原文題目(出處)： Extranodal non-Hodgkin’s lymphoma in the malar region-a case report. Oral Surgery 2012;5:22–5
原文作者姓名： V Naveen Shankar, NS Ashwini, G Kumar
通訊作者學校： Kothiwal Dental College Research Centre and Hospital, Department of Oral Medicine and Radiology,Moradabad, Uttar Pradesh, India
報告者姓名(組別)： 張克喬 Intern H組
Lymphomas account for 4–5% of all neoplasm.
Extranodal involvement in Hodgkin’s lymphoma are rare, but its frequency accounts for 25% or more in patients with non-Hodgkin’s lymphoma (NHL).
The head and neck region has the highest regional incidence of (NHL)
NHL generally responds to most modalities of treatment, including : 1. radiation therapy
2. single agent or combination chemotherapy 3. immunotherapy
4. radioimmunoconjugate therapy.
Recent studies have shown that radiotherapy is no more used in diffuse large B-cell lymphoma (DLBCL)
General data: 51-year-old man
Chief complaint: asymptomatic swelling in the right middle third of the face for 5 months.
Present illness: Initially, the swelling was of peanut size, which was slowly growing. He had undergone biopsy for the same condition, and the report was suggestive of a non-specific granuloma. Following the biopsy, the swelling grew rapidly to the present size. He gave no history of weight loss, fever, night sweats or sinus disease.
Past medical and dental histories: not significant.
a solitary, ovoid, ill-defined swelling over the right malar region
size: 5 x 4 cm (Fig. 1)
anterior-posteriorly: extending from lateral border of the nose to 2 cm ahead of tragus
superior-inferiorly : from 1 cm above the supra-orbital ridge to the corner of the mouth.
Skin over the swelling : taut with no secondary changes
Palpation pain: (+)the swelling was mildly tender with well-defined borders.
consistency : firm to hard, fixed to the underlying structures
Neither mobile nor reducible
Multiple ipsilateral parotid lymph nodes were palpable, mobile, firm in
consistency and non-tender.
Intraorally: no sign of the swelling was noted
Salivary flow was normal.
Eyelid movements: restricted
Facial nerve function: normal
Elsewhere in body:
abnormal swelling (-),
periapical radiograph over 14, 15, 16:
haziness in the right maxillary antrum
Waters’ view revealed: an illdefined radiopaque mass superimposed over the right malar region
Incisional biopsy was performed extraorally, H-P report:
‘Slit like’ vascular
spaces surrounded by endothelial cells with few mitotic figures and nuclear atypia were seen, which were suggestive of an intermediate vascular lesion.
The lesion was surgically excised using Weber–Ferguson’s incision.
Specimen: showed monotonous sheets of round cells with scanty cytoplasm and dense course nucleus. Nuclear atypia was seen, suggestive of a round cell tumour.
Immunohistochemical assay was carried out, which demonstrated CD20 (+) and CD3 (-) :
suggested of a tumour of B-lymphocyte origin.
diagnosis : extranodal NHL (DLBCL) , staged as II–E. (Ann Arbor).
Chemotherapy was given with
Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone (CHOP) regime, that is, each cycle is repeated every 3 weeks for 6–8 cycles, and patient is on regular follow-up.
CHOP is the acronym for a chemotherapy regimen used in the treatment of non-Hodgkin lymphoma. CHOP consists of:
Cyclophosphamide, an alkylating agent which damages DNA by binding to it and causing cross-links
Hydroxydaunorubicin (also called doxorubicin or Adriamycin), an intercalating agent which damages DNA by inserting itself between DNA bases
Oncovin (vincristine), which prevents cells from duplicating by binding to
Origin: NHL can either be of B-lymphocyte or T-lymphocyte origin
NHL accounts for 5% of new cancers in men
4% of new cancers in women
5% of deaths due to cancer each year
Incidence increases with age and peaks in individuals aged 80–90 years.
Men > Women: NHL is more common in men than women.
USA > Asian: The incidence is highest in white people in the USA and lowest in Asia.
Etiology : The cause of NHL is unknown. However, immunodeficiency and some infectious agents like Epstein-Barr Virus (EBV) and Human T-Lymphotropic Virus-1 (HTLV-I) have been suggested in the aetiology.
Predisposing factors that may be related to the development of NHL are:
exposure to drugs or infectious agents
history of Sjogren’s syndrome,
Hashimoto’s thyroiditis in association with thyroid lymphoma.
The most common site involved in the orofacial region is：
1. the tonsil (34%)
2. salivary glands (15%), 3. thyroid (14%)
4. sinus/nasal cavity (13%).
5. tongue, palate and orbit
Most common presenting feature : 1. a mass or a swelling (59%) 2. minimal symptoms: pain (21%) 3. dysphasia (7.9%)
4. weight loss (1.1%) 5. night sweats(0.5%) 6. nasal obstruction (7.9).
In our case, there was mild tenderness noted.
In our case, the lesion was stage II E and was diffused B-cell type
B-cell lineage cases show pan CD20 positivity : In this case CD20 (+)
The expression of individual antigens is related to different stages of B-cell differentiation : including
may help define groups of tumours with different clinical and pathological characteristics.
1. diffuse mixed small/large cell lymphoma 2. small lymphocytic lymphoma
3. extranodal natural killer (NK)/T-cell lymphoma: is the most common lymphoma involving the sinonasal region.
1 Which statement is wrong about Non-Hodgdin’s Lymphoma(NHL) ? (A) NHL most commonly originates from B-lymphocyte series.
(B) EBV(Epstein-Barr virus) may be the etiology of NHL (C) In the Korea and Japan, half of all lymphoma are extranodal (D) Clinical features is nontender mass that rapid swelling 答案(D) 出處：oral and maxillafacial pathology(second edition) p.519
2 Which statement about the classification of NHL is wrong?
(A) Lymphoma can be broadly grouped into three categories: low grade, intermediate grade, high grade
(B) The categories correlated with the increasing degree of aggressiveness
(C) Small lymphocytic lymphoma was classified as low grade
(D) Diffuse mixed/large cell lymphoma was classified as high grade lymphoma
答案(D) 出處：oral and maxillafacial pathology(second edition) p.518 table 13-3