Gluteal Region Lymphoma presenting as Neurolymphomatosis
Corresponding Author: Vinay Maurya, Department of Radiology, Base Hospital Delhi Cantt, Delhi, India, Phone: +91 7875932426, e-mail: email@example.com
Received on: 26 December 2022; Accepted on: 18 April 2023; Published on: 28 June 2023
Lymphoma is a malignancy of mature lymphocytes arising from B or T cells which can present as a nodal or extranodal disease. Extranodal lymphoma involving the skeletal muscle is a rare event with an incidence of 1.5–8.3% of non-Hodgkin lymphoma (NHL) cases. In all the cases reported in the literature, the pathology was localized to a gluteal region with the involvement of bones in some cases. Intradural extension of gluteal lymphoma along the lumbar plexus has not been reported so far. We report here a case of a 64-year-old male who presented with an isolated history of swelling in the right gluteal region with associated progressive severe weakness of bilateral lower limbs and pain in lower back and thigh.
How to cite this article: Maurya V, Sharma S, Sharma P, et al. Gluteal Region Lymphoma presenting as Neurolymphomatosis. J Med Acad 2023;6(1):36-39.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.
Keywords: Gluteal, Lymphoma, Neurolymphomatosis
Lymphoma is a malignancy of mature lymphocytes arising from B or T cells which can present as a nodal or extranodal disease. Extranodal lymphoma involving the skeletal muscle is a rare event with an incidence of 1.5–8.3% of NHL cases.1,2 In all the cases reported in the literature, the pathology was localized to the gluteal region with the involvement of bones in some cases. Intradural extension of gluteal lymphoma along the lumbar plexus has not been reported so far. We report here a case of a 64-year-old male who presented with an isolated history of swelling in the right gluteal region with associated progressive severe weakness of bilateral lower limbs and pain in lower back and thigh.
A 64-year-old male patient, with no previous comorbidities presented with a history of pain in the right gluteal region. After a month he noticed swelling in the right gluteal region which increased progressively over 3–4 months. Thereafter, he noticed weakness in both lower limbs. His symptoms gradually progressed to the point that the patient had debilitating pain radiating from the lower back to the thighs and severe weakness which made the patient bedbound.
On examination, the patient had stable vitals. Local examination of the right gluteal region revealed a large, diffuse soft swelling extending into the upper thigh with poorly defined margins. The overlying skin was normal. Neurologic examination revealed a power of +1/5 in the right lower limb and +2/5 in the left lower limb. Bowel and bladder were unaffected. No sensory deficit was noted. Deep tendon jerks were normal. Plantar reflex was down going. The hematological, biochemical, and serological investigations were essentially within normal limits.
Magnetic resonance imaging (MRI) of the lumbosacral spine and pelvis was done to evaluate the gluteal swelling and the patient’s neurological symptoms. MRI revealed a large mass measuring 9.5 × 5 × 15 cm (anteroposterior × transverse × craniocaudal) in the right gluteal region which was heterogeneously hyperintense on T2 weighted image (T2WI)/short tau inversion recovery (STIR) and hypointense on T1WI and showed heterogeneous postcontrast enhancement. The lesion was seen extending into the pelvis through the greater sciatic foramen giving a dumbbell appearance (Fig. 1). The lesion was also seen extending along the lumbar nerve roots and through the L5-S1, S2, and S3 neural foramen into the dural sac (Fig. 2). The lesion was seen extending cranially up to D12-L1 level and was seen encasing the nerve roots of cauda equina, conus medullaris, and lower lumbar nerve roots causing obliteration of cerebrospinal fluid (CSF) space in the thecal sac (Fig. 3). A few lymph nodes were also noted along right common iliac vessels and in pelvis largest measuring 15 mm. Based on the above MRI findings a differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) or myxopapillary ependymoma with extradural extension was given.
To evaluate further patient was subjected to a trucut biopsy of the right gluteal mass. The histopathological examination (HPE) showed a monomorphic population of atypical lymphoid cells infiltrating the skeletal muscle bundles in diffuse sheets. These atypical lymphocytes are 3–4 times the size of normal mature lymphocytes and have hyperchromatic nuclei with scant to vacuolated cytoplasm. No RS/RS-like cells were seen. Immunohistochemistry (IHC) was positive for CD20, CD10, BCl6, CD43, and MUM1 and negative for CD99, CD56, CD38, and PanCK. Ki-67 index was 60–70% in most proliferative areas (Fig. 4). A final diagnosis of diffuse B-cell lymphoma—activated B-cell type was made.
Malignancy of lymphocytes or lymphoblasts results in lymphoma which accounts for approximately 4% of all cancers.3 Lymphoma commonly presents as a nodal disease, however, presentation as an extranodal disease is not uncommon. Hodgkin lymphoma and low-grade Non Hodgkin Lymphoma (NHL) typically present as nodal disease, whereas high-grade NHL usually presents as extranodal disease. Extranodal disease can affect any part of the body. The most commonly involved site is the gastrointestinal tract followed by the lung, central nervous system, liver, spleen, and bone.4 Lymphoma of skeletal muscle is rare and accounts for up to 1.4% of all lymphomas.5 Literature search revealed a few cases of lymphoma originating in gluteal muscles.2,6 However, we did not come across any case of gluteal muscle lymphoma infiltrating and extending along the lumbosacral nerve roots and presenting as an intradural mass. The peripheral nerve infiltration by lymphoma cells has been termed neurolymphomatosis (NL).
Neurolymphomatosis (NL) is an infrequent presentation of B-cell NHL. It is characterized by the direct infiltration of cranial nerves, plexus, nerve roots, or peripheral nerves with lymphoma cells. The patients have varied presentations depending on the nerves affected. Patients may present with painful or painless polyneuropathy, radiculopathy, or mononeuropathy depending on the extent or number of nerves involved. In this case, the patient initially presented with pain and swelling in the right gluteal region which progressed to weakness and debilitating pain along the back of thighs incapacitating the patient and making him bedbound. MRI is the initial investigation modality that can depict the involvement of the nerves. MRI was done in our case which revealed an enhancing soft tissue mass that was tracking along the nerves and through the L4-5 and L5-S1 neural foramen into the intradural compartment (Fig. 2). There was complete obliteration of dural space with myelography cut off upto D12 level (Fig. 3). In view of these findings a differential diagnosis of MPNST or myxopapillary ependymoma with extraspinal extension was given. Rationale for giving this differential was the perineural spread of a tumor. However, both these diagnoses were proved to be wrong on HPE.
The limited literature available describes three appearances of NL on MRI—(1) infiltrative mass displacing neural fascicles, (2) diffuse thickening of nerve and enhancement, and (3) thickening of individual nerve fascicles. None of the cases reported in the literature presented with large skeletal muscle mass infiltrating the peripheral nerve and extending into the spinal canal. MRI is the imaging modality of choice for evaluating nerves. It can demonstrate the focal or diffuse thickening of nerves or show enhancement of affected nerves on postcontrast imaging. The international primary central nervous system lymphoma collaborative group has stated that fluorodeoxyglucose-positron emission tomography (FDG-PET)/computed tomography (CT) might be more sensitive than MRI in the diagnosis of NL as they will show increased uptake in the affected nerves. In our case, FDG-PET/CT was not done as the gluteal lesion was accessible to trucut biopsy and the diagnosis of diffuse B-cell lymphoma was established on HPE.
Primary involvement of skeletal muscle in lymphoma is very rare, however skeletal muscle lymphoma presenting neurologically as NL has not been reported in the literature. It is important to keep lymphoma as a differential diagnosis if a mass is seen involving both intraspinal and extraspinal compartments.
2. Sonnino R, Kambouris AA. Malignant lymphoma presenting in gluteal muscles: case report and brief review of the literature. Henry Ford Hosp Med J 1988;36(1):61–63. https://scholarlycommons.henryford.com/hfhmedjournal/vol36/iss1/16
5. Komatsuda M, Nagao T, Arimori S. [An autopsy case of malignant lymphoma associated with remarkable infiltration in skeletal muscles (author’s transl)]. Rinsho Ketsueki 1981;22(6):891–895. PMID: 7334615.
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