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


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