A Case of Buccal Clear Cell Carcinoma Caused by Rare Fusion Gene: EWSR1-CREM

Clear cell carcinoma (CCC) is a rare entity in the salivary gland tumor. So far, only 10 cases of primary CCC of the buccal mucosa have been reported. Here, we first report an extremely rare case of buccal CCC with the EWSR1-CREM fusion gene. The patient, a 69-year-old woman, presented with a painless mass in the right buccal mucosa. The tumor, which had been present for about 10 years, measured approximately 15 mm in diameter and was pedunculated, elastic hard, smooth, and mobile. Histopathological examination revealed proliferating tumor cells with vacuolated and clear cytoplasm partially surrounded by hyalinized stroma. The tumor was not encapsulated, and no contact with the overlying epithelium was evident. Duct-like structures were occasionally observed in the tumor nests composed of clear cells. The tumor had invaded into surrounding muscle and adipose tissues. Immunohistochemical examination revealed that the clear cells were positive for epithelial cell markers, and myoepithelial markers were negative. Fluorescence in situ hybridization (FISH), performed to search for genetic abnormalities, demonstrated split positivity for EWSR1, and fusion with CREM was confirmed. These findings suggested a diagnosis of CCC.

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A Case of Hyalinizing Clear Cell Carcinoma, So-Called Clear Cell Carcinoma, Not Otherwise Specified, of the Minor Salivary Glands of the Buccal Mucosa

Case Reports in Otolaryngology ◽

10.1155/2015/471693 ◽

2015 ◽

Vol 2015 ◽

pp. 1-7 ◽

Cited By ~ 3

Author(s):

Takahiro Yamanishi ◽

Kiwako Kutsuma ◽

Keisuke Masuyama

Keyword(s):

Cell Carcinoma ◽

Salivary Glands ◽

Tumor Cells ◽

Buccal Mucosa ◽

Clear Cell ◽

Clear Cell Carcinoma ◽

Minor Salivary Glands ◽

Hyalinizing Clear Cell Carcinoma ◽

Not Otherwise Specified ◽

The Right

Hyalinizing clear cell carcinoma (HCCC), so-called clear cell carcinoma, not otherwise specified (CCC (NOS)), of the salivary glands is a rare and low-grade malignant tumor. We report a case of HCCC so-called CCC (NOS) (referred to as HCCC) of the minor salivary gland of the buccal mucosa. A 52-year-old woman had presented with a gradually growing and indolent mass in the right buccal mucosa for about two years. The first biopsy histopathologically suggested the possibility of malignancy derived from the minor salivary glands. A month later, she visited our hospital. The tumor measured approximately 1.5 cm in diameter and was elastic hard, smooth, and well movable. Image examinations demonstrated internal homogeneity of the lesion, which had a smooth margin, in the right buccal mucosa. Complete tumor resection followed by covering with a polyglycolic acid sheet and fibrin glue spray was performed without surgical flap reconstruction. Histopathological findings revealed proliferating tumor cells with clear cytoplasm surrounded by hyalinizing stroma in the submucosal minor salivary glands. Immunohistochemical stains revealed these tumor cells to be positive for epithelial cell markers but negative for myoepithelial ones. These findings confirmed the diagnosis of HCCC. Good wound healing and no evidence of local recurrence and metastasis have been shown since surgery.

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Hyalinizing clear cell carcinoma with EWSR1-ATF1 fusion gene: report of three cases with molecular analyses

Virchows Archiv ◽

10.1007/s00428-014-1676-5 ◽

2014 ◽

Vol 466 (1) ◽

pp. 37-43 ◽

Cited By ~ 17

Author(s):

Takafumi Nakano ◽

Hidetaka Yamamoto ◽

Toshimitsu Nishijima ◽

Sadafumi Tamiya ◽

Hideki Shiratsuchi ◽

...

Keyword(s):

Cell Carcinoma ◽

Clear Cell ◽

Fusion Gene ◽

Clear Cell Carcinoma ◽

Molecular Analyses ◽

Hyalinizing Clear Cell Carcinoma

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Metachronous adrenal metastasis from operated contralateral renal cell carcinoma with adrenalectomy and iatrogenic Addison’s disease

Canadian Urological Association Journal ◽

10.5489/cuaj.2120 ◽

2014 ◽

Vol 8 (9-10) ◽

pp. 744

Author(s):

Hakan Öztürk ◽

Serap Karaaslan

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Radical Nephrectomy ◽

Clear Cell ◽

Clear Cell Carcinoma ◽

Histopathological Examination ◽

Addison’S Disease ◽

Adrenal Metastasis ◽

Addison's Disease

Metachronous adrenal metastasis from contralateral renal cell carcinoma (RCC) surgery is an extremely rare condition. Iatrogenic Addison’s disease occurring after metastasectomy (adrenalectomy) is an even rarer clinical entity. We present a case of a 68-year-old male with hematuria and left flank pain 9 years prior. The patient underwent left transperitoneal radical nephrectomy involving the ipsilateral adrenal glands due to a centrally-located, 75-mm in diameter solid mass lesion in the upper pole of the left kidney. The tumour lesion was confined within the renal capsule, and the histopathological examination revealed a Fuhrman nuclear grade II clear cell carcinoma. The patient underwent transperitoneal right adrenalectomy. The histopathological examination revealed metastasis of clear cell carcinoma. The patient was diagnosed with iatrogenic Addison’s disease based on the measurement of serum cortisol levels and the adrenocorticotropic hormone (ACTH) stimulation test, after which glucocorticoid and mineralocorticoid replacement was initiated. The patient did not have local recurrence or new metastasis in the first year of the follow-up. The decision to perform ipsilateral adrenalectomy during radical nephrectomy constitutes a challenge, and the operating surgeon must consider all these rare factors.

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SUN-480 An Unusual Clear Cell Carcinoma in the Thyroid. Where Is the Primary?

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.584 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Kuan-yu Lin ◽

Shyang-Rong Shih ◽

Jih-Hsiang Lee

Keyword(s):

Thyroid Gland ◽

Cell Carcinoma ◽

Clear Cell ◽

Fusion Gene ◽

Clear Cell Carcinoma ◽

Outpatient Surgery ◽

Thyroid Tissue ◽

Whole Body ◽

Molecular Testing ◽

Thymic Tissue

Abstract Background: Clear cell carcinoma in the thyroid gland is rare. It is important to distinguish primary thyroid clear cell carcinoma from metastases, since its clinical behaviors and treatment options were different. Clinical Case: A 47-year-old woman without past medical history presented to our outpatient surgery department with right neck mass for 8 months. Thyroid ultrasonography revealed a 3.9 cm nodule in the right thyroid. Thyroid function test was within the normal range. Fine needle aspiration cytology of the nodule showed suspicious for neoplasm. She underwent radical total thyroidectomy and lymph node dissection. Pathology revealed an unusual carcinoma comprising cuboidal cells with irregular nuclear contours, some eosinophilic or clear cytoplasm, arranged in infiltrating nests or cords with marked stromal hyalinization, highly suggestive of a clear cell carcinoma. Ectopic thymic tissue was present adjacent to the tumor. By immunohistochemical (IHC) staining, tumor cells were p63 (+), TTF-1 (-), thyroglobulin (-), PAX8 (-), synaptophysin (-), CD5 (-), and CD117 (-). Tumor genetic sequencing detected EWSR1-CREM fusion genes. For disease extent evaluation, two out of seven lymph nodes obtained during operation were positive of tumor metastases. Whole body computed tomography (CT) 3 months after operation revealed no residual thyroid tissue, neck lymphadenopathies or intra-abdominal metastases. A pulmonary ground-glass opacities of 7mm in diameter was found, which was stationary at a repeated CT scan 6 months later. Clinical Lesson: Clear cell carcinoma in the thyroid gland could be primary, arising from clear cell change of follicular or papillary thyroid carcinoma. The negative IHC stain of thyroglobulin, TTF, PAX8, as well as lack of papillary or follicular architecture made primary thyroid clear cell carcinoma unlikely. Most of the metastatic clear cell carcinoma to the thyroid gland arose from renal primary. However, there was no clinical or radiographic evidence of renal tumor in our case. Although ectopic thymic tissue was identified on pathology, negative IHC staining of PAX8, CD5 and CD117 made thymic origin less likely. Positive IHC staining of p63 and a novel EWSR1-CREM fusion gene confirmed the diagnosis of salivary clear cell carcinoma. According to our literature review, there were only 3 cases of clear-cell carcinoma with EWSR1-CREM fusion gene (1), and our case is the first case who presented with clear cell carcinoma in the thyroid. In conclusion, the importance of IHC stain and molecular testing in determining the primary origin of clear cell carcinoma were addressed in our case. Reference: (1) Chapman E, et al. Am J Surg Pathol. 2018;42(9):1182-1189

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A case of clear cell carcinoma, not otherwise specified （NOS） of the buccal mucosa

Japanese Journal of Oral & Maxillofacial Surgery ◽

10.5794/jjoms.58.288 ◽

2012 ◽

Vol 58 (5) ◽

pp. 288-291 ◽

Cited By ~ 1

Author(s):

Megumi ONODA ◽

Katsuhiro SEKI ◽

Tatsuya IKARI ◽

Wataru KUMAMARU ◽

Tsuyoshi SUGIURA ◽

...

Keyword(s):

Cell Carcinoma ◽

Buccal Mucosa ◽

Clear Cell ◽

Clear Cell Carcinoma ◽

Not Otherwise Specified

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An unusual case of hyalinizing clear cell carcinoma in a sinonasal cavity and cranial base

Journal of Surgical Case Reports ◽

10.1093/jscr/rjaa436 ◽

2020 ◽

Vol 2020 (10) ◽

Author(s):

Goran Gudelj ◽

Tena Simunjak ◽

Marica Zizic ◽

Boris Simunjak ◽

Martin Jurlina

Keyword(s):

Cell Carcinoma ◽

Clear Cell ◽

Clear Cell Carcinoma ◽

Minor Salivary Gland ◽

Salivary Gland Tumor ◽

Tumor Location ◽

Total Recovery ◽

Hyalinizing Clear Cell Carcinoma ◽

Left Posterior

Abstract Hyalinizing clear cell carcinoma (HCCC) is a rare, predominantly minor salivary gland tumor. Most of these tumors occur in the oral cavity, mainly the palate and tongue. Primary localization of the tumor in the region of the nasal cavity and paranasal sinuses is extremely rare and, with only a few cases reported in the literature so far. We present an extremely rare case of a 61-year-old woman with hyalinizing clear cell carcinoma (HCCC), occupying the left posterior nasal and nasopharyngeal cavity, as a primary tumor location. The patient total recovery was uneventful, and she is now free of disease at three years postoperative follow-up.

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Clear Cell Carcinoma of Palatal Minor Salivary Gland Harboring a Novel EWSR1-ATF1 Fusion Gene: Report of a Case and Review of the Literature

Head and Neck Pathology ◽

10.1007/s12105-020-01211-6 ◽

2020 ◽

Author(s):

Katsutoshi Hirose ◽

Yu Usami ◽

Masaharu Kohara ◽

Sunao Sato ◽

Yuri Iwamoto ◽

...

Keyword(s):

Salivary Gland ◽

Cell Carcinoma ◽

Clear Cell ◽

Fusion Gene ◽

Clear Cell Carcinoma ◽

Minor Salivary Gland ◽

Review Of The Literature

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Rare Case of Metastatic Ovarian Clear Cell Carcinoma With an In Vitro Fertilization Pregnancy

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqz113.036 ◽

2019 ◽

Vol 152 (Supplement_1) ◽

pp. S51-S52

Author(s):

Arati Inamdar ◽

Abraham Loo

Keyword(s):

In Vitro Fertilization ◽

Cesarean Section ◽

Cell Carcinoma ◽

Clear Cell ◽

Clear Cell Carcinoma ◽

Definitive Treatment ◽

Ovarian Clear Cell Carcinoma ◽

Vitro Fertilization ◽

The Right

Abstract The risk of ovarian malignancy is rare in pregnancy with a reported incidence of approximately 1 in 50,000. Ovarian clear cell carcinoma (OCCC) accounts for 5% to 10% of all ovarian carcinomas with mean age of 50 to 55 years and is often associated with endometriosis. We present a case of 38-year-old G2P1 Asian female with an in vitro fertilization pregnancy and history of endometriosis who presented with the right adnexal mass. The transvaginal ultrasound performed during the early pregnancy was suggestive of endometrioma or desmoid tumor. At 32 weeks, the patient presented to the emergency department with preeclampsia and severe headache. An emergency cesarean section was performed due to worsening of the patient’s condition. An ovarian mass arising from the right ovary and with a friable and hemorrhagic internal surface was excised, which weighed 63 g and measured 14.0 × 10.5 × 3.0 cm. Its external surface was smooth, hemorrhagic, but without any excrescences. The serial sectioning revealed multiple cystic lesions filled with necrotic material overall occupying 80% of the mass. Microscopically, the viable ovarian tissue demonstrated tubulocystic/papillary architecture lined by hyperchromatic hobnail cells along with focal areas of solid sheets of tumor cells displaying clear cytoplasm. Immunohistochemical stains for Wilm’s tumor 1 and p53 were negative while positive for PAX8. These findings were indicative of OCCC without overexpression of wild-type p53. The PET scan performed few weeks after delivery showed widespread metastasis. We present a unique case of metastatic OCCC removed at the time of cesarean section in the late-preterm period. Our case not only emphasizes the need for definitive treatment option for endometriosis but also a close imaging surveillance of all masses diagnosed during pregnancy in women with risk factors such as endometriosis and Asian ethnicity. Furthermore, our study advocates the need for possible guidelines for management of such rare cases.

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Diagnosis of Thymic Clear Cell Carcinoma by Cytology

Case Reports in Pathology ◽

10.1155/2013/617810 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4

Author(s):

Seema A. Lale ◽

Patricia G. Tiscornia-Wasserman ◽

Mohamed Aziz

Keyword(s):

Cell Carcinoma ◽

Thymic Carcinoma ◽

Clear Cell ◽

Clear Cell Carcinoma ◽

Histopathological Examination ◽

Pericardial Fluid ◽

Cytological Examination ◽

First Case ◽

Time Of Presentation ◽

Aggressive Clinical Behavior

Clear cell carcinoma of the thymus is a rare tumor. Few cases of clear-cell carcinoma of thymus have been documented (Truong et al., 1990 and Wolfe III et al., 1983). All these cases were diagnosed by histopathological examination of the tissue. Diagnosis of thymic clear cell carcinoma on cytology is extremely challenging. Here we report the first case of thymic clear cell carcinoma diagnosed by cytological examination of the pericardial fluid with the help of immunocytochemistry. Differential diagnosis included adenocarcinoma, mesothelioma, and thymic clear cell carcinoma. Thymic carcinoma with clear cell features has an aggressive clinical behavior including our case, where it was already metastasized at the time of presentation.

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