Primary peripheral T-cell lymphoma of the cervix with mononeuritis multiplex: an unusual case presentation

Ratul Seal; Mayur Parkhi; Rajesh Kumar; Suvradeep Mitra

doi:10.4322/acr.2021.354

Clinical Case Report

Primary peripheral T-cell lymphoma of the cervix with mononeuritis multiplex: an unusual case presentation

Ratul Seal; Mayur Parkhi; Rajesh Kumar; Suvradeep Mitra

http://dx.doi.org/10.4322/acr.2021.354 Autops Case Rep, vol.12, e2021354, 2022

PDF

Downloads: 0

Abstract

Peripheral neuropathy (PN) is characterized by the injury to the peripheral nervous system of varied etiology. Lymphoma is one of the etiologies of PN, presenting various neurological manifestations. Neuropathy associated with peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is unusual and fewer cases are documented in the literature. In addition, PTCL, NOS is extremely rare as primary in the female genital tract, especially uterine cervix, and exhibits aggressive clinical course with poor therapy response. We hereby describe a 47-year-old female who presented with fever and chills for 15 days. Clinical examination revealed left-sided lower motor neuron type of facial nerve palsy with Bell’s phenomenon. Nerve conduction study of all four limbs illustrated asymmetrical axonal neuropathy (motor > sensory), suggesting mononeuritis multiplex. She developed vaginal bleeding during her hospital stay. Pelvic examination and imaging revealed a 4x3cm polypoidal mass on the posterior lip of the cervix, which was excised and diagnosed as extranodal primary PTCL, NOS based on morphology, immunohistochemistry, and in-situ hybridization findings. Besides, the cerebrospinal fluid (CSF) was infiltrated by the lymphoma cells, detected on cell block preparation. The patient succumbed to her illness within one week despite best efforts and the commencement of chemotherapy. No consent was obtainable for nerve biopsy and autopsy. Thus, we report an extremely rare case of primary extranodal PTCL, NOS of the uterine cervix with unusual presentation of mononeuritis multiplex. Further, we discussed the differentials of PTCL, NOS at this extranodal site.

Keywords

Lymphoma, T-Cell, Peripheral, Cervix Uteri, Mononeuropathies, Cerebrospinal Fluid, Immunohistochemistry

References

1 Swerdlow SH, Campo E, Harris NL, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. rev. Lyon: IARC Press; 2017.

2 Kosari F, Niknejad N, Nili F, Jahanbin B, Malek M. Peripheral T-cell lymphoma presenting as a primary uterine cervix mass: a report of a rare case. Int J Gynecol Pathol. 2017;36(6):523-7. http://dx.doi.org/10.1097/PGP.0000000000000358. PMid:28244895.

3 Nasioudis D, Kampaktsis PN, Frey M, Witkin SS, Holcomb K. Primary lymphoma of the female genital tract: an analysis of 697 cases. Gynecol Oncol. 2017;145(2):305-9. http://dx.doi.org/10.1016/j.ygyno.2017.02.043. PMid:28284518.

4 McLeod JG. Peripheral neuropathy associated with lymphomas, leukemias, and polycythemia vera. In: Dyck PJ, Thomas PK, editors. Peripheral Neuropathy. 3rd ed. Philadelphia: W.B. Saunders; 1993. p. 1591-8.

5 Kawanishi K, Ohyama Y, Kanai Y, et al. Sub-acute demyelinating polyradiculoneuropathy as an initial symptom of peripheral T cell lymphoma, not otherwise specified (PTCL, NOS). Intern Med. 2012;51(15):2015-20. http://dx.doi.org/10.2169/internalmedicine.51.7457. PMid:22864129.

6 Tomita M, Koike H, Kawagashira Y, et al. Clinicopathological features of neuropathy associated with lymphoma. Brain. 2013;136(Pt 8):2563-78. http://dx.doi.org/10.1093/brain/awt193. PMid:23884813.

7 Lagoo AS, Robboy SJ. Lymphoma of the female genital tract: current status. Int J Gynecol Pathol. 2006;25(1):1-21. http://dx.doi.org/10.1097/01.pgp.0000183049.30212.f9. PMid:16306779.

8 Kosari F, Daneshbod Y, Parwaresch R, Krams M, Wacker HH. Lymphomas of the female genital tract: a study of 186 cases and review of the literature. Am J Surg Pathol. 2005;29(11):1512-20. http://dx.doi.org/10.1097/01.pas.0000178089.77018.a9. PMid:16224219.

9 Mourad N, Mounier N, Briere J, et al. Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d’Etude des Lymphomes de l’Adulte (GELA) trials. Blood. 2008;111(9):4463-70. http://dx.doi.org/10.1182/blood-2007-08-105759. PMid:18292286.

10 Ekiz E, Ozkok A, Ertugrul NK. Paraneoplastic mononeuritis multiplex as a presenting feature of adenocarcinoma of the lung. Case Rep Oncol Med. 2013;2013:457346. http://dx.doi.org/10.1155/2013/457346. PMid:24455360.

11 Huda S, Krishnan A. An unusual cause of mononeuritis multiplex. Pract Neurol. 2013;13(1):39-41. http://dx.doi.org/10.1136/practneurol-2012-000343. PMid:23315459.

12 Koike H, Tanaka F, Sobue G. Paraneoplastic neuropathy: wide-ranging clinicopathological manifestations. Curr Opin Neurol. 2011a;24(5):504-10. http://dx.doi.org/10.1097/WCO.0b013e32834a87b7. PMid:21799410.

13 Went P, Agostinelli C, Gallamini A, et al. Marker expression in peripheral T-cell lymphoma: a proposed clinical-pathologic prognostic score. J Clin Oncol. 2006;24(16):2472-9. http://dx.doi.org/10.1200/JCO.2005.03.6327. PMid:16636342.

14 Kern WF, Spier CM, Hanneman EH, Miller TP, Matzner M, Grogan TM. Neural cell adhesion molecule-positive peripheral T-cell lymphoma: a rare variant with a propensity for unusual sites of involvement. Blood. 1992;79(9):2432-7. http://dx.doi.org/10.1182/blood.V79.9.2432.2432. PMid:1373974.

Submitted date:
08/05/2021

Accepted date:
12/29/2021

Publication date:
02/11/2022

Primary peripheral T-cell lymphoma of the cervix with mononeuritis multiplex: an unusual case presentation

Ratul Seal; Mayur Parkhi; Rajesh Kumar; Suvradeep Mitra

Abstract

Keywords

References

Links

Share

Autops Case Rep