Isolated involvement of the mandible by non-Hodgkin's lymphoma.
AK Sarda, R Kannan, A Gupta, V Mahajan, PK Jain, S Prasad, K Uma
Dept of Surgery, Maulana Azad Medical College, New Delhi, India., India
A K Sarda
Dept of Surgery, Maulana Azad Medical College, New Delhi, India.
A case report of isolated involvement of the mandible by non-Hodgkin lymphoma is presented. The patient presented with a non-healing ulcer following a tooth extraction. Biopsy revealed an undifferentiated cancer. Investigations failed to reveal any involvement of the organs. A hemimandibulectomy was performed followed by radiotherapy on receipt of the histopathological diagnosis of non-Hodgkin lymphoma. The patient is asymptomatic two and a half years after treatment.
|How to cite this article:|
Sarda A K, Kannan R, Gupta A, Mahajan V, Jain P K, Prasad S, Uma K. Isolated involvement of the mandible by non-Hodgkin's lymphoma. J Postgrad Med 1995;41:90-2
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Sarda A K, Kannan R, Gupta A, Mahajan V, Jain P K, Prasad S, Uma K. Isolated involvement of the mandible by non-Hodgkin's lymphoma. J Postgrad Med [serial online] 1995 [cited 2020 Jul 2 ];41:90-2
Available from: http://www.jpgmonline.com/text.asp?1995/41/3/90/482
While skeletal involvement in generalised lymphoma occurs in up to 25% of patients, only 5% of all primary extranodal lymphomas occur in bones. Primary malignant lymphomas of the bones constitute about 5% of all malignant bone tumours and 2.5% of all bone tumours. Primary malignant lymphomas of bone usually involve the long tubular bones and less frequently the fiat bones. There are only occasional reports of isolated involvement of the mandible by primary malignant lymphoma. Since the first report by Jackson and Parker in 1939, only 40 cases of primary lymphoma of the mandible were available in the literature upto 1988; only one more case has been reported since.
A 48-year-old female patient had her left lower second molar tooth extracted for a complaint of toothache. Two months later she presented to our hospital with a non-healing wound at the dental extraction site. She had no other complaints. On examination, a 2 x 3 cm ulcer was present over the gingiva in the region of the extracted tooth. Both the tables of the mandible were expanded giving an external appearance of a swelling about 4 x 5 cm in diameter in the region over the left angle of the mandible. There was no altered sensation over the chin. The left temporomandibular joint was normal. There were no palpable cervical lymph nodes.
A biopsy of the ulcer was reported as a poorly differentiated malignancy suggesting a secondary deposit. On investigation she was found to have a haemoglobin of 9 gm/dl, PCV of 32%, a total leucocyte count of 8800 /cmm and a differential leucocyte count of 68% polymorph, 24% lymphocytes, 3% eosinophils, and 5% monocytes, a platelet count of 120,000 /cmm and a peripheral blood smear which did not reveal any abnormality. Her liver function tests were within normal limits. Radio-logic examination of the mandible showed a solitary lytic lesion in the body of the mandible near the left angle [Figure:1]; otherwise her skeletal survey was normal. A bone marrow biopsy, an ultrasound of the abdomen and a whole body CT scanning did not reveal any abnormality.
With a provisional diagnosis of metastatic deposit from an unknown primary, a hemimandibulectomy preserving the symphysis menti was performed and the tumour, which was infiltrating into the adjacent soft tissues, was removed. A primary reconstruction of the mandible was not carried out.
On gross examination, the tumour had greyish white and fleshy appearance. Histopathological examination of the bone and subepithelial soft tissue showed a dense infiltrate of predominantly large size round to polygonal cells with large vesicular nuclei and inconspicuous or solitary nucleoli admixed with a small number of intermediate size cells [Figure:2]. A few cells showed cleaving or indentations. Karyorrhexis, high mitotic rate and areas of vascular proliferation were also seen. The diagnosis was a non-Hodgkin lymphoma large cell type (intermediate grade).
The patient was also administered radiotherapy and the patient remains asymptomatic two and a half years after completion of her treatment.
Primary lymphoma of the mandible commonly occurs between 20 and 50 years of age and like other primary lymphomas of the bones, shows a male preponderance with a male to female ratio of 3:2,. Common presentations of these tumours include body swelling and bone pain, anaesthesia or paraesthesia of the lower lip and chin, resorption of teeth, loosening of teeth, and trismus,,. Pathological fractures may occur rarely,. It is not uncommon for these tumours to present as acute inflammatory swelling,,. It is thus not unusual to have a dental extraction or root canal treated or other local procedures carried out before a diagnosis of malignancy is suspected.
The commonest site of involvement of the mandible is the posterior body near the ramus where a large amount of medullary bone is found. However, the radiological features are not characteristic for primary lymphoma of the mandible. Radiolucent mottling mimicking osteomyelitis, lytic lesions with subperiosteal new bone formation resembling Ewing's sarcoma, a combination of lytic and osteoblastic findings along with cortical destruction and a "sun burst" appearance suggesting osteogenic sarcoma, and radiolucent juxtaepiphyseal bands as in leukaemias have all, been reported in relation to this disease,,,.
Since involvement of the mandible is more common with generalised lymphomas, the following criteria applicable to other lymphomas are also valid for establishing a primary, isolated disease of the mandible: (i) Histo-logical pattern of the specimen from the bone (not from other tissues) must be that of a lymphoma; (ii) Primary focus should be in a single bone on admission; (iii) Metastasis on admission, if present, should be localised only in the region of the lymph nodes; (iv) There should be a six months interval between onset of the symptoms of the primary focus and appearance of distant metastases; and (v) Generalised organ symptoms or palpable abnormal distant lymph nodes suggesting lymphoma should not appear before symptoms in a single bone. In this regard, although the value of staging is not established for primary lymphomas of extranodal sites, staging procedures serve to confirm the diagnosis of primary lymphoma of the mandible since no tumour is found elsewhere. Besides the routine clinical and biochemical assessments advocated for this disorder, staging procedures should include bone marrow biopsy, ultrasound of the abdomen. CIT scanning of the chest and abdomen, and a bone scan: a staging laparotomy may also be needed. Sometimes, despite all these procedures, it is impossible to determine whether the bone involvement is secondary to an adjacent soft tissue lymphoma or vice versa when there is both bond and soft tissue involvement.
Primary lymphoma of the mandible is a potentially curable lesion in contrast to secondary bone involvement by lymphomas which are invariably fatal. Treatment consists of radiotherapy, chemotherapy and surgery in various combinations. Since radio logical findings do not correlate with the true extent of the lesion and since there is a wide extension of the tumour locally radiotherapy should include the whole mandible and the regional lymph nodes and may be curative in early lesions. Chemotherapy plays a significant role in achieving a cure in patients with histopathology suggesting an aggressive behaviour; it is also used for palliation in extensive disease and as an adjunct to radiotherapy. Radical surgery is no longer acceptable as the first modality of treatment and is preferred for post radiation recurrences or for complications precluding radiotherapy.
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