Primary Cauda Equina Lymphoma Treated with CNS-Centric Approach: A Case Report and Literature Review

Figures & data

Figure 1 (A) Gadolinium enhanced sagittal and coronal T1 MRI showing diffuse involvement of cauda equina with encasement of conus medullaris (arrows). (B) Staging whole-body 18F-FDG PET-CT coronal and sagittal views showing hypermetabolic mass in the cauda equina and lumbar nerve root sleeves (arrows).

Figure 2 (A) Pathology examination by H&E staining revealing diffusely infiltrating large atypical lymphocytes dissecting through the nerve bundles (arrow indicating residual nerve). IHC studies showed that the neoplastic lymphocytes were positive for (B) CD20, (C) BCL2, (D) BCL6, (E) MUM1, (F) with a high proliferate rate (>90%) by Ki-67.

Figure 3 Follow up MRI (A) and PET-CT (B) after chemotherapy showing complete resolution of the hypermetabolic cauda equina lesion. Linear FDG uptake in the posterior lumbar dura (arrows) is postoperative in nature.

Table 1 Traits of 25 PCEL Patients Reported in the Literature

Reference	Age/Sex	Pathology	CSF Involvement (Cytology)	Mass-Like Lesion	Treatment	Outcome
Nakashima^2014	59/M	DLBCL	Yes	Yes	Radiotherapy, intravenous methotrexate	Alive; 1 year
Mauney^1983	68/F	DLBCL	Yes	Yes	Radiotherapy	Alive; 3 months
Toner^1989	59/M	DLBCL	Yes	No	Radiotherapy, intrathecal methotrexate, intravenous cyclophosphamide, adriamycin, vincristine, etoposide, and prednisolone	Alive; 2 years
Klein^1990	29/F	B-cell lymphoma	No	Yes	Tumor resection	Died; 5 weeks
Knopp^1994	69/F	N/a	N/a	No	N/a	N/a
Ooi^1996	16/M	T-lymphoblastic lymphoma	N/a	Yes	Radiotherapy, intrathecal methotrexate, intravenous methotrexate, bleomycin, doxorubicin, cyclophosphamide, vincristine, and dexamethasone	Died; 8 months
Zagami^2003	71/F	DLBCL	Yes	No	Intrathecal methotrexate, cyclophosphamide, doxorubicin, vincristine, prednisone	Died; 16 months
Kumar^2005	60/M		No	No	IVIG, Rituximab	Alive; 6 months
Tajima^2007	67/F	DLBCL	No	Yes	Radiotherapy and intrathecal methotrexate, IVIG, intravenous carboplatin	Alive; 3 years
Morita^2009	67/M	NK/T-cell lymphoma	N/a	Yes	Radiotherapy, surgical resection, and etoposide	Died; 14 months
Teo^2012	58/M	DLBCL	No	Yes	Radiotherapy, steroids, and intravenous chemotherapy	Alive; 2 years
Iwasaki^2012	69/M	DLBCL	N/a	No	Radiotherapy and intravenous methotrexate	Died; 1.5 years
Nishida^2012	47/M	DLBCL	Yes	Yes	Radiotherapy, intravenous methotrexate and cytarabine, and intrathecal methotrexate, cytarabine, prednisolone	Alive; 1.5 years
Broen^2014	75/F	DLBCL	No	No	Oral dexamethasone	Died; 10 months
Broen^2014	71/F	DLBCL	No	No	Intravenous doxorubicin, vincristine, cyclophosphamide, prednisone, and rituximab, and intrathecal methotrexate.	Alive; n/a
Shin^2016	79/F	DLBCL	N/a	Yes	Radiotherapy and chemotherapy	Alive; N/a
Belcastro^2016	47/M	DLBCL	No	Yes	Intravenous steroids, intrathecal methotrexate, cytarabine, and rituximab	Died; 2 months
Giobbia^1999	30/F	DLBCL	Yes	No	Radiotherapy, intrathecal methotrexate, cytosine arabinoside, and hydrocortisone	Alive; 1 year
Khong^2008	16/M	DLBCL	No	Yes	Radiotherapy, intravenous dexamethasone, cyclophosphamide, cytarabine, doxorubicin, leucovorin, methotrexate, vincristine, rituximab	Alive; 1 year
Beitzke^2010	69/M	DLBCL	Yes	No	Glucocorticoid and intravenous chemotherapy	Died; days after diagnosis
Cugati^2012	11/M	B-cell NHL	N/a	Yes	Radiotherapy and intravenous cyclophosphamide, doxorubicin, vincristine, prednisone	Alive; 1 year
Wang^2016	69/M	B-cell nerve	Yes	Yes	N/a	N/a
Sasaki^2019	62/M	B-cell lymphoma	No	No	Intravenous methotrexate and rituximab, radiotherapy	Alive; 2 years
Suzuki^2018	65/M	DLBCL	Yes	Yes	Intravenous cytarabine and methotrexate	Alive; 6 years
Current Case	55/F	DLBCL	Yes	Yes	Intravenous rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone, methotrexate, cytarabine, thiotepa, BCNU, auto-SCT	Alive; 1.5 years

Abbreviations: DLBCL, Diffuse large B-cell lymphoma; NHL, Non-Hodgkin lymphoma; LPL, Lymphoplasmacytic lymphoma.