Clear cell odontogenic carcinoma: a rare jaw tumor. A summary of 107 reported cases

Guastaldi, Fernando Pozzi Semeghini; Faquin, William C.; Gootkind, F.; Hashemi, Sean; August, Meredith; Iafrate, A. John; Rivera, Miguel N.; Kaban, Leonard B.; Jaquinet, A.; Troulis, Maria J.

doi:10.1016/j.ijom.2019.05.006

Cited by 34 publications

(43 citation statements)

References 72 publications

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“…A recent systematic review found 107 well-documented cases of CCOC in the English literature [10]. This tumor is most often reported in female patients in the sixth decade of life and posterior mandible is the most affected site, with no ethnic predilection.…”

Section: Discussionmentioning

confidence: 99%

Clear Cell Odontogenic Carcinoma Harboring the EWSR1–ATF1 Fusion Gene: Report of a Rare Case

Santana

Andrade

Melo

et al. 2019

Head and Neck Pathol

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Clear cell odontogenic carcinoma (CCOC) is a rare and aggressive malignant epithelial neoplasm, which occurs most frequently in the mandible of elderly patients. Morphologically, CCOC shares similar characteristics with other clear cell tumors, especially hyalinizing clear cell carcinoma of the salivary glands (HCCC). Both CCOC and HCCC are known to harbor EWSR1 rearrangements, especially the EWSR1-ATF1 gene fusion, which indicates a possible link between the two lesions. So far, this fusion has been demonstrated in five cases of CCOC in the literature. Herein, we add another CCOC case to the literature, which arose in the mandible of an 82-year-old female patient and was proven to harbor the EWSR1-ATF1 gene fusion. Immunohistochemically, this case was focally positive for CK7, CK14, CK19 and p63. The patient was referred to surgical treatment; however, she died of disease 2 months after the diagnosis, thereby demonstrating the aggressive nature of this tumor.

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Section: Discussionmentioning

confidence: 99%

Clear Cell Odontogenic Carcinoma Harboring the EWSR1–ATF1 Fusion Gene: Report of a Rare Case

Santana

Andrade

Melo

et al. 2019

Head and Neck Pathol

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“…Less than 120 cases have been reported in the literature. A recent review of the literature of 107 patients diagnosed with CCOC showed a predilection for females, the 5th decade of life, and the mandible [3]. In the same review, 13.6% (12/88) of CCOC patients manifested regional nodal involvement, and 11.2% (12/107) of CCOC patients died of disease [3].…”

Section: Discussionmentioning

confidence: 98%

“…A recent review of the literature of 107 patients diagnosed with CCOC showed a predilection for females, the 5th decade of life, and the mandible [3]. In the same review, 13.6% (12/88) of CCOC patients manifested regional nodal involvement, and 11.2% (12/107) of CCOC patients died of disease [3]. In this report, we present a case of CCOC in a 43-year-old female with progressive growing mass, 18 cm in size in its largest diameter, located in the mandible associated with regional lymph node involvement and alive without disease and no recurrence 15 months posttherapy.…”

Section: Discussionmentioning

confidence: 99%

“…However, due to its aggressive clinical characteristics such as invasive growth, regional lymph node involvement, local recurrence, and distant metastasis, the WHO reclassified it as a malignant tumor of odontogenic origin in 2005. CCOC has a preference for females in the 5th decade of life and for the mandible, with 73.8% of reported cases occurring in the mandible [3].…”

Section: Introductionmentioning

confidence: 99%

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Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations

Owosho

Okwuosa

Donald

et al. 2021

Case Reports in Dentistry

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Clear cell odontogenic carcinoma (CCOC) is a low-grade malignant neoplasm that affects the jaws. We report an 18 cm massive case of mandibular CCOC in a 43-year-old female. The tumor was composed of nests and cords of round to polygonal monomorphic clear cells separated by prominent stromal hyalinization. Immunohistochemically, the tumor cells showed focal cytokeratin 5/6 positivity and intracytoplasmic PAS-positive granules and were negative for S100 and after diastase treatment (PAS-D). Molecularly, this case was positive for EWSR1 rearrangement by FISH. The following should be included in the histopathological differential diagnosis: hyalinizing clear cell carcinoma of the salivary gland, clear cell variant of central mucoepidermoid carcinoma, clear cell variant of calcifying epithelial odontogenic tumor, and metastatic renal cell carcinoma. CCOC is a rare entity, with only 79 cases reported in the mandible. This case highlights the propensity for CCOC to exhibit invasiveness, destructive nature, and facial disfigurement if left untreated.

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“…Clear cell odontogenic carcinoma (CCOC) is a rare malignant odontogenic tumor histologically characterized by sheets and lobules of vacuolated and clear cells. To date, only 107 cases have been reported in literature since its first description by Hansen et al in 1985 [1]. Initially classified as a benign neoplasm with the tendency to local invasion., in 2005 the World Health Organization redefined CCOC as a malignant tumor of odontogenic origin, characterized by local recurrence and presence of nodal metastases.…”

Section: Introductionmentioning

confidence: 99%

Clear Cell Odontogenic Carcinoma of the Mandible: A Case Report

Nisi¹,

Izzetti²,

Gabriele³

2019

The XV National and III International Congress of the Italian Society of Oral Pathology and Medicine

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BackgroundClear cell odontogenic carcinoma (CCOC) is a rare malignant odontogenic tumor histologically characterized by sheets and lobules of vacuolated and clear cells. To date, only 107 cases have been reported in literature since its first description by Hansen et al. in 1985 [1]. Initially classified as a benign neoplasm with the tendency to local invasion., in 2005 the World Health Organization redefined CCOC as a malignant tumor of odontogenic origin, characterized by local recurrence and presence of nodal metastases. Due to its infrequency, diagnostic criteria, protocols, and prognosis of CCOC are often not fully understood [2,3]. Additionally, CCOC shares comparable clinical and pathological characteristics with other diseases, possibly leading to misdiagnosis [4]. Case PresentationA 64-year-old female patient was referred to the Unit of Dentistry and Oral Surgery, University of Pisa, for the evaluation of a gingival tumefaction in the anterior mandible. Considering medical history, the patient was affected by diabetes mellitus and hypertension, and was under pharmacologic treatment with Metformin, Levothyroxine, and Amlodipine.At clinical examination, the patient showed an ulcerated gingival mass localized in the right mandible, associated with grade III mobility of tooth 43 (Figure 1). Panoramic radiograph showed a multilocular mixed area involving the right side of the mandible (Figure 2). CT-scan showed an osteolytic lesion with bicortical bone destruction. Incisional biopsy was performed, and on the basis of the histopathologic examination a diagnosis of amelobastic carcinoma was made. Head and neck MR and total body PET-CT were performed to further investigate the lesion. Radical surgery included hemimandibulectomy and lymph node dissection, obtaining clear resection margins. A fibula osteoseptocutaneous flap was employed for the reconstruction of the post-surgical composite-tissue defect of the mandible. interestingly, second histology of the surgical specimen revealed a diagnosis of CCOC (positive immunodetection for anti-AE1/AE3, anti-CK19).

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Clear cell odontogenic carcinoma: a rare jaw tumor. A summary of 107 reported cases

Cited by 34 publications

References 72 publications

Clear Cell Odontogenic Carcinoma Harboring the EWSR1–ATF1 Fusion Gene: Report of a Rare Case

Clear Cell Odontogenic Carcinoma Harboring the EWSR1–ATF1 Fusion Gene: Report of a Rare Case

Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations

Clear Cell Odontogenic Carcinoma of the Mandible: A Case Report

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