Gliomatosis peritonei (GP) is a rare condition characterized by mature glial tissue implants widespread in the peritoneum. The GP is often associated with ovarian teratoma. However, little is known about the characteristics and prognosis of GP. The purpose of this study was to describe the features, treatment, and prognosis of GP. Additionally, we review previously reported cases of GP, summarizing the presently known data. From January 2000 to January 2016, cases of ovarian teratoma and GP treated at Peking Union Medical College Hospital were reviewed. We assessed the pathology, treatments, and outcomes along with prognostic information. Additionally, the literature regarding this clinical condition was also reviewed. Eight patients met the inclusion criteria. Patients had a median age of 20 (range, 15–25) years. GP was diagnosed as the primary tumor in 6 patients and at a secondary surgery in two patients. The primary ovarian tumor consisted of immature teratoma (n = 7) and mature teratoma (n = 1). Grades of immature ovarian teratoma were 2, grade 1; 3, grade 2; and 2, grade 3. Tumors mean had a size of 20.4 (range, 11–30) cm. The median follow-up time was 60.5 (range, 3–144) months. All cases had conservative surgery and seven of them had macroscopic residual disease postoperatively. During the study period, the eight patients remained alive and asymptomatic. Three patients in the study experienced spontaneous pregnancy. After reviewing the existing literature, a total of 14 patients with nodal gliomatosis were present and 10 of them were alive. According to the literature review, five articles reported more than five cases. Of a total of 67 patients, 60 of them remained alive. The prognosis of immature ovarian teratoma with GP is favorable. Complete resection of GP is often difficult. Residual peritoneal disease in GP can be asymptomatic and quiescent over a long period. A more conservative surgical approach may be carried out in patients with massive peritoneal spread after the presence of metastatic immature elements is excluded. Owing to the risk of recurrence and malignant transformation of GP, a long-term follow-up is necessary for patients with residual peritoneal disease.
Intravascular NK-cell lymphoma (IVNKL) is an extremely rare variant of non-Hodgkin lymphoma. To our knowledge, there are only a few cases reported in the English literature. Here, a case of a 29-year-old male with IVNKL involving the skin of the trunk and 4 extremities and liver is presented. A comprehensive literature review is undertaken to summarize the clinical and pathological features of this disorder. In our case, large neoplastic lymphoid cells are restricted to the lumen of small vessels and exhibit the phenotype of a true NK cell. The morphology and immunophenotype, positivity of EBER and NK-cell origin are similar to other IVNKL cases. In addition, some cases including ours carry a poor prognosis as multiple systems or vital organs are involved. In summary, we report a case of an unusual intravascular lymphoma of NK-cell lineage that displays both clinical and pathological features and compare it with other differential diagnoses. It is important to recognize this rare entity to make an appropriate diagnosis and achieve a better understanding regarding the treatment and prognosis.
Because of the rarity of endometrioid borderline ovarian tumours (EBOTs), there is a paucity of data concerning the natural history and prognosis of this condition. Thus, the objective of our study was to establish the feasibility of fertility preservation in young women with EBOTs, as well as their oncological and reproductive outcomes. Consecutive patients with EBOTs, treated at a tertiary referral centre during a span of 22 years, were retrospectively analysed. Recurrence-free interval, as well as its association with the type of surgery and with other clinical and pathological features, was assessed using the Kaplan-Meier and Cox proportional hazards methods. Of the 59 patients studied, the median follow-up time was 30 months (range, 6–177 months). Nine (15.3%) patients developed 13 recurrences 6–137 months after the initial surgeries, including three patients (5.1%; n = 3/59) who developed six invasive recurrences 8, 18 and 68 months after their initial surgeries. Conservative surgery showed a tendency towards a high recurrence rate (17.2% versus 13.3%); however, this difference was not significant (p = 0.45). The 5-year recurrence-free survival rate was significantly higher in the oophorectomy group than in the cystectomy group (p = 0.001). Cox regression analysis showed that none of the variables assessed were associated with an increased hazard ratio for recurrence, except for a younger age at diagnosis (p = 0.021). Of 20 patients who attempted to conceive, three pregnancies among two patients (10.0%) resulted in two live births. Conservative surgery with unilateral adnexectomy can be proposed for young women with EBOTs with fertility desire; however, the reproductive result is not satisfactory. In addition, careful evaluations of the endometria should be offered during the initial surgery and follow-up period. Retrospectively registered.
Objectives. Primary central nervous system lymphoma (PCNSL) usually has a poor prognosis. Cerebrospinal fluid (CSF) interleukin (IL)-10 has shown diagnostic, prognostic, and monitoring value in our previous studies. Cell-free circulating tumor DNA can be detected in the CSF of refractory/relapse cases and has also shown monitoring value. However, information about its monitoring value in newly diagnosed PCNSL patients and comparisons of CSF IL-10 and CSF cell-free DNA (cfDNA) are scarce. Methods. We performed next-generation sequencing on paraffin-embedded tissue and the serial CSF cfDNA of 10 newly diagnosed PCNSL patients and on the baseline CSF cfDNA of 11 other central nervous system lymphoma patients. We also monitored the CSF IL-10 levels of the 10 newly diagnosed PCNSL patients. Results. In seven newly diagnosed PCNSL patients with sufficient baseline CSF cfDNA, six had 1 mutated genes in their CSF cfDNA. The most common were MYD88(4/7), PIM1(3/7), MLL2(3/7), and ETV6(2/7). We also identified multiple somatic mutations, most commonly in PIM1. MYD88L265P can be detected in both tumor tissue and CSF cfDNA. The genomic profiles of CFS cfDNA were similar in PCNSL and PIOL patients. Newly diagnosed PCNSL patients with persistently positive cfDNA and negative IL-10 progressed quickly, while those with negative cfDNA and negative IL-10 were in maintenance therapy for more than 18months. Two patients without cfDNA had increased CSF IL-10 concentrations before disease relapse. These results indicate that negative CSF cfDNA predicts better results, and persistently positive CSF cfDNA predicts disease progression earlier than conventional magnetic resonance imaging. Conclusion. In conclusion, CSF cfDNA is a potential predictor of relapse and progression, which complements the monitoring value of CSF IL-10 in newly diagnosed PCNSL patients.
Objective: This study aimed to investigate the clinicopathological features of indolent T-cell lymphoproliferative disease of the gastrointestinal tract (ITLPD-GI) and to improve its diagnostic and therapeutic approaches.Methods: A retrospective analysis was conducted on eight ITLPD-GI patients treated between January 2018 and January 2024. Clinical data, pathological features, immunophenotypes, molecular testing results, and follow-up records were reviewed.Results: Clinical characteristics: Male-to-female ratio 3:5; mean age at onset 42 years. Symptoms: Predominantly diarrhea and abdominal pain. Endoscopic findings: Erosions, multiple shallow ulcers, and small polypoid lesions. Pathological features: Histology: Atrophy of gastric/intestinal glands with diffuse infiltration of small lymphocytes (round/irregular nuclei, dense chromatin) in the lamina propria; rare mitoses; absence of angioinvasion or necrosis. Notably, two cases showed prominent plasma cell infiltration in the superficial mucosa. Immunophenotype: Pan-T-cell markers positive (5/8); CD4−/CD8+ (5/8), CD4+/CD8+ (2/8), CD4+/CD8− (1/8); aberrant CD20 expression (2/8); low Ki-67 index. TCR rearrangement: Monoclonal in all four tested cases. Treatment and prognosis: Supportive therapy (five cases): Dietary modification, immunosuppression, immunomodulation, and anti-infective agents. Symptoms resolved in one case but persisted in four. Targeted therapy (one CD20+ case): Rituximab added, with no improvement after 14 months of follow-up. Chemotherapy (two cases): Prednisone + thalidomide; one achieved significant remission at 9 months, while the other showed no response (persistent diarrhea/anxiety) at 35 months. No disease progression was observed during follow-up.Conclusion: ITLPD-GI is a rare indolent monoclonal T-cell proliferation with non-specific clinical/endoscopic features, necessitating differentiation from aggressive lymphomas to avoid misdiagnosis and overtreatment. Diagnosis relies on histomorphology, immunohistochemistry, and TCR clonality assessment (critical for atypical cases, e.g., CD20+). The majority of patients have favorable outcomes with conservative management. Enhanced clinical awareness and novel therapeutic targets warrant further exploration.