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We present the case of a man in his 40s who initially presented with severe leg pain. A complete blood examination revealed an elevated white blood cell (WBC) count of 96.6 ×10ˆ9/L, with a differential leucocyte count (DLC) showing 3% neutrophils and 95% lymphocytes. Additional findings included an elevated lactate dehydrogenase (LDH) level of 550 U/L (reference range=235–470 U/L). Peripheral blood film analysis revealed markedly elevated WBCs, predominantly composed of immature and mature lymphocytes (95%), along with the presence of Smudge cells. Further investigations, including sonographic imaging, indicated hepatomegaly with hypoechoic space occupying lesions, moderate splenomegaly, and widespread bulky retroperitoneal lymphadenopathy.
A bone marrow examination confirmed the infiltration of intermediate-sized tumour cells with monomorphic features, including irregular nuclear contour, hyperchromatic to coarse chromatin, inconspicuous nucleoli, and scant cytoplasm. Immunohistochemistry (IHC) revealed positivity for CD20, Cyclin D1, CD3, CD5, SOX11, MUM1, and Ki67 proliferation index of 80%. These findings led to a diagnosis of classical variant mantle cell lymphoma (MCL), stage IV, with a high-risk MIPI score of 11.3.
Standard chemotherapy consisting of 6 cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) and R-DHAP (rituximab, dexamethasone, cytarabine, cisplatin) was administered to the patient. Autologous stem cell transplantation was recommended but declined by the patient, who opted for rituximab maintenance therapy instead. However, the patient experienced a relapse after 2 months, presenting with B symptoms, leucocytosis (WBC count=58.8 ×10ˆ9/L, with a DLC showing 82% lymphocytes, 12% neutrophils, 4% monocytes, and 2% eosinophils) and epistaxis. LDH was 892 U/L (reference range=235–470 U/L). Imaging studies, including a contrast-enhanced CT neck with CT angiography, revealed an ill-defined heterogeneously enhancing mass centred in the left maxillary sinus, causing erosion of the medial wall and extending medially into the nasal cavity. The mass resulted in destruction of the superior, middle, and inferior turbinates, involvement of the left ethmoidal sinuses, and deviation of the nasal septum to the right.
A subsequent endoscopic endonasal biopsy confirmed the presence of a reddish fragile mass filling the entire left nasal cavity, which bled on touch. Histopathological examination of multiple punch biopsies revealed infiltration by lymphoid cells positive for CD20, PAX5, CD3, CD5, Cyclin D1, SOX11, and Ki67 proliferation index of 90% on IHC. This confirmed relapse of refractory mantle cell lymphoma. Notably, a rare presentation of a fungating mass (5 cm × 3 cm) (figure 1) protruding from the left external nare was observed. The patient was then initiated on a therapeutic regimen of bendamustine with rituximab and ibrutinib. After two cycles of treatment, a complete resolution of the protruding mass was achieved. The patient is currently undergoing counselling for autologous transplantation.
The clinical course of MCL can indeed vary from an indolent (slow-growing) disease to an aggressive and rapidly progressive malignancy. MCL is often diagnosed at an advanced stage, typically stage III or IV, with involvement of multiple lymph nodes and extranodal sites. Extranodal involvement is common in MCL, and various sites can be affected like bone marrow, peripheral blood, gastrointestinal tract, spleen, and Waldeyer’s ring.1
A high Ki-67 proliferation index (>30%) in neoplastic cells has been found to be associated with poor survival in patients with MCL.2 The MIPI is the first prognostic index specific to MCL patients. MIPI is based on the four independent prognostic factors: age, ECOG performance status, LDH, and WBC counts. Our patient had high-risk MIPI score. A study by Hoster et al reported a poor outcome with a median survival of only 29 months in patients with high-risk MIPI score.3
While cases reporting relapse of MCL involving the nasopharynx have been reported, the occurence of a fungating mass (5 cm × 3 cm) protruding out from external nare is an extremely rare presentation and highlights the extent of aggressive nature the cancer can develop.4–6
Our patient had an early relapse within 2 months, which is associated with a poor prognosis. In a study by Bond et al, an inferior median overall survival of 1.3 years was seen in patients with early progression of disease within 6 months after both intensive and less-intensive frontline treatment.7
For cases of relapsed MCL, studies have shown that the combination of bendamustine, rituximab, ibrutinib, and venetoclax is well tolerated and achieves a therapeutic response of 80%.8 Due to the lack of availability of venetoclax in India, our patient was given a combination of bendamustine, rituximab and ibrutinib and a good response was seen with resolution of the mass.
The only literate person in the patient’s family was his daughter, on being asked about her father’s disease, she said, “I am currently a pre-medical student and my father developed this disease just when I had started my preparation. Since I’m the only literate person in my family and the sole child of my father, a lot of the responsibility falls on me to talk to the doctors and try to understand his disease, the treatment protocols and the likelihood of his survival. I then have to explain all of this to my mother and entire family which sometimes becomes very hard for me too to take, but I try to be patient with everyone. My studies have taken a huge hit and I only can take out a few hours of focused studying. When the first chemotherapy protocol was completed and the doctor said that my father was now disease-free, it was a very happy moment for our entire family. But then when the disease came back and now my father is again on chemotherapy and is being planned for a transplant soon, I am very worried as to how things are going to work out. We all pray to god for mercy and hope that all treatment is successful and my father returns healthily soon.
Isolated fungating nasal mass is an extremely rare presentation of relapsed mantle cell lymphoma.
Novel therapeutic agents like ibrutinib are efficient options in relapsed refractory setting.
Patient consent for publication
Contributors VS conceived of the presented idea. VB wrote the main body of the paper. VS verified the paper written by VB and made technical corrections. VS encouraged VB to investigate further literature on the topic so as to provide latest updates. Both authors contributed to the final manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.