Sinonasal renal cell-like adenocarcinoma arising in von Hippel Lindau (VHL) syndrome

Maharaj, Satish; Seegobin, Karan; Wakeman, Kristina; Chang, Simone; Potts, Kevin; Williams, Brian J.; Redman, Rebecca

doi:10.1016/j.oraloncology.2021.105705

Cited by 3 publications

(8 citation statements)

References 15 publications

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“…Sinonasal RCC-like adenocarcinoma (SNRCLA) is a rare malignant sinonasal tumor that is thought to originate from sinonasal seromucinous glands. 1,2,5 The lesion was rst described in 2002 by Moh'd Hadi et al and zur et. al.…”

Section: Discussionmentioning

confidence: 99%

“…There are 3 cases of SNRCLA with VHL that were described in the literature. 2,9,14 All lesions were seen in patients in the 4th decade and it were associated with non-speci c symptoms. Additionally, all of the cases had prior diagnosis of VHL associated neoplasms as well as aggressive behavior unlike our case that was small in size with indolent clinical course.…”

Section: Discussionmentioning

confidence: 99%

“…1 One interesting aspect of the lesions is its association with Von Hippel Lindau (VHL) syndrome which was reported on 3 cases. 2 We herein present the rst reported case of SNRCLA in Saudi Arabia with the morphological and immunohistochemical result and discuss its association with VHL syndrome.…”

Section: Introductionmentioning

confidence: 93%

“…Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare malignant tumor with around 20 cases reported in the literature to date. 1,2 The term sinonasal renal cell-like adenocarcinoma was described initially in two independent papers. 3,4 Under microscopy, it shows uniform low grade clear tumor cells in a hemorrhagic background with cells usually arranged in a follicular or glandular pattern.…”

Section: Introductionmentioning

confidence: 99%

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Sinonasal Renal Cell Like Adenocarcinoma; A Report of Rare Entity with Emphasis on its Association with Von Hippel Lindau Syndrome

AlMadan

Almohammed

Bardisi³

et al. 2023

Preprint

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Background Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare entity that was introduced to head and neck tumors classification on 2017 as an emerging entity. Clinically, it could be asymptomatic or show non-specific symptoms like epistaxis, nasal obstruction, or hyposmia. Diagnosis of the lesion is challenging, especially in small biopsies, and requires utilization of clinical, radiological, histopathological and ancillary tests for accurate characterization of the lesion. There is around 20 cases reported in the literature with 3 cases associated with VHL syndrome. Case Presentation A 41 years old female with a nasal mass for two years ago that presented initially as frequent epistaxis from the right side. Histopathological examination revealed proliferation of clear cells associated with hemorrhagic background forming follicular and glandular structure and dense eosinophilic secretion. Tumor cells were diffusely positive for CK7, EMA, and Inhibin while were negative for CK20, P63, CK 5/6, CD10, RCC, TTF1, PAX8, CEA, and GATA3. Proliferation index (KI67) was less than 5% (Figure.3). The diagnosis was consistent with sinonasal renal cell-like adenocarcinoma. The patient has no recurrence and no symptoms after 1 year. Conclusion: Our study report a rare case of SNRCLA with discussion of the histological features and its association with VHL syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 93%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Sinonasal Renal Cell Like Adenocarcinoma; A Report of Rare Entity with Emphasis on its Association with Von Hippel Lindau Syndrome

AlMadan

Almohammed

Bardisi³

et al. 2023

Preprint

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“…Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare malignant tumor with around 20 cases reported in the literature [ 1 , 2 ]. The term “sinonasal renal cell-like adenocarcinoma” was described initially in two independent papers [ 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

Sinonasal Renal Cell-Like Adenocarcinoma: A Report of a Rare Entity With Emphasis on Its Association With Von Hippel Lindau Syndrome

AlMadan¹,

Almohammed²,

Bardisi³

et al. 2023

Cureus

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Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare malignant sinonasal tumor with relatively indolent clinical course. Clinically, it could be asymptomatic or show non-specific symptoms such as epistaxis, nasal obstruction, or hyposmia. Diagnosis of the lesion is challenging, especially in small biopsies, and requires clinical, radiological, histopathological, and ancillary tests to characterize the lesion accurately. We herein report a case of a 41-year-old female with a nasal mass noted two years ago, which presented initially as frequent epistaxis from the right side. Histopathological examination revealed proliferation of clear cells associated with hemorrhagic background forming follicular and glandular structure and dense eosinophilic secretion. Tumor cells were diffusely positive for CK7, EMA, and inhibin, while they were negative for CK20, P63, CK 5/6, CD10, renal cell carcinoma (RCC), TTF1, PAX8, CEA, and GATA3. The proliferation index (KI67) was less than 5%. The diagnosis was consistent with SNRCLA. The patient has no recurrence and no symptoms after one year. Thus, our study reports a rare case of SNRCLA with a discussion of the histological features and its association with von Hippel Lindau syndrome.

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A Case of Hemangioma of Nasal Cavity in Von Hippel-Lindau Syndrome

Kang,

Jung,

Chae

et al. 2023

Korean J Head Neck Oncol

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We report a unique case of hemangioma of the nasal cavity in von Hippel-Lindau (VHL) syndrome. A 26-year-old female with VHL syndrome who had previously undergone surgery for pancreatic and adrenal mass presented with a 4-month history of left-sided nasal obstruction. The patient had an expansile mass lesion in the left nasal cavity and an imaging test demonstrated the mass in the left maxillary sinus extending to the nasal cavity. The tumor was removed with an endoscopic prelacrimal recess approach considering the possibility of not only a benign tumor such as hemangioma but also a malignant tumor of the maxillary sinus and histopathologic examination confirmed cavernous hemangioma. This case is the rarely reported manifestation of the paranasal sinus in VHL disease.

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Sinonasal renal cell-like adenocarcinoma arising in von Hippel Lindau (VHL) syndrome

Cited by 3 publications

References 15 publications

Sinonasal Renal Cell Like Adenocarcinoma; A Report of Rare Entity with Emphasis on its Association with Von Hippel Lindau Syndrome

Sinonasal Renal Cell Like Adenocarcinoma; A Report of Rare Entity with Emphasis on its Association with Von Hippel Lindau Syndrome

Sinonasal Renal Cell-Like Adenocarcinoma: A Report of a Rare Entity With Emphasis on Its Association With Von Hippel Lindau Syndrome

A Case of Hemangioma of Nasal Cavity in Von Hippel-Lindau Syndrome

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