Objective: To study the clinicopathological characteristics, and further understand primary central nervous system T-cell lymphoma (PCNSTCL) in children and adolescents. Methods: Five cases of PCNSTCL in children and adolescents were collected from December 2016 to December 2021 at the First Affiliated Hospital of Zhengzhou University. The clinicopathological characteristics, immunophenotypic, and molecular pathologic features were analyzed, and relevant literatures reviewed. Results: There were two male and three female patients with a median age of 14 years (range 11 to 18 years). There were two peripheral T-cell lymphomas, not otherwise specified, two anaplastic large cell lymphoma, ALK-positive and one NK/T cell lymphoma. Pathologically, the tumor cells showed a variable histomorphologic spectrum, including small, medium and large cells with diffuse growth pattern and perivascular accentuation. Immunohistochemistry and in situ hybridization showed CD3 expression in four cases, and CD3 was lost in one case. CD5 expression was lost in four cases and retained in one case. ALK and CD30 were expressed in two cases. One tumor expressed CD56 and Epstein-Barr virus-encoded RNA. All cases showed a cytotoxic phenotype with expression of TIA1 and granzyme B. Three cases had a high Ki-67 index (>50%). T-cell receptor (TCR) gene rearrangement was clonal in two cases. Conclusions: PCNSTCL is rare, especially in children and adolescents. The morphology of PCNSTCL is diverse. Immunohistochemistry and TCR gene rearrangement play important roles in the diagnosis.
Female ; Humans ; Male ; Central Nervous System/pathology* ; Central Nervous System Neoplasms/pathology* ; Epstein-Barr Virus Infections ; Herpesvirus 4, Human ; Lymphoma, T-Cell/pathology* ; Lymphoma, T-Cell, Peripheral/genetics* ; Receptor Protein-Tyrosine Kinases/genetics* ; Receptors, Antigen, T-Cell ; Child ; Adolescent

OBJECTIVETo investigate the association of peripheral nerve invasion (PNI) with clinicopathological factors and prognosis of colorectal cancer.

METHODSClinicopathological data and Surgical specimens of 372 colorectal cancer patients who underwent radical resection from January 2011 to June 2012 in The Second Affiliated Hospital of Harbin Medical University were collected. Histopathological evaluation of tissue samples was conducted with hematoxylin and eosin-stained sections. PNI was considered positive when cancer cells were observed inside the nerve sheath, or when at least 33% of the nerve periphery was surrounded by cancer cells. The relationship between PNI and clinicopathological factors of colorectal cancer was analyzed by χtest or Fisher's exact test. Three-year overall survivals of PNI positive and negative patients were determined using the Kaplan-Meier method. Detection results were compared using log-rank test.

RESULTSOf 372 colorectal cancer patients, 133 (35.8%) were PNI positive. Among the PNI positive patients, 63 cases were male and 70 cases female; 76 cases were more than 60 years old and 57 cases less than 60 years old; tumors of 6 cases located in the ileocecal colon, of 33 cases in the ascending colon, of 7 cases in the transverse colon, of 8 cases in the descending colon, of 22 cases in the sigmoid colon, and of 57 cases in the rectum; tumor diameter was greater than 4 cm in 83 cases, and less than 4 cm in 50 cases; tumors of 48 cases were moderately or highly differentiated, and of 85 cases poorly-differentiation; tumor invasion depth in 2 cases, T2 in 7 cases, T3 in 93 cases, T4 in 31 cases; lymphatic metastasis was N0 phase in 56 cases, N1 in 41 cases, and N2 in 36 cases; tumors were stage I( in 2 cases, stage II( in 40 cases, of stage III( in 75 cases and stage IIII( in 16 cases. The positive rate of PNI was significantly associated with tumor location (χ=11.20, P=0.048), tumor size (χ=21.80, P=0.000), differentiation (χ=60.90, P=0.000), depth of invasion (χ=19.00, P=0.000), lymph node metastasis (χ=19.70, P=0.000) and TNM staging (χ=70.80, P=0.000), but not with sex, age or vascular invasion(P>0.05). The median follow-up time was 48 (8 to 62) months. Kaplan-Meier survival curve showed that the 3-year survival rate of PNI positive patients was 52.6%, significantly lower than that of PNI negative patients(78.3%, P=0.000). Further analysis of patients with stage II( and III( colorectal cancer showed that the 3-year survival rates of PNI positive patients were 62.3% and 43.5%, respectively, which were significantly lower than those of PNI negative patients with stage II( and III((91.7% and 79.4%), and the differences were statistically significant(P=0.000).

CONCLUSIONSPNI is a poor prognostic factor of colorectal cancer. It may be a complement of the classic TNM staging classification in stratifying colorectal cancer patients, especially in stages II( and III(.

Aged ; Colorectal Neoplasms ; diagnosis ; epidemiology ; mortality ; pathology ; Female ; Follow-Up Studies ; Humans ; Kaplan-Meier Estimate ; Lymphatic Metastasis ; pathology ; Male ; Middle Aged ; Neoplasm Grading ; statistics & numerical data ; Neoplasm Invasiveness ; pathology ; physiopathology ; Neoplasm Staging ; statistics & numerical data ; Peripheral Nervous System Neoplasms ; mortality ; pathology ; Prognosis ; Retrospective Studies ; Risk Factors ; Survival Rate

PURPOSE: The purpose of this study was to examine the mediation of psychological distress in the relationship between disturbance in ADL from chemotherapy induced peripheral neuropathy and quality of life in order to provide a basis for planning nursing interventions to improve the quality of life in cancer patients. METHODS: A purposive sample of 130 patients treated with chemotherapy were recruited in the cross-sectional survey design. Data were collected using self-report questionnaires. The instruments were the Chemotherapy Induced Peripheral Neuropathy Assessment Tool (CIPNAT), Hospital Anxiety Depression Scale (HADS), and Functional Assessment of Cancer Therapy-General (FACT-G). RESULTS: The mean score for disturbance in ADL from chemotherapy induced peripheral neuropathy was 3.30. Overall quality of life was 2.48. The mean score was 1.04 for psychological distress. The prevalence was 35.4% for anxiety and 47.7% for depression. There were significant correlations among the three variables, disturbance in ADL from chemotherapy induced peripheral neuropathy, psychosocial distress, and quality of life. Psychosocial distress had a complete mediating effect (beta= -.74, p <.001) in the relationship between disturbance in ADL from chemotherapy induced peripheral neuropathy and quality of life (Sobel test: Z= -6.11, p <.001). CONCLUSION: Based on the findings of this study, nursing intervention programs focusing on disturbance of ADL management, and decrease of psychological distress are highly recommended to improve quality of life in cancer patients.
*Activities of Daily Living ; Adult ; Aged ; Antineoplastic Agents/*adverse effects/therapeutic use ; Anxiety ; Cross-Sectional Studies ; Depression/etiology ; Female ; Humans ; Male ; Middle Aged ; Neoplasms/drug therapy/pathology/*psychology ; Peripheral Nervous System Diseases/*etiology ; *Quality of Life ; Self Report ; Stress, Psychological ; Surveys and Questionnaires

OBJECTIVETo study the clinicopathologic characteristics of peripheral neuroblastic tumors and to evaluate the prognostic significance of these features.

METHODSThe clinical and pathologic findings were retrospectively reviewed in 121 cases of peripheral neuroblastic tumor. The clinical outcomes of patients were evaluated. The three-year event-free survival rate was analyzed, with respect to age of patients, Evan's staging, International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.

RESULTSThe median age at diagnosis was 2.7 years; and 96 cases (79.3%) occurred in patients younger than 5 years old. The number of cases in Evan's staging I, II, III, IV and IVs was 24, 39, 24, 29 and 5, respectively. There were 82 cases of neuroblastoma (NB) (including 2 cases of undifferentiated NB, 52 cases of poorly differentiated NB and 28 cases of differentiating NB), 9 cases of ganglioneuroblastoma, intermixed type (GNBi), 19 cases of ganglioneuroma, maturing type (GN) and 11 cases of ganglioneuroblastoma, nodular type (GNBn). Forty-nine cases were in the favorable histology subgroup and 72 cases in the unfavorable histology subgroup. The overall three-year event-free survival rate of the 121 cases was 73.0% ± 4.3%. The three-year event-free survival rates were associated with age (P = 0.002), Evan's staging (P = 0.000), histologic category (P = 0.000), mitosis-karyorrhexis index (P = 0.043), prognostic subgroup (P = 0.000).

CONCLUSIONSMost of the peripheral neuroblastic tumors occur in the children younger than 5 years old. It is composed of NB, GNBi, GN and GNBn. The three-year event-free survival rate is approximately 70%. Significant prognostic parameters include age of patients, Evan's staging, International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.

Age Factors ; Antigens, Nuclear ; metabolism ; Child ; Child, Preschool ; Disease-Free Survival ; Female ; Ganglioneuroblastoma ; metabolism ; pathology ; surgery ; Ganglioneuroma ; metabolism ; pathology ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasm Staging ; Nerve Tissue Proteins ; metabolism ; Nestin ; metabolism ; Neuroblastoma ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; Retrospective Studies ; S100 Proteins ; metabolism

Age Factors ; Child ; Child, Preschool ; Ganglioneuroblastoma ; genetics ; metabolism ; pathology ; ultrastructure ; Ganglioneuroma ; genetics ; metabolism ; pathology ; ultrastructure ; Gene Amplification ; Gene Expression Regulation, Neoplastic ; Humans ; Infant ; N-Myc Proto-Oncogene Protein ; Neoplasm Staging ; Neuroblastoma ; genetics ; metabolism ; pathology ; ultrastructure ; Nuclear Proteins ; genetics ; metabolism ; Oncogene Proteins ; genetics ; metabolism ; Peripheral Nervous System Neoplasms ; classification ; genetics ; metabolism ; pathology ; ultrastructure ; Prognosis ; Proto-Oncogene Proteins c-myc ; metabolism ; Receptor, trkA ; metabolism ; S100 Proteins ; metabolism

Female ; Humans ; Mesothelioma ; pathology ; Middle Aged ; Neoplasm Metastasis ; Peripheral Nervous System Neoplasms ; secondary ; Radiography ; Sciatic Neuropathy ; diagnosis ; diagnostic imaging ; etiology

OBJECTIVETo study the clinicopathologic features of lipomatosis of nerve (NLS).

METHODSThe clinical, radiologic and pathologic features were analyzed in 15 cases of NLS.

RESULTSThere were a total of 10 males and 5 females. The age of patients ranged from 4 to 42 years (mean age = 22.4 years). Eleven cases were located in the upper limbs and 4 cases in the lower limbs. The median nerve was the most common involved nerve. The patients typically presented before 30 years of age (often at birth or in early childhood) with a soft and slowly enlarging mass in the limb, with or without accompanying motor and sensory deficits. Some cases also had macrodactyly and carpal tunnel syndrome. MRI showed the presence of fatty tissue between nerve fascicles, resembling coaxial cable in axial plane and assuming a spaghetti-like appearance in coronal plane. On gross examination, the affected nerve was markedly increased in length and diameter. It consisted of a diffusely enlarged greyish-yellow lobulated fusiform beaded mass within the epineural sheath. Histologically, the epineurium was infiltrated by fibrofatty tissue which separated, surrounded and compressed the usually normal-appearing nerve fascicles, resulting in perineural septation of nerve fascicles and microfascicle formation. The infiltration sometimes resulted in concentric arrangement of perineural cells and pseudo-onion bulb-like hypertrophic changes. The perineurial cells might proliferate, with thickening of collagen fibers, degeneration and atrophic changes of nerve bundles. Immunohistochemical study showed that the nerve fibers expressed S-100 protein, neurofilament and CD56 (weak). The endothelial cells and dendritic fibers were highlighted by CD34. The intravascular smooth muscle cells were positive for muscle-specific actin.

CONCLUSIONSNLS is a rare benign soft tissue tumor of peripheral nerve. The MRI findings are characteristic. A definitive diagnosis can be made with histologic examination of tissue biopsy.

Adolescent ; Adult ; Antigens, CD34 ; metabolism ; CD56 Antigen ; metabolism ; Carpal Tunnel Syndrome ; complications ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Extremities ; innervation ; Female ; Hand Deformities, Congenital ; complications ; pathology ; Humans ; Lipoma ; pathology ; Lipomatosis ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Median Nerve ; metabolism ; pathology ; Nerve Sheath Neoplasms ; pathology ; Neurofibroma ; pathology ; Neurofilament Proteins ; metabolism ; Neuroma ; pathology ; Peripheral Nervous System Diseases ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; pathology ; Retrospective Studies ; S100 Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo study the clinicopathologic characteristics of peripheral neuroblastic tumors and to investigate the prognostic significance of International Neuroblastoma Pathology Classification (INPC).

METHODSOne hundred and thirty-five cases of peripheral neuroblastic tumors encountered in Shanghai Children's Medical Center were enrolled into the study. All the cases were classified according to INPC and International Neuroblastoma Staging System (INSS). The follow-up data were analyzed.

RESULTSThe consensus diagnoses of the 135 cases were as follows: 80 cases (59.2%) of neuroblastoma, 24 cases (17.8%) of ganglioneuroblastoma, intermixed, 17 cases (12.6%) of ganglioneuroma and 14 cases (10.4%) of ganglioneuroblastoma, nodular. The cases were subdivided into 2 subgroups: favorable histology (number = 90, 66.7%) and unfavorable histology (number = 45, 33.3%). According to INSS, the number of cases in stages I, II, III and IV was 22 (16.3%), 24 (17.8%), 34 (25.2%) and 55 (40.7%), respectively. The survival of peripheral neuroblastic tumors correlated with histologic diagnosis, INPC and INSS (P < 0.05).

CONCLUSIONDiagnostic categorization of peripheral neuroblastic tumors according to INPC is of prognostic value.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Ganglioneuroblastoma ; pathology ; surgery ; Ganglioneuroma ; pathology ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasm Staging ; Neuroblastoma ; classification ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; classification ; pathology ; surgery ; Retrospective Studies ; Survival Rate

PURPOSE: In treating schwannoma patients, it is critical to determine the origin of the tumor to preserve nerve function. We evaluated the validity of preoperative imaging studies in distinguishing the neurological origin of the schwannomas of the head and neck, and the efficacy of intracapsular enucleation in preserving nerve function. MATERIALS AND METHODS: In 7 cases of schwannomas in the head and neck region, we predicted whether the tumor originated from the vagus nerve or the cervical sympathetic chain through imaging studies including computed tomography (CT) and magnetic resonance imaging (MRI). All patients were performed intracapsular enucleation, and the function of the vagus nerve and the sympathetic nerve was evaluated preoperatively and postoperatively. RESULTS: Preoperative imaging studies showed 6 cases where the tumor was located between the carotid artery and the internal jugular vein, and 1 case where the tumor was located posteriorly, displacing the carotid artery and the internal jugular vein anteriorly. At the time of operation, we confirmed schwannoma originating from the vagus nerve on the first 6 cases, and schwannoma originating from the sympathetic nervous system on the last case. All patients went through successful intracapsular enucleation, and of the seven schwannoma cases, 6 patients maintained normal postoperative neurological function (85.7%). CONCLUSION: Preoperative imaging studies offer valuable information regarding the location and origination of the tumor, and intracapsular enucleation helped us to preserve the nerve function.
Aged ; Diagnostic Imaging/methods ; Female ; Follow-Up Studies ; Head and Neck Neoplasms/complications/diagnosis/*pathology ; Humans ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Neurilemmoma/complications/diagnosis/*pathology ; Peripheral Nervous System/injuries/physiology ; Sympathetic Nervous System/physiology ; Tomography, X-Ray Computed/methods ; Treatment Outcome ; Vagus Nerve/physiology

Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangiosarcoma ; pathology ; Humans ; Median Nerve ; pathology ; Median Neuropathy ; metabolism ; pathology ; surgery ; Melanoma ; pathology ; Middle Aged ; Peripheral Nervous System Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism