Sclerosing odontogenic carcinoma of maxilla: A case report

Table 1 Literature review of reported cases of sclerosing odontogenic carcinoma

Ref.	Gender, age (yr)	Site, symptoms	Duration	Radiological features	Treatment	Histopathological features			Outcome
						H&E	IMC
							Positive staining	Negative staining
Landwehr and Allen[2], 1996 (Koutlas et al[3], 2008)	F, 46	Pain in the right mandible	Not mentioned	Poorly defined osteolytic lesion with perforation of buccal plate and thinning of lingual plate	Wide resection with 1 cm margin	Islands of moderately pleomorphic neoplastic epithelial cells interspersed with dense fibrous connective tissue	CK5/6, CK19	Not mentioned	No recurrence after 12 yr
Koutlas and Warnock[8], 2005 (Koutlas et al[3], 2008)	M, 72	Left mandibular mass (33-35) protruding into vestibule with mental nerve paraesthesia	“Long” duration	Radiolucency affecting the lower left canine and premolar	Wide resection with ipsilateral neck dissection	Thin cords and small nests of epithelium in densely collagenized stroma with invasion of striated muscle and perineural infiltration	CK5/6, CK19, CK7 (focal), p63, E-cadherin	CK8/18, CK20, S-100, SMA, CEA, desmin	No recurrence after 5 yr
Chaisuparat et al[6], 2006 (Koutlas and Warnock[8], 2005; Koutlas et al[3], 2008)	F, 73	Enlargement of right maxilla	Not mentioned	Diffuse radiolucency involving alveolar ridge and extended into maxillary sinus	Wide resection with post-operative radiotherapy	Small nests and slender cords of epithelial cells in densely collagenized stroma with muscle and perineural infiltration	p63	Not mentioned	No recurrence after 3.5 yr
Ide et al[13], 2009	F, 47	2 cm mass on lower left lingual gingiva	2 yr	Unilocular radiolucency with sclerotic inferior border surrounding roots of mandibular left second premolar and first molar	Resection with neck dissection	Small islands of tumour cells reminiscing epithelial cell rests of Malassez infiltrating the cancellous bone	Not mentioned		No recurrence after 6 yr
Irié et al[12], 2010	M, 67	Paraesthesia in left mental region	Not mentioned	Focally expansile lesion with thinning of buccal cortical bone with admixed of radiolucent and radiopaque areas	First surgery: curettage; Second surgery: Segmental mandibulectomy with chemotherapy	Foci of thin cords and small nests of epithelial cells in fibrous stroma with epithelial cells invading into the mandibular canal	p63, CK6, CK19, CK7 (focal), AE1/AE3	S100, CEA, calretinin, CD34, vimentin, CK8, CK20, SMA, amelogenin, MIB-1 < 3%	Recurrence 8 mo after the first surgery.No recurrence after 15 mo of the second surgery
Hussain et al[11], 2013	M, 54	Sensitivity of upper right canine with a firm lump	Not mentioned	Well defined radiolucency associated with the upper right lateral incisor and canine teeth with loss of the lamina dura and irregular resorption of the canine root was seen	Resection with close follow-up	Small infiltrative islands in densely fibrous stroma with perineural infiltration	AE1-3, CK5/6, CK19	Not mentioned	No recurrence after 19 mo
Saxena et al[9], 2013	M, 42	Firm swelling at left mandibular lateral incisor to second premolars	11 mo	Well-defined unilocular lytic lesion and perforation of both buccal and lingual cortices	First surgery: excision; Second surgery: Hemimandibulectomy with radical neck dissection and radiotherapy	Cords and nests of tumour cells in dense fibrous sclerosing stroma with vascular invasion	CK5/6, P63	S100, SMA, Desmin	No recurrence after 10 mo
Tan et al[10], 2014	F, 31	1 cm hard swelling at lower right first molar region	Lower right first molar was extracted 10 yr ago	Well-circumscribed round radiolucent lesion with scattered specks of radiopacities with a distinct sclerotic peripheral margin	Enucleation	Small clusters neoplastic cells in diffusely sclerotic stroma	CK7, CK5/6, CK19, CK8/18, CAM 5.2, p63, p16 (weak), p53E-cadherin	Vimentin, CEA, EMA, CK20, SMA, S-100, CD1a, ER, PR, FISH EWSR 1, calretinin, CD34, desmin, Ki-67 < 2%	No recurrence after 1 yr
Wood et al[7], 2016 (Gordon et al[18], 2015)	F, 43	Asymptomatic firm lump at right anterior hard palate	Not mentioned	Enhancing soft tissue mass arising from the right hard palate with no bone destruction	Maxillectomy with wide margins and reconstruction with obturator	Small groups and prominent cords of bland hyperchromatic cells with minimal nuclear pleomorphism and eosinophilic cytoplasm	CK14, CK19, E-cadherin, weak nuclear staining to p63	S100, PR, FISH EWSR 1	Disease free after 17 mo
Hanisch et al[4], 2017	M, 60	Swelling at left premolar/molar region	Not mentioned	Ill-defined osteolytic changes with expansion, erosion, and perforation	Left hemimandibulectomy with ipsilateral radical neck dissectionSecondary reconstruction with CAD/CAM endoprosthesis (replacement of TMJ) and reconstruction with fibula flap	Small epithelial tumour cells and cords infiltrating lamellar bone	CK5/6, p40, p63, and MNF116	Not mentioned	Disease free after 22 mo
Todorovic et al[5], 2019	M, 62	Progressive left maxillary swelling with recurrent sinus infections and mobility of teeth	6 mo	Ground glass appearance with loss of trabeculations of left maxilla	Left maxillectomy and removal of skull base involving the infratemporal fossaUnderwent high-dose radiotherapy (66Gy in 33 fractions) for recurrence	Non-encapsulated tumour with mixed epithelial and mesenchymal components. Epithelial component consisted of highly infiltrative nests and cords of small polygonal and cuboidal cells with eosinophilic cytoplasm and mild-moderate nuclear atypia, usually associated with a dense background stroma. Significant intratumoral variability was observed	CK5/6, CK14, p63	CK7, CK19, CK20, EBER ISH, ER, PAX8, CDX2, FISH EWSR 1, Ki-67 10%	Recurrence at 5 mo after surgery;No recurrence 19 mo following radiotherapy
Seyiti et al[15], 2020	F, 54	Discomfort at left posterior mandibular region, associated with numbness of lower lip	3 mo	CBCT/SCT: irregular extensive osteolytic lesion with poorly defined borders and patchy calcifications were noted in the lesion. Slight resorption of cementum in apical region was seen. Obvious thickening of bilateral mandibular body was seen	Extensive resection and reconstruction with free fibula flap	Strands of epithelial tumor cells with clear cytoplasm infiltrating the fibrous stroma, osseous trabeculae and perineural invasion was observed	CK5/6, p63,	SMA, S-100, desmin, Ki-67 approx. 10%, EWSR1	Not mentioned
O’Connor et al[16], 2019	F, 43	Asymptomatic, incidental finding of radiolucency of right anterior maxilla	16 yr	Well-defined radiolucency with resorption of tooth roots and cortical thinning and erosion	First surgery: Biopsy; Second surgery: Conservative enucleation; Third surgery: Resection with a margin of 5 mm	Islands of epithelium within fibrous connective tissue that are mostly collagenous and sclerosed. Evidence of perineural invasion was seen	AE1/3, CK5, CK14, CK19	CK7, Ki-67 < 1%, FISH EWSR1	No recurrence 12 mo post-op
Kataoka et al[17], 2018	F, 68	Rapid, painless swelling of anterior mandibular region, with ulcerated overlying gingiva	3 mo	CT: Radiolucency around the root of central incisor, with resorption of labial cortex; no root resorption; MRI: Well-defined internal heterogenous and extraosseous mass	En-bloc resection of 4 incisors and alveolar bone preserving lingual periosteum	Eosinophilic polyhedral tumor cells scattered under epithelium. Dispersed tumor nests with circular patterns and pressed by sclerosing fibrous stroma. No perineural and vascular infiltration, or invasion of skeletal muscle	AE1/AE3, EMA, p63, CK19	CK5/6, Ki-67 approx. 2%, CK7	No recurrence or metastasis more than 5 yr after surgery
Present case	F, 62	Small indolent swelling at anterior palate, associated with intermittent toothache	7 yr	Well-defined sclerotic border of the right maxilla extending into the right maxillary sinus with significant root resorption was seen on upper right central and lateral incisors and upper right first molar	First surgery: Excisional biopsy; Second surgery: Right subtotal maxillectomy and reconstruction with free fibular flap	Small nests or cords of small epithelial cells, and occasionally clear cells, immersed in a diffuse sclerotic and collagenous stroma. The epithelial cells appeared to be faintly hyperchromatic and mitotic figures were uncommon. Perivascular and perineural infiltration were observed	CK7, CK19, p40, p63	Vimentin, Ki-67 5%-10%	Disease free after 22 mo

CT: Computed tomography; H&E: Hematoxylin and eosin; CK: Cytokeratin; F: Female; M: Male; IMC: Immunohistochemistry.