Apollo Cancer Centre, Plot no, 251, Sainik School Rd, Bhubaneswar, Odisha

Clear cell odontogenic carcinoma of maxilla : a rare entity

  • MRS. X a 55 year old female
  • Evaluated at outside hospital with c/o swelling over right side of nasal region for 1 month duration
  • Investigations
    • CT PNS: 3×2.8×3,4cm lytic soft tissue lesion in right aspect of maxillary alveolus involving periapical region of 1st four teeth and midline alveolus
    • Superiorly the lesion is eroding the bony floor and anterior wall of right maxillary sinus
    • Also extending into right nasal cavity
  • Biopsy from right maxilla : clear cell odontogenic carcinoma
  • Metastatic work up were normal
  • She underwent Anterior right maxillectomy with reconstruction
  • HPR : Tumor composed of nests and cords of pleomorphic cells with mild anisonucleosis and moderate to dense abundant clear cytoplasm, PNI Positive, margins negative
    • Clear cell carcinoma, NOS type of minor salivary gland
    • Clear cell odontogenic carcinoma

a) large lobules of clear cells separated by fibrous septae (H&E stain, ×100) (b) Clear cells with cellular and nuclear pleomorphism, hyperchromatism and few mitotic figures (H&E stain, ×400)

    IHC: P63, CD56: Focally positive

    • pan CK positive
    • Clear cell odontongenic carcinoma

  • In the present case there being PNI Positive : adjuvant RT is indicated and she received a dose of 63.6Gy / 30#
  • Introduction
    Odontogenic carcinomas – malignant epithelial odontogenic counterparts of 2005 WHO classification of odontogenic tumors

    Pathological classsification of odontogenic tumours:

    • Metastasizing (malignant) ameloblastoma
    • Ameloblastic carcinoma
    • Primary intraosseous squamous cell carcinoma
    • Solid type
    • Derived from keratocystic odontogenic tumor
    • Derived from odontogenic cysts
    • Clear cell odontogenic carcinoma
    • Ghost cell odontogenic carcinoma

    Clear cell odontogenic carcinoma (CCOC)

    • A rare neoplasm of the jaws : first described by Hansen et al in 1985
    • Defined it as a benign neoplasm with a capacity for locally invasive growth
    • Due to its behavior as an infiltrative neoplasm with a marked tendency for local recurrence, regional lymph node metastasis and possible distant pulmonary metastasis, WHO classification of 2005, CCOC was denoted as a malignant tumor of odontogenic origin.
    • Rare, < 100 cases have been reported
    • Most common in 5th to 6th decades
    • More common in females
    • Mandible most common site (75%)
    • Soft tissue involvement common as lesion often perforates through the bone
    • The tumor cells resemble clear cell rests of primitive dental lamina
    • Often presents as jaw swelling with loosening of the teeth
    • Can be painful, asymptomatic or associated with paresthesias
    • Recurrence rate of 30% of resected and 87% of curetted / enucleated lesions
    • Metastases to lymph nodes, lung and bone
    • Up to 25% die of disease
    • The aggressiveness of these neoplasms are documented as extensive invasion of adjacent tissues, regional metastasis to the lymph nodes, less frequent distant metastasis to the lungs, and a recurrence rate of 55%
    • Various therapeutic approaches have been applied by the surgeons over the years including Curettage, Enucleation, En bloc resection, Subtotal mandibulectomy/maxillectomy.
    • 80% recurred within 2 year and/or developed metastasis where the primary treatment was curettage or enucleation,
    • Hence wide local resection with partial mandibulectomy/maxillectomy with clear margins was the treatment of choice
    • A recent literature review suggests that CCOC has potential for multiple recurrences (41%), metastasis (31%), thus demanding an aggressive treatment approach and long-term surveillance.
    • Surgical resection with wide margins is the treatment of choice for CCOC, with adjuvant radiotherapy for cases showing perivascular and perineural invasion
    • Treatment protocol included lymph node resection and radical surgery if the nodes were positive
    • Adjuvant chemotherapy and/or radiotherapy for those with tumor positive margins and/or regional/neural/vascular invasion
    • Adjuvant radiotherapy to a dose of 6000 cGy at 200 cGy per fraction delivered once a day

    References:

    • Maiorano E, Altini M, Viale G, Piattelli A, Favia G, et al. (2001) Clear Cell Odontogenic Carcinoma: Report of Two Cases and Review of the Literature. Am J Clin Pathol 116: 107-114.
    • Panda S, Sahoo RS, Srivastav G, Padhiary S, Dhull KS, et al. (2014) Pathogenesis and Nomenclature of Odontogenic Carcinomas: Revisited. J Oncol 2014: 197425.
    • Eversole LR, Belton CM, Hansen LS (1985) Clear-Cell Odontogenic Carcinoma: A New Case and Long-Term Follow-Up of an Old Case, and Review of the Literature. J Oral Pathol 14: 603-614.
    • Bilodeau EA, Hoschar AP, Barnes EL, Hunt JL, Seethala RR, et al. (2011) Clear Cell Carcinoma and Clear Cell Odontogenic Carcinoma: a Comparative Clinicopathologic and Immunohistochemical Study. Head Neck Pathol 5: 101-107.
      Waldron CA, Small IA, Silverman H (1985) Clear cell ameloblastoma–an odontogenic carcinoma. J Oral Maxillo fac Surg 43: 707-717.
    • Xavier FA, Rodini CO, Ramalho LP, Sarmento VA, Nunes FD, et al. (2008) Clear cell odontogenic carcinoma: case report with immunohistochemical findings adding support to the challenging diagnosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 106: 403-410.
    • Loyola AM, Cardoso SV, De Faria PR, Servato JS, De Paulo LB, et al. (2015) Clear cell odontogenic carcinoma: report of 7 new cases and systematic review of the current knowledge. Oral Surg Oral Med Oral Pathol Oral Radiol 120: 483-496.
    • Chera BS, Villaret DB, Orlando CA, Mendenhall WM (2008) Clear cell odontogenic carcinoma of the maxilla: a case report and literature review. Am J Otolaryngol Head Neck Med Surg 29: 284-290.
    • Li T, Yu S, Gao Y, Wang E (2001) Clear Cell Odontogenic Carcinoma: A Clinicopathologic and Immunocytochemical Study of 5 Cases. Arch Pathol Lab Med 125: 1566-1571.
    • Richardson MS, Muller S (2014) Malignant Odontogenic Tumors: An Update on Selected Tumors. Head Neck Pathol 8: 411-420.
    • Werle H, Blake FAS, Reichelt U, Schmelzle R, Heiland M, et al. (2009) Clear-Cell Odontogenic Carcinoma: A New Case and Long-Term Follow-Up of an Old Case, and Review of the Literature. J Oral Maxillo fac Surg 67: 1342-1348.