Mantle cell lymphoma (MCL) is a subtype of B-cell non-Hodgkin lymphoma. Heterogeneous and extensive lymphadenopathy is the most common clinical manifestation. Although skeletal involvement is not uncommon in other types of non-Hodgkin lymphoma, primary bone MCL is rare. The present study reported a case of primary tibia MCL in a 50-year-old male presenting with left tibia pain and a rapidly growing lump. Computed tomography and magnetic resonance imaging scans revealed a progressive lesion in the cortical bone and surrounding soft tissue mass. A positron emission computed tomography scan demonstrated increased glucose metabolism in the middle tibia without involvement of regional lymph nodes. An aspiration biopsy was performed, and pathological examination revealed small-medium sized cells strongly positive for cluster of differentiation (CD)5, CD20 and cyclin D1. Fluorescent
Mantle cell lymphoma (MCL) is a distinct entity of B-cell non-Hodgkin lymphoma, accounting for 6–9% of malignant lymphoma cases (
A 50-year-old male presented with left tibia pain with no apparent cause, at Fenghua District People's Hospital (Ningbo, China) in September, 2014. An X-ray revealed that local bone density was decreased. Computed tomography (CT) revealed local cortical bone rupture with abnormal density in the medullary cavity and surrounding soft tissue (
Initial blood tests identified a lactate dehydrogenase level of 646 U/l, β2-microglobulin level of 2388 ng/ml, C-reactive protein level of 4.7 mg/l, white cell count of 5,200 cells/µl, hemoglobin content of 15.0 g/l and a platelet count of 169,000 platelets/µl. Screening tests for human immunodeficiency virus, cytomegalovirus, EB virus, and hepatitis B and C were negative.
CT (
Following a thorough examination, bulky stage IE was diagnosed according to the Ann Arbor staging system (
Generally, primary bone lymphoma is considered to consist of a single bone lesion with or without regional lymphadenopathies (
Diagnosis of MCL should be based on pathological examination of surgical specimens, preferably lymph nodes. The most characteristic morphological feature is small- or medium-sized lymphocytes with irregular nuclei. In addition, immunohistochemistry for the detection of typical patterns of an immunophenotype is mandatory. In the current case, the cells were positive for cyclin D1, CD5 and CD20, but negative for CD10. Additionally, FISH analysis detected IGH/CCND1 gene fusion and consequently MCL was diagnosed.
MIPI, Ki-67 index and bulky mass are effective markers in evaluating patient prognosis in MCL (
To date, the initial regimen for younger patients with MCL remains controversial. A phase II study from the Groupe d'Etudes des Lymphomes de l'Adulte suggested that CHOP and DHAP plus rituximab were safe and effective (
Generally, MCL is an incurable disease; nonetheless, early detection and treatment is essential to improve its management. The present case report confirmed the possibility of primary bone MCL with comprehensive and detailed clinical data. Nevertheless, the level of evidence supporting definitive methods of diagnosis and treatment of MCL remains low, and further studies are required to confirm the appropriate methods of detection and management, and to improve prognosis.
Not applicable.
The present study was supported by Zhejiang Natural Science Foundation (grant no. LY16H160005).
The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.
SC collected the data and wrote the manuscript. MY contributed to the conception of the study, revised the manuscript and helped perform the analysis with constructive discussions.
This case report was approved by The Ethics Committee of Ningbo University. Consent for publication was obtained from the patient.
Consent was obtained from all participants in the present study.
All authors declare that they have no competing interests.
Radiological data prior to treatment. (A) CT demonstrated a small cortical bone defect with abnormal density shadows inside the medullary cavity with surrounding soft tissue swelling in September 2014. (B) At 1 month later, the bone lesion and soft tissue mass were visibly aggravated. Magnetic resonance imaging scans of the (C) horizontal and (D) coronal axes revealed longT1 and T2 signal intensities within bony marrow and mass around the left tibia in September 2014. (E) At the time of admission, positron emission tomography-CT demonstrated increased glucose metabolism in the middle of the left tibia (max SUV, 17) and left adrenal gland (max SUV, 7.6). CT, computed tomography; SUV, standardized uptake value.
Histological and immunohistochemical staining (magnification, ×400). (A) Hematoxylin and eosin staining demonstrated abnormal, diffuse, atypical, small-sized, slightly irregular cells. Immunohistochemical staining revealed that tumor cells were positive for (B) cluster of differentiation 20 and (C) cyclin D1. (D) Ki-67 was expressed in 80–90% of the cells.
Initial treatment schedule. Thee cycles of R-CHOP (rituximab 375 mg/m2 mg on day 1, cyclophosphamide 700 mg/m2 on day 2, vindesine 250 mg/m2 on day 2, epirubicin 60 mg/m2 on day 2, prednisone 50 mg every 12 h on days 2–6) every 15 days and three cycles of R-DHAP (375 mg/m2 on day 1, cisplatin 100 mg/m2 on day 2, cytarabine 200 mg/m2 every 12 h on day 3, dexamethasone 40 mg on days 2–5) every 21 days were followed by ASCT. Then, rituximab at a dose of 375 mg/m2 was administered every 3 months for maintenance therapy. ASCT, autologous stem cell transplantation.
Radiological findings after treatment. (A) Horizontal and (B) coronal magnetic resonance imaging revealed that the range of abnormal signaling was notably reduced in April 2015. (C) positron emission tomography-CT demonstrated a slightly high-density shadow in the tibia with normal glucose metabolism following chemotherapy (max SUV, 4.8). SUV, standardized uptake value.