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Tumefactive appearance of peripheral nerve involvement in hematologic malignancies: a new imaging association

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Abstract

Objective

In neurolymphomatosis (NL), the affected nerves are typically described to be enlarged and hyperintense on T2W MR sequences and to avidly enhance on gadolinium-enhanced T1WI. This pattern is highly non-specific. We recently became aware of a “tumefactive pattern” of NL, neuroleukemiosis (NLK) and neuroplasmacytoma (NPLC), which we believe is exclusive to hematologic diseases affecting peripheral nerves.

Materials and methods

We defined a “tumefactive” appearance as complex, fusiform, hyperintense on T2WI, circumferential tumor masses encasing the involved peripheral nerves. The nerves appear to be infiltrated by the tumor. Both structures show varying levels of homogenous enhancement. We reviewed our series of 52 cases of NL in search of this pattern; two extra outside cases of NL, three cases of NLK, and one case of NPLC were added to the series.

Results

We identified 20 tumefactive lesions in 18 patients (14 NL, three NLK, one NPLC). The brachial plexus (n = 7) was most commonly affected, followed by the sciatic nerve (n = 6) and lumbosacral plexus (n = 3). Four patients had involvement of other nerves. All were proven by biopsy: the diagnosis was high-grade lymphoma (n = 12), low-grade lymphoma (n = 3), acute leukemia (n = 2), and plasmacytoma (n = 1).

Conclusions

We present a new imaging pattern of “tumefactive” neurolymphomatosis, neuroleukemiosis, or neuroplasmacytoma in a series of 18 cases. We believe this pattern is associated with hematologic diseases directly involving the peripheral nerves. Knowledge of this association can provide a clue to clinicians in establishing the correct diagnosis. Bearing in mind that tumefactive NL, NLK, and NPLC is a newly introduced imaging pattern, we still recommend to biopsy patients with suspicion of a malignancy.

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Abbreviations

NL:

Neurolymphomatosis

NLK:

Neuroleukemiosis

NPLC:

Neuroplasmacytoma

NHL:

Non-Hodgkin’s lymphoma

DLBCL:

Diffuse large B-cell lymphoma

MALT:

Mucosa-associated lymphatic tissue lymphoma

LG BCL with PD:

Low-grade B-cell lymphoma with plasmacytic differentiation

LPL:

Lymphoplasmacytic lymphoma

NOS:

Not otherwise specified

AML:

Acute myelogenous leukemia

W:

Weakness

N:

Numbness

P:

Pain

Par:

Paresthesias

MM:

Multiple myeloma

FDG:

18-fluorodeoxyglucose

PET/CT:

Positron emission tomography/computed tomography

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Acknowledgments

S.C. is supported by European Regional Development Fund - Project FNUSA-ICRC (No. CZ.1.05/1.1.00/02.0123). We thank Dr. Brian P. O’Neill for the wisdom he shared with us during numerous consultations.

Conflict of interest

The authors declare that they have no conflicts of interest.

Ethical Declaration

An approval from the institutional review board was obtained before the conduct of the study. The patients have consented to the inclusion in the study. This study was conducted in accordance with the Helsinki Declaration of 1975, as revised in 2000 and 2009.

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Correspondence to Robert J. Spinner.

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Capek, S., Hébert-Blouin, MN., Puffer, R.C. et al. Tumefactive appearance of peripheral nerve involvement in hematologic malignancies: a new imaging association. Skeletal Radiol 44, 1001–1009 (2015). https://doi.org/10.1007/s00256-015-2151-3

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