Mandible-Maxilla
Malignant tumors
Lymphoma

Author: Anthony Martinez, M.D. (see Authors page)
Editor: Kelly R. Magliocca, M.D.

Revised: 13 March 2016, last major update March 2016

Copyright: (c) 2004-2016, PathologyOutlines.com, Inc.

PubMed Search: Lymphoma [title] mandible maxilla
Definition / General
  • More commonly non-Hodgkin lymphoma (NHL) (B or T cell); also Hodgkin lymphoma
  • Primary extranodal lymphomas of the mandible and maxilla are rare
Terminology
  • Also called intrabony lymphoma or primary osseous lymphoma at this site
Epidemiology
  • #2 most common malignant tumor of head and neck after squamous cell carcinoma (3.5% of intra-oral malignancies)
  • Mean age for extranodal lymphoma: 5th decade
  • Male to female ratio for lymphoma is 1.4:1
  • Approximately 16% of NHLs involve the jaw bones
  • 2/3 are diffuse large B cell lymphoma (DLBCL)
  • Plasmablastic lymphoma, a subtype of DLBCL, more common in the oral cavity of immunocompromised / HIV patients, can also involve the jaw, and can occur in elderly immunocompetent patients
  • Burkitt lymphoma and plasma cell neoplasms such as multiple myeloma (MM) can also involve the jaw bones
  • Sites
    • Most head and neck lymphomas involve Waldeyer's ring
    • ~1/3 of NHLs involve Waldeyer's ring
    • The mandible is the most common site for osseous lymphomas followed by the maxilla
    Clinical Features
    • Clinically, patients can present with tooth mobility, swelling, pain or a mass
    • Lymphadenopathy and B symptoms (fever, night sweats, weight loss, anorexia, fatigue, etc) can also be present
    • May also present as numb chin syndrome (NCS): sensory neuropathy characterized by unilateral hypoesthesia or paresthesia over the lower lip, chin and occasionally gingival mucosa
      • Most common non-hematologic cause is breast cancer
      • Most common hematologic cause is NHL
    Diagnosis
    • May be challenging because there is usually a low index of suspicion for lymphoma
    • Usually diagnosed at biopsy with flow cytometry, immunohistochemistry and molecular studies to help subclassify the lesion and to help risk stratify the patient
    Prognostic Factors
      Diffuse large B cell lymphoma
      • Favorable: limited disease (no or <10% marrow involvement), favorable international prognostic index
      • Favorable: germinal center gene expression (bcl6+, CD10+, negative for MUM1/IRF4, negative for CD138) versus activated B cell-like profile (Am J Surg Pathol 2004;28:464)
      • Unfavorable: p53 mutations present
      • Unfavorable: CD10+ and bcl2+ coexpression (Am J Clin Path 2001;116:183)

      Plasmablastic lymphoma
      • Clinical course is very aggressive with most patients dying in the first year after diagnosis
      • Outcome may have improved recently due to better management of HIV

      Burkitt lymphoma
      • In endemic and sporadic cases, the tumor is highly aggressive but potentially curable
      • Poor prognosis if bone marrow involvement, unresected tumor >10 cm and high serum LDH

      Multiple Myeloma (MM)
      • Poor prognosis - 1 year survival if multiple lesions and no treatment, but many years if indolent
    Radiology Images

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    Panoramic

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    Primary NHL

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    Axial and coronal section

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    Coronal CT

    Case Reports
    Treatment
    • Managed by chemotherapy, radiotherapy and surgery in various combinations
    Clinical Images

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    Intra-oral

    Micro Description
      Diffuse large B cell lymphoma
      • Monotonous population of large to medium sized lymphoid cells with the morphologic features of centroblasts (large noncleaved cells), immunoblasts (round nuclei with vesicular chromatin and single large nucleolus) or cells with intermediate features

      Plasmablastic lymphoma
      • Subtype of DLBCL most commonly associated with HIV that has predilection for oral cavity
      • Characterized by diffuse infiltrate of large lymphoma cells that can also have a “starry sky” pattern like Burkitt lymphoma
      • The cells can be plasmacytoid with eccentric nuclei, a single, centrally located prominent nucleoli, abundant basophilic cytoplasm and a paranuclear hof

      Burkitt lymphoma (BL)
      • "Starry sky" appearance composed of monotonous, medium sized lymphocytes
      • The cell size approximates the nuclear size of macrophages
      • A so called "squaring off" of the cytoplasm may be encountered, as the cell borders appear to abut one another

      Multiple myeloma
      • Sheets and aggregates of plasma cells that have abundant basophilic cytoplasm, low N/C ratio, eccentrically placed nucleus with clumped "spoke wheel" or "clock face" chromatin
      • The less mature plasma cells show loose reticular chromatin, prominent nucleoli, high N/C ratio
      • Mott cells (blue grapelike cytoplasmic inclusions), Russell bodies (cytoplasmic, cherry red, refractive round bodies), Dutcher bodies (intranuclear crystalline rods) can also be present
    Micro Images

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    "Starry sky"



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    Malignant lymphocytes

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    DLBCL: CD20, CD10, BCL6, CD3

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    Plasmablastic: CD20, CD138

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    Myeloma plasma cells

    Positive Stains
    Negative Stains
    Flow Cytometry Description
      Diffuse large B cell lymphoma: clonally rearranged immunoglobulin genes, BCL2 rearranged in ~20%, BCL6 rearranged in ~30%, BCL6 mutated in ~70%, MYC rearranged in <10%
      Plasmablastic lymphoma: clonal IgH chain gene rearrangement
      Burkitt lymphoma: IG-MYC fusions of either t(8;14)(q24;q32), t(2;8)(p12;q24), or t(8;22)(q24;q11); t(8;14) most common
      Myeloma
      • del 17p, del 13 or 13q; t(4;14)(p16.3;q32) in 25% of cases, causing increased expression of FGFR3 (fibroblast growth factor receptor 3) and IgH
      • t(11;14)(q13;q32) [cyclin D1] is usually part of complex karyotype
        • May be missed by routine cytogenetics, particularly if the proliferative rate is low (Am J Clin Pathol 2000;113:831)
        • Hyperdiploidy and hypoploidy
    Differential Diagnosis
    • Because there is usually a low index of suspicion, the differential is broad, mainly based on radiologic findings, and includes:
    • Odontogenic cysts
    • Benign and malignant odontogenic tumors
    • Reactive / inflammatory processes related to odontogenic infection or inflammation
    • Other metastases