Sinonasal Renal Cell-like Adenocarcinoma Research Articles

Top 10 Clear Cell Head and Neck Lesions to Contemplate.

Optically clear cytoplasm may occur in neoplastic and non-neoplastic conditions, either as a characteristic feature of a disease entity or as a morphologic rarity, potentially creating diagnostic dilemmas in various organ systems. In the head and neck, clear cell change can occur in lesions of salivary, odontogenic, thyroid, parathyroid, or sinonasal/skull base origin, as well as in metastases to these regions. This review elaborates the top ten clear cell lesions in the head and neck, emphasizing their distinguishing histologic, immunohistochemical, and molecular attributes, and presents a rational approach to arriving at an accurate classification. Cytoplasmic pallor or clearing may be caused by accumulations of glycogen, lipid, mucin, mucopolysaccharides, water, foreign material, hydropic organelles, or immature zymogen granules. Overlapping morphologic features may present a diagnostic challenge to the surgical pathologist. Similarity in immunohistochemical profiles, often due to common cell type, as well as rare non-neoplastic mimics, furthers the diagnostic conundrum. The top ten lesions reviewed in this article are as follows: (1) clear cell carcinoma (salivary and odontogenic), (2) mucoepidermoid carcinoma, (3) myoepithelial and epithelial-myoepithelial carcinoma, (4) oncocytic salivary gland lesions, (5) squamous cell carcinoma, (6) parathyroid water clear cell adenoma, (7) metastatic renal cell carcinoma (especially in comparison to clear cell thyroid neoplasms), (8) sinonasal renal cell-like adenocarcinoma, (9) chordoma, and (10) rhinoscleroma.

Sinonasal Renal Cell-Like Adenocarcinoma

Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a newly defined, rare malignant tumor of the nasal cavity. The clinical course and response to treatment remain uncertain. The purpose of this study is to report a new case of SNRCLA and review the literature to determine clinical characteristics, treatment options, and outcomes. A 26-year-old male presented with headache, epistaxis, and nasal obstruction. Physical examination revealed a tumor involving bilateral ethmoid sinuses and MRI revealed extension through the cribriform plate. Surgical excision with endonasal and a bifrontal craniotomy was performed followed by adjuvant radiotherapy (RT). After RT, the patient had persistent disease requiring salvage surgery.There are few previously reported cases of SNRCLA. A literature review yielded 14 previously reported cases with convincing diagnostic evidence of SNRCLA. Common presenting symptoms were epistaxis and nasal obstruction. Surgical excision was the primary treatment in fourteen cases, nine received RT, and none received chemotherapy. However, three cases had persistent or recurrent disease. Surgical excision is the mainstay of treatment for SNRCLA and adjuvant RT has been used in some patients with varying outcomes. The tumor is low grade with no reported cases of metastases or death. The best practice for treatment is yet to be determined.

Sinonasal renal cell-like adenocarcinoma: a clinicopathological analysis of five cases

Objective: To investigate the clinicopathological features, immunophenotype, differential diagnosis and prognosis of sinonasal renal cell-like adenocarcinoma. Methods: Retrospective analysis was performed on the cases of sinonasal carcinoma from August 2014 to December 2018 at Eye, Ear, Nose and Throat Hospital, Fudan University. Renal cell-like adenocarcinoma was screened for clinicopathologic feature analysis, and relevant literatures were reviewed. Results: There were 460 cases of sinonasal carcinoma, among which 70 cases (15.2%) were adenocarcinoma, with five (1.1%) being renal cell-like adenocarcinomas. Four patients were male and one was female, with a mean age of 46.5 years (range 29-52 years). The main clinical manifestations were nasal obstruction and epistasis. A red polypoid mass was found under nasal endoscopy. Imaging showed nasal cavity and ethmoid sinus mass with invasion into surrounding structures and bone destruction. Microscopically, the tumor cells were arranged in nests, alveoli and microcapsules with abundant intervening capillaries, accompanied by hemorrhage. The cytoplasm of the cells was clear with low nuclear grade, and the nucleoli were inconspicuous. In some areas, the tumor invaded bone tissue. Immunohistochemical markers CKpan, CK7, CAⅨ, S-100 and vimentin were positive, with low Ki-67 proliferation index. RCC, CD10, PAX8, p63, SMA, HHF35, Calponin, CD117, TTF-1 and neuroendocrine markers Syn and CHG were all negative. EWSR1 and ETV6 gene rearrangements were not detected by FISH. All five patients underwent surgical resection after initial diagnosis. One patient underwent surgical resection after second recurrence and adjuvant radiotherapy, one patient received postoperative radiotherapy, one patient underwent surgical resection after recurrence, one patient had no recurrence and one patient received radiotherapy after recurrence. All five patients had no distant metastasis and survived without tumor up to December 2019. Conclusions: Primary sinonasal renal cell-like adenocarcinoma is a special subtype of low-grade non intestinal adenocarcinoma, with low incidence and inert biologic behavior. At present, most of the literatures are case reports. Before a diagnosis is made, other primary and metastatic clear cell tumors need to be excluded. Immunohistochemistry is helpful for diagnosis and differential diagnosis. Surgical resection is the mainstay of treatment, and may be supplemented by radiotherapy.

Sinonasal renal cell-like adenocarcinoma with EGFR overexpression of the maxillary sinus: Report of a high-grade case and a review of the literature

Sinonasal renal cell-like adenocarcinoma (SRCLA), which is an extremely rare but established entity, belongs to the non-salivary gland type of low-grade adenocarcinomas arising from the sinonasal tract. We report a case of high-grade SRCLA. The patient was a 63-year-old Japanese male, who complained of nasal obstruction. Histologically, the tumor exhibited solid nests and sheet-like structures composed of atypical clear or eosinophilic cells with central necrosis. The tumor cells contained periodic acid Schiff-positive glycogen. Immunohistochemically, they were positive for cytokeratin (CK)7, CD10, carbonic anhydrase (CA)-IX, the epidermal growth factor receptor (EGFR), mucin 1, and cancer antigen 125, whereas they were negative for CK20, caudal type homeobox 2, CK5/6, p40, vimentin, S-100, a renal cell carcinoma marker, paired box gene 8, and the human epidermal growth factor receptor 2. Both local recurrence and late lymph node metastases arose in this case. Therefore, the tumor was considered to be clinically aggressive and was diagnosed as a high-grade SRCLA. Immunostaining of CD10 and CA-IX was very diagnostically useful. Although 14 cases of SRCLA have been reported previously, all of them involved low-grade malignancies, and no descriptions of their EGFR statuses were provided. This is the first report about a high-grade SRCLA exhibiting EGFR overexpression.

Clinical management of emerging sinonasal malignancies.

Several emerging sinonasal malignancies have recently been described in the pathology literature. Although not all distinctly classified by the World Health Organization, these rare tumors present a management challenge to surgeons and oncologists. While prior studies have summarized histologic details, a clinically focused review is currently lacking in the literature. This review describes the presentation, histopathology, imaging, treatment, and prognosis of newly described or recently evolving sinonasal malignancies while highlighting the distinguishing features of these entities. It includes teratocarcinosarcoma, human papillomavirus-related multiphenotypic carcinoma, biphenotypic sinonasal sarcoma, sinonasal renal cell-like adenocarcinoma, NUT-midline carcinoma, squamous cell carcinoma associated with inverted papilloma, sinonasal undifferentiated carcinoma, and INI-1-deficient sinonasal carcinoma. By describing the diagnosis, treatment, and prognosis of these recently defined entities, this clinical review aims to help guide oncologists in the clinical management of these patients.

Sinonasal renal cell-like adenocarcinoma

A case of clinical observation of a Sinonasal Renal Cell-like Adenocarcinoma in a patient of 17 years is described. In the article the description of the case of Sinonasal Renal Cell – like Adenocarcinoma is described for the first time in the domestic literature and clinical and histological features are analyzed in detail. Patient's parents agreed to use personal dats and photos in research and publications.

Sinonasal renal cell–like adenocarcinomas: robust carbonic anhydrase expression

We report 3 new patients with sinonasal renal cell-like adenocarcinoma (SNRCLA). One case submitted in consultation demonstrated robust carbonic anhydrase IX (CA-IX) expression, leading us to a broader inquiry of CA-IX and carbonic anhydrase II (CA-II) expression in other SNRCLA, Schneiderian tissues, and histologic mimickers. Robust cytoplasmic and membranous CA-IX expression is demonstrated in 6 of 7 SNRCLAs; CA-II expression was demonstrated in 2 of 5 cases. Robust, diffuse CA-II expression is demonstrated throughout sinonasal seromucinous glands in all 10 normal Schneiderian samples. CA-IX is also expressed in all normal sinonasal samples, albeit focally. The closest salivary mimic to SNRCLA is hyalinizing salivary clear cell carcinoma; only focal CA-IX expression was demonstrated in 1 of 2 cases studied. Carbonic anhydrase expression in Schneiderian tissue speaks to its role in regulating the ion concentration of sinonasal secretions and may also explain the origin of this rare tumor.

Envisioning the Next WHO Head and Neck Classification

The next WHO classification should abandon "salivary duct carcinoma"; conventional salivary duct carcinoma should be classified as "high-grade salivary duct carcinoma". Low-grade salivary duct carcinoma should replace the current nosology of "low-grade cribriform cystadenocarcinoma". Cystadenocarcinoma should be classified with the descriptor "Not Otherwise Specified" and should be considered an exclusionary diagnostic category. On the other hand, "Not Otherwise Specified" does not fit for hyalinizing clear cell carcinoma (HCCC). The EWSR1-ATF1 fusion is specific for HCCC within the context of salivary neoplasia. We recommend adding "hyalinizing" even though this feature is not present in all cases; the benefit of which is the mental association with a salivary clear cell malignancy. Sinonasal Renal Cell-like Adenocarcinoma (SNRCLA) is a distinct clear cell neoplasm and should be added to the next WHO classification. Future studies will bear out whether SNRCLA is even a low-grade carcinoma, or may be reclassified as "adenoma". Lastly, the next WHO monograph should include the Risk Model in the general introductory statements on oral squamous cell carcinoma, under a subheading of "Histological Prognosticators". The positive predictive value for developing locoregional recurrence in patients with low-stage oral cavity squamous carcinoma (OSCC) and "worst pattern of invasion type-5" (WPOI-5) is 42 %. Low-stage high-risk OSCC with a combination of features other than WPOI-5 is associated with 32 % likelihood for locoregional progression. WPOI-5 also predicts occult metastatic disease (p = 0.0001, Chi squared, 2 DF). Thus the Risk Model can also be used to make decisions regarding staged elective neck dissections.

Sinonasal tumors: a clinicopathologic update of selected tumors

The sinonasal cavities show a wide variety of neoplasms of epithelial, mesenchymal, neural/neuroectodermal or hematopoietic origin. The differential diagnosis for these tumors may be difficult due to overlapping morphologies, variable patterns in ancillary studies, and potentially confusing terminology. In this report, an updated review of the spectrum of neoplasia is provided, using the World Health Organization 2005 classification as a guide. Classic tumors that are generally limited to the sinonasal tract are described and new information regarding molecular pathogenesis is reviewed. Also new entities that have the sinonasal tract as a site of predilection, such as sinonasal renal cell-like adenocarcinoma and NUT midline carcinoma are highlighted.

Sinonasal Renal Cell-like Adenocarcinoma: A Report on Four Patients

We have described an unusual sinonasal neoplasm which is a histological mimic of renal cell carcinoma (RCC) and coined the nosological classification "sinonasal renal cell-like adenocarcinoma" (SRCLA) to describe this unusual entity. Since the original description (Zur et al. Otolaryngol Head Neck Surg 128:441-7, 2002), we have reviewed the case reported by Moh'd Hadi et al. (Rhinology 40:44-7, 2002) and have seen two new cases in consultation. Our purpose here is to describe the additional cases and to extend the reported outcome for these patients. Four patients were identified. Slides and immunohistochemistry results were reviewed in consultation. Updated clinical follow-up was obtained from the respective clinicians. This group consisted of three women, one man, 22-69 years, and mean 46. Three tumors were in the nasal cavity and one was in the nasopharynx. Histologically, these tumors were uniformly composed of clear cells, forming either solid or glandular patterns. The tumor cells were cuboidal to polyhedral; transition to short spindle cells was seen in one case. One case revealed moderate nuclear pleomorphism. No perineural or vascular invasion, or necrosis was seen. No mucin-producing or squamous elements were seen. Immunohistochemistry (IHC) revealed the following staining profile: CK7 + (4/4), CK20 + (focal 1/4), S100 + (1/4), and CD10 + (1/2). No staining was seen for vimentin (0/4), RCC (0/2), thyroglobulin (0/2), actin (0/2), or calponin (0/2). Three patients were treated primarily with surgery, two patients also received adjuvant radiotherapy (RT); the fourth patient was treated with primary RT. All patients are disease-free, based on endoscopy and/or radiography, 2, 4, 5 and 8 years after diagnosis. Renal cell carcinoma has not been identified in any patient. Sinonasal renal cell-like adenocarcinoma is a rare and distinct entity noteworthy in its resemblance to RCC. Immunohistochemistry can easily distinguish between these two tumors. No patient developed recurrent or metastatic disease, or was found to have RCC. Greater experience will allow us to fully understand its long-term behavior and arrive at more standardized therapeutic recommendations.