Primary Extranodal NK/T-Cell Lymphoma Presenting as Neurolymphomatosis Involving Multiple Cranial Nerves: A Case Report.

Neurolymphomatosis (NL) is a rare condition caused by the lymphomatous or leukemic infiltration of nerves and manifests as neuropathy. Most often, NL is associated with B-lineage non-Hodgkin lymphoma (NHL) and only infrequently occurs in conjunction with T- or NK-lineage NHL. Extranodal NK/T-cell lymphoma (ENKTL)-associated NL is exceedingly unusual, with only 9 cases described in the English language literature, in addition to our case. Diagnosis of NL is challenging, as the entity can mimic neuropathies of more common etiologies, and an adequate biopsy may be difficult to obtain. Timely diagnosis demands a high index of suspicion, especially for patients without a history of hematologic malignancy. We expand upon a unique case of NL exclusively involving cranial nerves and cauda equina nerve roots, as the initial manifestation of ENKTL, and contextualize our findings within the framework of previously reported NK/T-lineage NL cases.

Cerebral aspergillosis within new tumour site presents as incidental new brain lesion in patient receiving temozolomide for glioblastoma multiforme.

Glioblastoma multiforme (GBM) is an aggressive tumour that can lead to lymphopaenia. Its standard treatment involves temozolomide (TMZ) chemotherapy with radiation, often with addition of corticosteroids for symptomatic management. Although TMZ is also immunosuppressive, patients receiving TMZ rarely develop disseminated opportunistic infections. Here, we report the case of a patient with GBM receiving TMZ, radiotherapy and corticosteroids, who develops an incidental new brain lesion that is found to be disseminated Aspergillus within a new GBM tumour site. The patient received successful early treatment of her central nervous system aspergillosis. This case illustrates the profound immunosuppressive potential of GBM in conjunction with TMZ and corticosteroids, which can lead to high-morbidity opportunistic infections concurrently with tumour progression. Future research is needed to elucidate GBM, TMZ and corticosteroids' compound immune effects and guide management that strikes a balance between treating high-morbidity infections and continuing with immunosuppressive chemotherapy.

Paraneoplastic neurological syndromes: a single institution 10-year case series.

BACKGROUND: Given its rare incidence, there are few epidemiological case series on paraneoplastic neurologic syndromes (PNS). METHODS: We present a 10-year series compiled in the Section of Neuro-Oncology, Yale Cancer Center between 2002 and 2012. RESULTS: Twenty-five cases met the PNS Euro-network criteria for definitive PNS. Most (64%; 16/25) had no known neoplasm. Cerebrospinal fluid pleocytosis declined logarithmically over time. Neuroimaging abnormalities were seen in 88% of cases (15/17), but with delayed onset. Therapeutic benefit correlated strongly to pre-treatment modified Rankin Scale (mRS) (p < 0.01), but not with time elapsed between syndrome onset to treatment (p = 0.8), first immunotherapy modality (corticosteroids: n = 10; IVIG: n = 10; PLEX: n = 3; p = 0.37), or number of immunotherapy modalities provided (p = 0.17). PNS-related mortality was high (24%; 6/25). Nonetheless, 16% (3/18; 7 living patients censored) survived over 6 times the anticipated median expected by tumor type and stage. CONCLUSIONS: PNS are rare, at an estimated incidence of 3.1 cases per million-person-years. Detection of CSF pleocytosis and MRI abnormalities depend on time of analysis. While PNS-related mortality was high, immunotherapy benefit correlated strongly with pre-treatment mRS and long-term survival is possible.

Incidence and Outcomes of Intraoperative Descemet Membrane Perforations During Deep Anterior Lamellar Keratoplasty.

PURPOSE: To report the incidence and outcomes of intraoperative Descemet membrane (DM) perforations during deep anterior lamellar keratoplasty (DALK). DESIGN: Retrospective, consecutive, interventional case series. METHODS: A retrospective audit of all DALK cases performed from 2004 to 2015 in a tertiary center, with and without intraoperative DM perforations. We excluded cases with preexisting corneal perforations before surgery. RESULTS: There were a total of 540 eyes, of which 101 (18.7%) had intraoperative DM perforations. These included 79 eyes (78.2%) with microperforations and 15 eyes (14.9%) with macroperforation. The most common steps at which DM perforation occurred intraoperatively were during deep lamellar dissection (32 cases; 31.7%), air injection (27 cases; 26.7%), and suturing (21 cases; 20.8%). Management of the DM perforations included a combination of intracameral air tamponade (49 cases; 48.5%), stromal patching (10 cases; 9.9%), fibrin glue (8 cases; 7.9%), and suturing of the defect (1 case; 1.1%). There were 2 eyes (2/540; 0.37%) that were converted to penetrating keratoplasty (PK). There were no significant differences in the postoperative unaided or best-corrected visual acuity, or in the numbers of patients with postoperative graft failure, graft rejection, or subsequent surgery at postoperative years 1 and 3. CONCLUSIONS: DALK cases with DM perforations intraoperatively are often able to be managed without conversion to PK. Cases with DM perforations intraoperatively have equivalent visual acuity outcomes compared to those without DM perforations, and did not have any increased risk of graft failure, rejection, or subsequent surgery at postoperative years 1 and 3.