Lymphoma is considered as cancer of the lymphoid tissue that exists in two main categories, including Hodgkin’s and non-Hodgkin’s lymphoma. Primary extranodal non-Hodgkin’s lymphoma of the mandibular condyle is extremely rarely reported. Comprehensive examination is required to establish more possible diagnosis for every patient. To learn more about this lesion, this case report of a patient with anaplastic large cell lymphoma occurring in the mandibular condyle has been presented. The pathological examination of this tumor included diffused atypical lymphocytic cells infiltrating, hyperchromatic nuclei and atypical mitosis. The patient has been treated chemotherapeutically by 6 cycles of EPOCH (Pirarubicin 20mg d1-2,10mg d3-4 and 151 Etoposide 50mg d1-4 and Vinorelbine 10mg d1-4 and Ifosfamide 1.5g d5 and 152 Prednisone 100mg d1-5) and has been followed up to 13 months. Considerable bone regeneration progressed through the follow up time. In conclusion, the primary extranodal non-hodgkin’s lymphoma of the mandibular condyle should not be excluded from the differential diagnosis and this disease can be treated by chemical therapy.
This case report which discussed rare case of Primary extranodal NHL of the mandibular condyle.
Primary extranodal NHL of the mandibular condyle should not be excluded from differential diagnosis of the TMJ disorder.
Primary extranodal NHL of the mandibular condyle can be treated by chemotherapy with good prognosis with follow up to 2 years.
Depending on the Reed-Sternberg cells, lymphoma of the lymphoid tissue is classified into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), 90% of lymphomas are NHL [ ]. The NHL can subgrouped to B and T-cell types, which in turn the T-cell type represents 25%. Anaplastic large cell lymphoma has been classified by the World Health Organization as a subtype of peripheral T-cell lymphoma [ , ]. Most of NHLs arise in lymph nodes, 25%–40% of patients with NHL present with extranodal involvement [ ]. With the gastrointestinal tract being the most commonly affected site followed by the head and neck region, subcutaneous tissue, and skin. In the oral cavity, NHL affects mostly the Waldeyer’s ring which involves the lymphoid tissue of the tonsils, the soft palate, the nasopharynx, and the base of the tongue, but also can affect buccal mucosa, the tongue, the floor of the mouth, the retromolar area, and the jaw bones [ ].
The majority of primary bone lymphomas are NHL [ ]. It was first described by Oberling in 1928 [ ], and further reported by Parker and Jackson 1939 [ ] in their series on primary reticulum cell sarcoma of bone [ , ]. The mechanism of the NHLs occurrence is not clear, the most common factors include immunodeficiency, autoimmune diseases, infections, exposure to noxious chemical agents, radiation chemotherapy and hereditary factors [ ]. Sometimes, the NHL can develop without any other related conditions. The clinical presentation of oral NHL varies from non-tender swellings to ulcerated masses, with varying degrees of mouth opening limitation and may mimic some benign or malignant lesions depending on their clinical characteristics such as parotid gland adenoid cystic carcinoma that can lead to limit opening mouth [ ].
The aim of this report is to describe a rare case of primary extranodal NHL of the mandibular condyle and to get more knowledge about this lesion.
A 19-year-old male patient was referred to our department for consultation of a swelling and painful lesion on his right cheek with mouth opening limitation that started 30 days prior to his visit. The patient did not reveal any medical or family history, and denied tobacco smoking and betel nut chewing. Upon clinical examination, there was facial asymmetry with swollen right side, extending below and above the zygomatic arch and to the anterior temporal region, inferiorly to the lower border of the mandible, and from the commissure of the mouth to the ear [ Fig. 1 A]. Examination of the lesion revealed normal color and temperature of the skin, mild tenderness on palpation, clicking on the right TMJ, and limited mouth opening about 2.5 cm [ Fig. 1 B]. The intercuspal position was normal [ Fig. 1 C], but the Bennett movement was found to be abnormal [ ] [ Fig. 1 D]. No palpable nodes were found.
Axial CT showed a large mass with diffused destruction in the right mandibular condyle involving surrounding muscles [ Fig. 3 A]. Lytic destruction was found at the center of the mandibular condyle. The cortical destruction and soft tissue swelling (lateral pterygoid, temporalis, masseter) were obvious in the right side of the face. MRI demonstrated bone marrow involvement and swelling of the surrounding soft tissues. The lesion showed diffused hyperintensity on T2-weighted imaging. The lateral pterygoid, temporalis, masseter and medial pterygoid muscles were obviously swollen with streaky shape of the surrounding soft tissue [ Fig. 3 B].
Small sample was taken by preauricular approach. During the surgical procedure, destructed bone with granulation tissue has been seen and the boundaries were not clear between bone and soft tissue. From the histological examination, the hematoxylin-eosin staining showed diffused atypical lymphocyte cells infiltrating bone tissue with hyperchromatic nuclei and atypical mitosis [ Fig. 4 A and B]. By the immunohistochemical and the extensive immunohistochemical staining the tumor cells stained positive for ALK T-cell markers, CD30, EMA, GB (part), TIA-1 (part) and negative for B-cell markers CD20, CD79a, CD5 and CD7. The Ki-67 proliferation index is >85% [ Fig. 4 C–F]. A panel of markers is decided based on morphological differential diagnosis (no single marker is specific). This case seems very interesting based on the synchronous positive expression of CD30 and partial expression of TIA-1). Recognition with the immunohistochemical staining pattern of the normal lymph node and the variety seen in lymphoma, and Perceiving the distinctive distribution pattern of the positive CD20 and CD3 lymphocytes between normal and lymphoma conditions are crucial to know the accurate determination of lymphoma. As well as, B cell lymphoma-2 (Bcl-2) positivity in the follicular focus must be assessed by the number and circulation pattern of germinal center T cells that is essential in differentiating follicular lymphoma from reactive proliferation [ ].