Subcutaneous panniculitis-like T-cell lymphoma invading central nervous system in long-term clinical remission with lenalidomide: A case report.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) involvement in the central nervous system (CNS) is particularly rare. SPTCL with CNS involvement has an exceedingly poor prognosis, and no optimum therapeutic method has been discovered. To the best of our knowledge, this is the first reported case of SPTCL invading the CNS achieving long-term remission with lenalidomide maintenance therapy. CASE SUMMARY: A 63-year-old man diagnosed with SPTCL was admitted to the hospital with severe headache for 15 d after four cycles of chemotherapy. Subsequent to the treatment, the patient developed CNS involvement. Craniotomy biopsy was pathologically diagnosed as CNS T-cell lymphoma, and two courses of chemotherapy were performed postoperatively. Due to the intolerance of the side effects of chemotherapeutic drugs, the patient received lenalidomide instead. The magnetic resonance imaging of the head at the 8 mo follow-up indicated no signs of recurrence, and the vital signs were stable. CONCLUSION: Lenalidomide deserves further investigation as a targeted drug for SPTCL cases involving the CNS.

Infective endocarditis mimicking ANCA-associated vasculitis: does it require immunosuppressive therapy?: A case report and literature review.

RATIONALE: In the course of endocarditis, the development of antineutrophil cytoplasmic antibody (ANCA)-mediated disease introduces the dilemma of determining the best treatment approach for immune conditions, whether immunosuppressant therapy should be added to antibiotic treatment has remained controversial. PATIENT CONCERNS: A 33-year-old man presented with progressive fever lasting for 7 months, and swelling, pain, and purpura in the arms and legs. The patient showed multiple autoantibodies including cytoplasmic ANCA, antiproteinase 3, rheumatoid factor, and anti-beta 2 glycoprotein I. Blood culture was positive for viridans streptococcus, and renal biopsy revealed glomerulonephritis and interstitial nephritis. DIAGNOSIS: Endocarditis caused by viridans streptococci, ANCA-associated vasculitis, and congenital ventricular septal defect. INTERVENTIONS: In addition to effective antibiotics, he also received twice intravenous corticosteroids and intravenous immunoglobulin therapy, and a low dose of cyclophosphamide. At last, the patient received congenital ventricular septal defect repair and debridement. OUTCOMES: The abnormal clinical manifestations, including renal failure and loss of strength, recovered rapidly with corticosteroid therapy in addition to antibiotic treatment. After 6 months without any medications, he remained asymptomatic and was able to live normally. LESSONS: In this case with endocarditis and ANCA-associated vasculitis, we highlighted the importance of biopsy and immunosuppressive therapy. Histopathologic examination is required for diagnosis and treatment in such case. Identifying patients who have endocarditis and ANCA positivity with vasculitis pathologic features will require corticosteroid/immunosuppressives in addition to the antibiotics therapy.

Paraplegia from spinal intramedullary tuberculosis: A case report.

BACKGROUND: Tuberculosis (TB) mostly attacks the lungs, and extrapulmonary TB involving the central nervous system is uncommon; among these cases, spinal intramedullary TB is even more rare. The clinical manifestations of spinal intramedullary TB are similar to those of intramedullary spinal cord tumors. Therefore, it is necessary to make a careful differential diagnosis of spinal intramedullary lesions to achieve the appropriate treatment and favorable prognosis. We report a rare case of a young male patient with paraplegia due to spinal intramedullary TB, which is uncommon and regrettable. CASE SUMMARY: A 23-year-old male presented with fever accompanied by nausea and vomiting lasting for 2 mo and was then diagnosed with tubercular meningitis. After anti-TB treatment, his symptoms were significantly improved. However, 2 mo after the diagnosis of tubercular meningitis, the patient felt numbness below the costal arch level, which lasted for 1 wk, and he paid no attention to this symptom. What followed was paraplegia and urine/fecal incontinence. Magnetic resonance imaging of the thoracic spine showed a ring-enhanced intramedullary cord lesion at T8-T9. Lesion exploration showed enlargement of the spinal cord at T8-T9, and the lesion could be observed by incision. The lesion was adhered to the peripheral tissue and was grayish-white and tough with a poor blood supply and a diameter of approximately 0.8 cm. The lesion was resected completely. The results of pathological examination by both hematoxylin-eosin staining and acid-fast bacilli staining confirmed TB, accompanied by acute and chronic suppurative inflammation and granulation tissue formation. The patient was instructed to continue anti-TB treatment after the operation, but he did not follow the medical advice. Follow-up continued for ten years, the patient had persistent paraplegia, the numbness disappeared and urine/fecal sensation recovered. CONCLUSION: Although TB is a kind of benign disease, some cases progress rapidly. Moreover, spinal intramedullary TB may seriously endanger quality of life and still needs timely diagnosis and proper treatment.

CD4+Foxp3+CD25+/- Tregs characterize liver tissue specimens of patients suffering from drug-induced autoimmune hepatitis: A clinical-pathological study.

BACKGROUND: The diagnosis of drug-induced autoimmune hepatitis (DIAIH) and its differentiation from idiopathic autoimmune hepatitis (AIH) is challenging. This study aimed to differentiate DIAIH from AIH by comparing the biochemical changes, histological features, and frequencies of CD4+Foxp3+CD25+/- regulatory T cells (Tregs) in liver tissues or peripheral blood lymphocytes. METHODS: A total of 15 DIAIH patients and 24 AIH patients who underwent liver biopsies at initial presentation were enrolled in this study. The liver histological changes were assessed by HE staining. The phenotypic recognition and distribution of CD4+Foxp3+CD25+/- Tregs in liver tissues were evaluated by single/double immunostains in serial sections. The CD4+Foxp3+CD25+/- Tregs in peripheral blood were analyzed by flow cytometry. RESULTS: The median values of ALT and AST were 404.50 U/L and 454.10 U/L in DIAIH patients and 309.50 U/L and 315.00 U/L in AIH patients, respectively. More importantly, for the first time we found that patients with DIAIH had higher levels of serum ALT and AST, more severe degree of lobular inflammation, higher frequencies of zone 3 necrosis and higher number of lobular CD4+Foxp3+CD25-Tregs compared with AIH (P < 0.05). Furthermore, there were positive correlations in DIAIH between the degree of lobular inflammation and either the AST/ALT level or the number of lobular CD4+Foxp3+CD25- Tregs (P < 0.05). However, the frequency of peripheral blood CD4+Foxp3+CD25+/- Tregs were not significantly different between DIAIH and AIH. CONCLUSIONS: The differences of ALT, AST and the number of lobular CD4+Foxp3+CD25- Tregs between patients with DIAIH and those with AIH are clinically helpful in differentiating these two diseases in their early stage.

Striated muscle-derived intraspinal choristoma: A case report.

Choristoma is a mass of tissue that is histologically normal for the organ or part of the body from which it originates, but is located at an abnormal site. Choristoma of the nervous system involves the occurrence of residual dysplastic tissues outside the nervous system and exhibits a low incidence. Thus far, there has been no prior report of intraspinal choristoma originating from the striated muscle. The patient in the present case was a young man who was admitted to the hospital wing to intermittent lumbago. A lumbar magnetic resonance imaging examination revealed a cystic-solid lesion in the spinal canal at the S1 level. Preoperatively, the lesion was suspected to be a teratoma and was therefore completely removed. The postoperative pathological report suggested that the mass was an intraspinal choristoma, a benign lesion associated with a good prognosis, which had originated from striated muscle. Early diagnosis and treatment can prevent progression of nerve dysfunction. The patient recovered well postoperatively and the symptoms of lower back pain disappeared. Although intraspinal choristomas are rare, when a lesion in the spinal canal is diagnosed, it should be considered in the differential diagnosis tumors.

Desmoplastic fibroma in the proximal femur: A case report with long-term follow-up.

Desmoplastic fibroma of the bone is an extremely rare primary benign tumor. The present study reports a case of desmoplastic fibroma of the bone with the longest published follow-up. A 21-year-old female presented to The First Hospital of Jilin University (Changchun, Jilin, China) with thigh pain. Radiography demonstrated a lytic expansile lesion in the proximal femur. Curettage was performed, followed by use of an allogeneic graft. One month later, the patient suffered a pathological fracture and was treated with an open reduction and internal fixation. There was no recurrence of the tumor over a 28-year follow-up period. In conclusion, desmoplastic fibroma in the proximal femur is rare and an intralesional resection is strongly recommended to prevent recurrence. The disease may be misdiagnosed as a bone cyst, so the diagnosis should be confirmed with a histological examination.

IgG4-related autoimmune pancreatitis overlapping with Mikulicz's disease and lymphadenitis: a case report.

Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that is categorized as type 1 or type 2 according to the clinical profile. Type 1 AIP, which predominantly presents in a few Asian countries, is a hyper-IgG4-related disease. We report a case of IgG4-related AIP overlapping with Mikulicz's disease and lymphadenitis, which is rare and seldom reported in literature. A 63-year male from Northeast China was admitted for abdominal distension lasting for one year. He presented symmetric swelling of the parotid and submandibular glands with slight dysfunction of salivary secretion for 6 mo. He had a 2-year history of bilateral submandibular lymphadenopathy without pain. He underwent surgical excision of the right submandibular lymph node one year prior to admission. He denied any history of alcohol, tobacco, or illicit drug use. Serological examination revealed high fasting blood sugar level (8.8 mmol/L) and high level of IgG4 (15.2 g/L). Anti-SSA or anti-SSB were negative. Computed tomography of the abdomen showed a diffusely enlarged pancreas with loss of lobulation. Immunohistochemical stain for IgG4 demonstrated diffuse infiltration of IgG4-positive plasma cells in labial salivary gland and lymph node biopsy specimens. The patient received a dose of 30 mg/d of prednisone for three weeks. At this three-week follow-up, the patient reported no discomfort and his swollen salivary glands, neck lymph node and pancreas had returned to normal size. The patient received a maintenance dose of 10 mg/d of prednisone for 6 mo, after which his illness had not recurred.

Coexistence of spinal teratoma of the conus medullaris and arteriovenous malformation in an adult: a case report.

The coexistence of spinal teratoma of the conus medullaris and arteriovenous malformation (AVM) is exceptional, which has not been reported previously in the literature. The precise mechanism of the coexistence of these conditions is not known, however, the dysembryonic origin of spinal cord teratoma and AVM seems to play a part in this process. A 34-year-old male patient was admitted with lower back pain, bilateral lower extremity numbness and weakness, and sexual disturbance. Magnetic resonance imaging (MRI) showed an AVM extended cranially from the top of a heterogeneous expansile lesion of the conus medullaris. Surgical exploration and histopathological examination revealed a mature teratoma associated with the AVM. A literature review supported the dysembryonic origin of spinal cord teratomas and AVMs. This unique case may provide insight into the etiopathogenesis of the coexistence of spinal teratoma of the conus medullaris and AVM.

[Suboccipital transtentorial approach to pineal region meningiomas by 3D-CTA assistance: surgical considerations].

OBJECTIVE: To evaluate the three-dimensional CT angiography (3D-CTA) assisted suboccipital transtentorial approach (Poppen's approach) in the treatment of pineal region meningioma. METHODS: During the period of January 2005 to January 2010, 8 patients with pineal region meningioma were successfully treated using Poppen's approach through cerebral falx and tentorium. There were three male patients and five female patients were aged at a range of 41 - 64 years, average age was (54 ± 10) years. According to the Karnofsky performance scale (KPS), 5 patients' KPS scores were more than or equal to 80 and 3 were less than 80. MRI was used for the diagnosis of meningioma. 3D-CTA was applied to detect meningioma staining and blood supply. For preoperative concurrent hydrocephalus, follow-up observations were given. If hydrocephalus didn't get better or even became worse, ventriculoperitoneal shunt should be considered. RESULTS: All the surgery were successfully performed, and venous complexes (VC) were well protected according to the CTA images. Out of the eight cases whose meningiomas were removed, one patient had got postoperative intracranial infection and recovered after given antibiotics. All patients were followed up for a period of 6 - 24 months. Preoperative concurrent hydrocephalus in 7 patients were improved. However, there was an aggravation of the hydrocephalus in one patient who was treated with ventriculoperitoneal shunt. The MRIs which were performed at the end of follow-up period, showed no recurrence of meningiomas, and preoperative symptoms were improved to varying degrees, 7 patients' KPS scores were more than or equal to 80 and 1 was less than 80. A χ(2) test was used to analyze and to make comparisons between preoperative and postoperative KPS. The significance was indicated (χ(2) = 1.33, P < 0.05). CONCLUSIONS: For meningiomas in the pineal region, 3D-CTA is of great clinical value to distinguish the anatomic relationship among the meningioma, blood supply and VC. This case study has strongly supported using Poppen's approach assisted by 3D-CTA to proceed with the operation.

Pilomyxoid astrocytoma in cerebellum.

Pilomyxoid astrocytoma is a new identified variant type of pilocytic astrocytoma, and typically locates in the hypothalamic and chiasmatic region. Herein, we reported a nine-year-old boy with pilomyxoid astrocytoma in the cerebellum. MRI scanning showed a tumor involved the cerebellar vermis, tonsil, the forth ventricle and brainstem. It was homogeneous isointensity on T1WI, relative hyper-intensity on T2WI, hyper-intensity on fluid attenuated inversion recovery (FLAIR) images, and uniform enhancement on contrast T1WI. The tumor was sub-totally removed and was proved histologically to be pilomyxoid astrocytoma. Follow-up at the 5th month, MRI showed the residual tumor enlarged at the brainstem. The patient survived 10 months after the operation, and finally died of respiration failure resulting from brainstem dysfunction.

Giant cell reparative granuloma in the temporal bone of the skull base: report of two cases.

Giant cell reparative granuloma (GCRG) in the temporal bone of the skull base is a very rare benign osteolytic lesion. Here, we report two cases that were initially misdiagnosed according to the patients' histories, clinical symptoms, and brain imaging prior to surgery. One case had a history of resection of a middle cranial fossa meningioma. The other case had a history of otitis media and mastoiditis. Pathological examination of the surgical specimens led to the diagnosis of GCRG for both cases. Both patients recovered well after surgical removal of the lesion without radiotherapy. Follow-up for 2 years indicated no recurrence of GCRG. These two cases support the hypothesis that repairing responses of bone tissue to either trauma or inflammation may underlie the pathogenesis of GCRG.

[Inorganic nitrogen and phosphate and potential eutrophication assessment in Liaodong bay].

On basis of field data measured during 4 cruises from June to September in 2002, variation feature of inorganic nitrogen and phosphate in Liaodong Bay was analyzed. Regression analyses of NH4+(-)N, NO3-(-)N, NO2-(-)N and PO4(3-)-P versus pH as well as inorganic nitrogen and phosphate versus salinity were also conducted. Finally, potential eutrophication of Liaodong Bay was assessed. It is shown that inorganic nitrogen and phosphate in Liaodong Bay vary with different months and different locations. In coast areas inorganic nitrogen and phosphate are mainly affected by rivers, while in central areas of the bay, they are subject to marine biochemical process. Ratio of inorganic nitrogen to phosphate rises to 60.6 from 52.5 in early June, then drops to 39, all far above Redfield value 16. Inorganic nitrogen and phosphate versus pH as well as salinity show different correlation. For example, the correlation coefficient of NO2(-)-N and pH even reaches 0.93. During 4 cruises in Liaodong Bay the total inorganic nitrogen is 212.0 microg/L, phosphate 10.1 microg/L, N/P 50.77. Potential eutrophication assessment indicates that during the survey period Liaodong Bay can be classified as a phosphate-limiting moderate-level nutrient area.