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Year : 2014  |  Volume : 51  |  Issue : 4  |  Page : 475--476

Extra nodal diffuse large B cell lymphoma at a rare site: A case report

D Shanmugam1, NRV Prasad1, K Srinivasan1, D Basu2,  
1 Department of Surgery, JIPMER, Pondicherry, India
2 Department of Pathology, JIPMER, Pondicherry, India

Correspondence Address:
Dr. D Shanmugam
Department of Surgery, JIPMER, Pondicherry
India




How to cite this article:
Shanmugam D, Prasad N, Srinivasan K, Basu D. Extra nodal diffuse large B cell lymphoma at a rare site: A case report.Indian J Cancer 2014;51:475-476


How to cite this URL:
Shanmugam D, Prasad N, Srinivasan K, Basu D. Extra nodal diffuse large B cell lymphoma at a rare site: A case report. Indian J Cancer [serial online] 2014 [cited 2020 Sep 27 ];51:475-476
Available from: http://www.indianjcancer.com/text.asp?2014/51/4/475/175370


Full Text

Sir,

Diffuse large B cell lymphoma (DLBCL) is a rapidly growing, aggressive form of non-Hodgkin's lymphoma (NHL). It is the most common NHL, and about 40% are extra nodal. We report a case of extra nodal diffuse large B cell lymphoma in the thigh, a rare site of occurrence.

A 55-year-old male presented with pain and swelling in the left thigh, of 3 days duration. He gradually developed paraesthesia over the lateral aspect of left leg and dorsum of foot on admission, which rapidly progressed to hypoesthesia and then anesthesia, with weakness of the extensor group of muscles in the leg, resulted in foot drop. On examination, he had diffuse swelling in the posterior 2/3rd left lower thigh. There was tenderness and warmth over the swelling with surrounding induration. There was decreased sensation to pain and fine touch in the left sole, and the power of the plantar and dorsi flexors of the left ankle was 1/5 with absent ankle reflexes.

Initially, he was thought to have a deep muscle abscess. Ultrasonogram revealed a 10 × 10 cm sized heterogeneous collection in the intermuscular plane, suggestive of an abscess. Hence, a diagnostic needle aspiration was done, which was hemorrhagic and inconclusive. A repeat FNAC revealed a malignant round cell tumor. Contrast-enhanced CT scan revealed a large hypodense lesion in the posterior aspect of left thigh [Figure 1]. He underwent wide excision of the fusiform tumor encasing the sciatic nerve trunk [Figure 2] and [Figure 3]. The excised specimen was reported as extra nodal DLBCL, with positive for LCA and CD20 [Figure 4] and [Figure 5]. His revisedinternational prognostic index (IPI) score was 0/3, and the disease was found to be stage IAEX. Hence, he was given 6 cycles of chemotherapy (CHOP regimen) and local field radiotherapy. There was no loco-regional recurrence or distant spread of disease after one year of follow-up.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}

A retrospective review of 3556 cases of NHL revealed that nearly one-third of all cases were extra nodal in origin, giving a world standardized incidence of 1.9/105 persons per year.[1] The common sites of involvement were being the skin, stomach, and small intestine, with high-grade histologic types predominantly seen.[1] Glass et al. found that patients with NHL arising from lymph node sites tended to present with more advanced disease (55.8% with stage III or IV disease), whereas patients with primary extra nodal disease and non–lymph node sites presented at an earlier stage (64.7% and 74.0% with stage I and stage II disease, respectively).[2] DLBCL presenting in the thigh is very rare, and there is only one case reported by Bolke E et al., occurring in the right thigh.[3] DLBCL patients with low (0 or 1) IPI risk have a survival rate of 73%, whereas it is 26% when IPI score is high (4 or 5). All patients with diffuse large B cell lymphoma should be treated with a combination chemotherapy regimen. The most popular regimen is CHOP plus rituximab.

Primary skeletal muscle lymphoma is very rare, accounting for approximately 1.5% of NHLs. In a 15-year series, only 8 patients had primary skeletal muscle NHL, and it was common in the upper extremities and gluteis.[4] Primary lymphoma of the muscle has been associated with a poor prognosis and usually has diffuse large cell histologic features. There are few cases reported as primary skeletal lymphomas, one occurring in the right gastrocnemius, reported by Laura Bourdeanu et al.,[5] responded well for the chemoradiation. Another case, reported by Domenico Marotta et al.,[6] was an HIV-positive young patient with primary lymphoma arising from the right deltoid muscle, who also responded well, following surgical ablation and chemotherapy. Todd J. Alekshun et al. report a case with multiple skeletal muscle T cell Lymphoma detected by FDG-PET scan.[7] However, the majority of cases develop secondarily following hematogenous dissemination from nodal disease.

We conclude saying that though DLBCL is a common NHL, with extra nodal manifestions, it's unusual presentation and arising primarily from soft tissue of the right thigh has enlightened us, and the rare possibilities to be considered in such situations.

References

1Gurney KA, Cartwright RA. Increasing incidence and descriptive epidemiology of extranodal non-Hodgkin lymphoma in parts of England and Wales. Hematol J 2002;3:95-104.
2Glass AG, Karnell LH, Menck HR. The National Cancer Data Base report on non-Hodgkin's lymphoma. Cancer 1997;80:2311-20.
3Bölke E, Peiper M, Matuschek C, Schieren G, Glombick R, Förster C, et al. Extranodal diffuse non hodgkin lymphoma in the thigh. Eur J Med Res 2010;15:367-8.
4Samuel LM, White J, Lessells AM, Roddie H, Matheson LM. Primary non-Hodgkins lymphoma of muscle. Clin Oncol (R Coll Radiol) 1999;11:49-51.
5Bourdeanu L, Menon R, Somlo G. Diffuse Large B-Cell Lymphoma with Calf Muscle Localization. Case Report Hematology 2011;2011:292494.
6Marotta D, Sgambato A, Cerciello S, Magarelli N, Martini M, Larocca LM, et al. Soft tissue non-Hodgkin lymphoma of shoulder in a HIV patient: A report of a case and review of the literature. World J Surg Oncol 2008;6:111.
7Alekshun TJ, Rezania D, Ayala E, Cualing H, Sokol L. Skeletal Muscle Peripheral T-Cell Lymphoma. J Clin Oncol 2008;26:501-3.