OBJECTIVETo study the clinicopathologic features of tuberous sclerosis complex (TSC).

METHODSThe clinicopathologic data of the patients diagnosed as TSC with refractory epilepsy and resection of epileptic focus were retrospectively analyzed.

RESULTSFourteen cases were included, the mean age was (15.8±12.9) years, with a male predominance (male to female ratio=10:4). Frontal lobe was the most common (13/14) site of involvement. MRI showed multiple patchy long T1 and long T2 signals. CT images showed multiple subependymal high density calcified nodules in nine cases. Histology showed mild to severe disruption of the cortical lamination, cortical and subcortical tubers with giant cells and/or dysmorphic neurons. The giant cells showed strong immunoreactivity for vimentin and nestin, while the dysmorphic neurons partially expressed MAP2 and NF. Vimentin also stained strongly the "reactive" astrocytes. Thirteen cases had follow-up information: Engel class I in six cases, Engel class II in six cases, and Engel class III in one case.

CONCLUSIONSDiagnosis of TSC relies on combined pathologic, clinical and neuroradiological features. Immunohistochemical staining can be helpful. Resection of epileptic focus is an effective method to treat refractory epilepsy in TSC.

Adolescent ; Astrocytes ; chemistry ; pathology ; Child ; Drug Resistant Epilepsy ; surgery ; Epilepsy ; complications ; metabolism ; pathology ; Epilepsy, Frontal Lobe ; complications ; metabolism ; pathology ; Female ; Giant Cells ; chemistry ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Nestin ; analysis ; Neurons ; metabolism ; pathology ; Retrospective Studies ; Tuberous Sclerosis ; complications ; metabolism ; pathology ; Vimentin ; analysis

Melorheostosis is a rare, progressive bone disease accompanied by hyperostosis and soft tissue fibrosis. While affected adults present with contracture and pain, children present with limb length discrepancy and deformity. We report the case of a 20-year-old woman with melorheostosis since childhood who presented with right hand deformity and numbness. Radiographs showed not only a combination of dense sclerosis and opacities, but also the classic 'flowing candle wax' appearance. Radiography can be used to identify melorheostosis, thus preventing unnecessary bone biopsies. Carpal tunnel release revealed the presence of a thickened flexor retinaculum and a degenerated median nerve distal to the retinaculum, but did not show hyperostosis. This case highlights the role of nerve decompression in melorheostosis and the importance of early identification of the disease to prevent unnecessary bone biopsies.
Carpal Tunnel Syndrome ; complications ; diagnostic imaging ; surgery ; Diagnosis, Differential ; Female ; Fibrosis ; pathology ; Hand ; pathology ; Hand Deformities ; diagnostic imaging ; surgery ; Humans ; Hypesthesia ; Median Nerve ; surgery ; Melorheostosis ; complications ; diagnostic imaging ; surgery ; Radiography ; Sclerosis ; diagnostic imaging ; Young Adult

PURPOSE: This study was conducted to assess the clinical relevance of diffusion tensor tractography (DTT) in pre- and post-operative evaluations of childhood epilepsy surgery. MATERIALS AND METHODS: Seventy-two patients who received epilepsy surgery between March 2004 and July 2008 were retrospectively analyzed (M : F=40 : 32, ages of 3 months to 24 years, mean age=8.9 years). DTT was performed using a 3.0 T scanner and single-shot spin-echo echo-planar imaging with 32-different diffusion gradient directions. We reviewed the data focusing on the type of surgery, final pathological diagnosis, and how the DTT data were clinically used. RESULTS: The most common form of childhood epilepsy surgery was complete resection of an epileptogenic lesion (n=52, 72.2%). The reported etiologies included cortical dysplasia (n=32, 44.4%), hippocampal sclerosis (n=9, 12.5%), brain tumors (n=7, 9.7%), and non-pathologic lesions (n=4, 5.6%) in the final diagnoses. Twenty-one dysplastic cortexes and four brain tumors involved an approximal relationship with the corticospinal tract (n=18), optic radiation (n=2), and arcuate fasciculus (n=5). Additionally, although DTT demonstrated white matter tracts clearly, DTT in the hippocampal sclerosis did not provide any additional information. In cases of callosotomy (n=18, 25%), post-operative DTT was utilized for the evaluation of complete resection in all patients. DTT information was not used in functional hemispherectomy (n=2, 2.8%). CONCLUSION: Preoperatively, DTT was a useful technique in cases of cortical dysplasia and brain tumors, and in cases with callosotomy, postoperatively. DTT should be included among the routine procedures performed in management of epilepsy.
Adolescent ; Adult ; Brain Neoplasms/pathology/surgery ; Child ; Child, Preschool ; Diffusion Tensor Imaging/*methods ; Epilepsy/*pathology/*surgery ; Female ; Hippocampus/pathology ; Humans ; Infant ; Male ; Retrospective Studies ; Sclerosis/pathology/surgery ; Seizures/surgery ; Treatment Outcome ; Young Adult

Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytosis, Sinus ; pathology ; surgery ; Humans ; Immunoglobulin G ; blood ; Paranasal Sinus Diseases ; pathology ; surgery ; Paranasal Sinuses ; pathology ; S100 Proteins ; metabolism ; Sclerosis ; pathology

OBJECTIVETo analyze the clinicopathologic features of chronic sclerosing submaxillaritis (CSS).

METHODSThe clinical and pathological characteristics of 9 CSS were analyzed.

RESULTSIn the 9 patients, there were 6 males and 3 females. The age of patients ranged from 51 - 77 years old. All of the tumors were located in the submandibular gland, presenting with painless and firm mass. Histologically, a well-defined mass lesion with extensive lymphocytes and plasma cells infiltration, preservation of lobular architecture, with acinar atrophy. The reactive hyperplasia of lymphoid follicles may be found in CSS. The phlebitis and obliterating phlebitis also formed. Immunohistochemistry showed evidence of diffuse infiltration of plasma cells. The mean number of IgG4-positive plasma cell per high-power field (HPF) was 186, mean value of the IgG4:IgG ratio was 0.71. Three of these 9 cases had manifestations of IgG4-associated systemic disease.

CONCLUSIONSCSS is considered as a part of IgG4-related sclerosing diseases, recognition of which is very essential for a successful treatment. When diagnosis is made, it is necessary to ascertain whether lesion occurs within salivary gland only or in combination with outside IgG4-related sclerosing disease. The establishment of follow-up is also necessary. Some patients show good response to steroid therapy.

Aged ; Female ; Humans ; Immunoglobulin G ; metabolism ; Male ; Middle Aged ; Plasma Cells ; immunology ; Sclerosis ; Sialadenitis ; metabolism ; surgery ; Submandibular Gland ; pathology ; surgery

Adult ; Diagnosis, Differential ; Female ; Fibroma ; metabolism ; pathology ; Fingers ; Giant Cell Tumors ; metabolism ; pathology ; Humans ; Mucin-1 ; metabolism ; Nerve Sheath Neoplasms ; metabolism ; pathology ; surgery ; Neurilemmoma ; metabolism ; pathology ; Sclerosis ; metabolism ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Tendons ; Young Adult

OBJECTIVE: To study the clinical characteristics and operative effect of hippocampus lesions. METHODS: We retrospectively analyzed the clinical characteristics and operative outcome of 44 patients with hippocampus lesions between August 2005 and April 2010. RESULTS: Seizure attack was the initial symptom among 40 of the 44 patients. Pathological examinations revealed 18 gliomas, 9 cavernous malformations, 12 hippocampus sclerosis, 2 focal cortical dysplasia, 1 atypical hyperplasia, 1 injury glial scar, and 1 encephalomalacia. Thirteen patients received anterior medial temporal lobectomy and the other 31 received lesionectomy or selective amygdalohippocampectomy via transsylvian approach. An average of 15.7 month follow-up was accomplished in 37 patients. Postoperative epileptic outcomes were evaluated according to Engel classification: Grade I 73.0%(27/37), Grade II 13.5%(5/37), Grade III 10.8%(4/37) and Grade IV 2.7%(1/37). No perioperative death occurred. One patient experienced hemiplegia but recovered 8 months after the operation. Noticeable postoperative visual field deficit was left in 2 patients. Two patients with glioma died of remote tumor recurrence during follow-up. CONCLUSION Seizure attack is a major complaint of hippocampus lesions. Satisfactory seizure and tumor control may be achieved through anterior medial temporal lobectomy or selective amygdalohippocampectomy with lesionectomy.
Adolescent ; Adult ; Brain Neoplasms ; surgery ; Child ; Child, Preschool ; Epilepsy ; surgery ; Female ; Glioma ; surgery ; Hippocampus ; pathology ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Sclerosis ; surgery ; Temporal Lobe ; surgery ; Treatment Outcome ; Young Adult

Child ; Epilepsy ; pathology ; surgery ; Humans ; Male ; Treatment Outcome ; Tuberous Sclerosis ; pathology ; surgery

Sclerosing encapsulating peritonitis (SEP) is a poorly understood and rarely documented cause of small bowel obstruction. Although recurrent peritonitis has been reported as the main contributory factor leading to secondary SEP, the pathogenesis of primary (idiopathic) SEP is still uncertain. A 40-year-old woman with a history of total abdominal hysterectomy due to gestational trophoblastic disease presented with progressive lower abdominal pain and abdominal distension. Ultrasonography and contrast-enhanced abdomen-pelvis computed tomography of the abdomen revealed encapsulation of the entire small bowel with a sclerotic capsule. At laparotomy, a fibrous thick capsule encasing small bowel loops was revealed. Extensive adhesiolysis and removal of the capsule from the bowel loops were performed. The patient recovered uneventfully; she was discharged without complications. SEP is a rare cause of small bowel obstruction. We treated a case of abdominal cocoon with intestinal partial obstruction in a woman with a history of abdominal hysterectomy due to gestational trophoblastic disease. Surgical treatment was effective and the patient recovered without complication.
Adult ; Female ; Humans ; Hysterectomy/*adverse effects ; Intestinal Obstruction/diagnosis/*etiology ; Intestine, Small/*pathology ; Peritonitis/diagnosis/*etiology/surgery ; Sclerosis/*pathology

OBJECTIVETo study the clinicopathologic features of sclerosing angiomatoid nodular transformation of spleen and its differential diagnosis.

METHODSThe clinicopathologic characteristics and immunophenotype of 4 cases of sclerosing angiomatoid nodular transformation of spleen were studied.

RESULTSHistologically, all cases were characterized by multiple angiomatoid nodules of various sizes in a fibrosclerotic stroma. The nodules were round and sometimes convoluted. They were composed of slit-like, irregular-shaped or slightly dilated vascular spaces lined by plump endothelial cells and interspersed with a population of spindly or ovoid cells. Immunohistochemical study showed a heterogeneous staining pattern, with the lining cells of the small capillaries expressing CD34 and those of the sinusoid-like structures expressing CD8. CD31 highlighted both the lining cells and interspersed cells, resulting in a complex meshwork. The lining cells were also focally positive for CD68. Smooth muscle actin revealed conglomerates of spindly shaped cells around and between the vascular channels. These spindly shaped cells in the intervening stroma were focally positive for actin, but negative for desmin, CD21 and CD35.

CONCLUSIONSSclerosing angiomatoid nodular transformation is a rarely encountered benign lesion of the spleen, which should be distinguished from other angiomatoid tumors and tumor-like lesions.

Adult ; Angiomatosis ; metabolism ; pathology ; surgery ; Antigens, CD34 ; metabolism ; CD8 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hamartoma ; pathology ; Hemangioma ; pathology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sclerosis ; pathology ; Spleen ; pathology ; Splenectomy ; Splenic Diseases ; metabolism ; pathology ; surgery ; Splenic Neoplasms ; pathology