◽

High Risk Hpv ◽

Female Genital

Abstract Introduction/Objective Adenoid cystic carcinoma (ACC) of the female genital tract is rare and the published literature is sparse. In the vulva it is considered to arise from Bartholin’s gland whereas, in cervix it arises from the reserve cells. Although morphologically ACC of female genital tract is similar to its counterparts in salivary glands, the clinical behavior is different. Methods/Case Report We describe the clinicopathologic and immunohistochemical features of three cases of adenoid cystic carcinoma of female genital tract with review of literature. The age of our patients ranged from 48 to 75 years with tumors ranging from 1.9 to 3.9 cm. The tumor sites were vulva (2 cases) and cervix (1 case). Two of the 3 patients presented with mass lesions and one patient presented with postmenopausal bleeding. Microscopically, all tumors showed classic morphologic features with cribriform, tubular and solid patterns. On immunohistochemistry, CD117 was consistently reactive in all 3 cases. In addition, the ductal and myoepithelial cell population were highlighted by keratin cocktail and p63 respectively. P16 was diffuse and block-like positive in two cases (one vulvar and cervical ACC). High risk HPV (HPV 33) was detected in one case of vulvar ACC. Two patients underwent surgical excision while one patient was deemed unfit for surgical treatment. All 3 patients received radiation therapy. On follow- up that ranged from 17 to 76 months, one patient is alive without evidence of disease. One patient died of disease with local recurrence and lung metastasis. The other patient died of complications (radiation proctocolitis with colonic perforation). Results (if a Case Study enter NA) NA Conclusion Our cases highlight the aggressive behavior of adenoid cystic carcinoma in the female genital tract and the need for close follow-up. Also high risk HPV has been demonstrated in cervical ACC previously, but our series demonstrates high risk HPV association with vulvar ACC.

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