Intraneural granular cell tumor: Histologic spectrum and histogenetic implication.

Granular cell tumor (GCT), despite its putative neural origin, commonly occurs in extraneural sites; only six single case reports of intraneural GCT have been described. We report an unique case as the only one with motor dysfunction, the longest duration of symptoms, and largest tumor size with resultant muscle atrophy and fatty infiltration. Upon review of these cases, the striking feature of intraneural GCT lies in its histologic variety, ranging from benign GCT, through plexiform, hybrid GCT and perineurioma to malignant GCT, encompassing the full spectrum of extraneural GCT. As the immunophenotypes of schwannoma and GCT are not exactly identical, together with evidence from ultrastructural studies, the more reasonable and likely possibility is that GCT originates from undifferentiated mesenchymal cells acquiring partial schwannian differentiation. Such postulation by virtue of the widespread occurrence of mesenchymal cells in the soft tissue, better explains the topographic distribution of GCT in extraneural and intraneural location.

Critical reappraisal of primary osseous composite sarcoma (malignant mesenchymoma) - analysis of four cases and literature review.

In accordance with recent terminology, it is proposed that malignant mesenchymoma should be renamed 'composite sarcoma' and defined as 'a sarcoma composed of two or more cellular types each of which is sufficiently differentiated to permit clear recognition of its histogenetic type microscopically, immunohistochemically or ultrastructurally; excluding fibrosarcomatous and high-grade pleomorphic undifferentiated sarcomatous component, dedifferentiated sarcoma and the combination of osteosarcoma and chondrosarcoma which is regarded as a single histogenetic type'. Four cases of primary osseous composite sarcoma (POCS) were identified among 928 primary bone sarcomas. Their age ranged from 10 to 87 years, peak incidence in the second decade with equal sex distribution. Most presented with pain, commonest in the knee, affecting the metaphysis, appearing radiologically as expansile infiltrative osteolytic lesions with cortical erosion, periosteal reaction, variable extent of osteoblastic areas and soft tissue extension. All contained variable amounts of conventional high-grade osteosarcoma with or without chondrosarcoma component; the other constituents were liposarcoma, rhabdomyosarcoma and leiomyosarcoma. In all cases, Ki67 proliferative index was over 35%, there was no CDK4 and MDM2 amplification. The absence of low-grade component supported the de novo origin of POCS rather than derivation from divergent dedifferentiation. The two older patients with hitherto undescribed osteoleiomyosarcoma died 2 and 10 months after operation, whereas the two younger with osteorhabdomyosarcoma and osteoliposarcoma enjoyed disease-free survival at 16 and 6 years after chemotherapy despite the latter showing lung metastasis at presentation. Identification of the different lines of differentiation together with their approximate amounts and histological grades is therefore mandatory for POCS as multi-agent chemotherapy catered for each sarcoma component might offer hope for long-term disease-free survival.

Fibular giant cell-rich osteosarcoma virtually indistinguishable radiographically and histopathologically from giant cell tumor-analysis of subtle differentiating features.

Giant cell-rich osteosarcoma by its abundance of osteoclastic giant cells and paucity of tumor osteoid, leads to its easy confusion with giant cell tumor during biopsy interpretation. In this report, we describe a unique case of upper fibular metaphyseal giant cell-rich osteosarcoma in a 12-year-old boy; the radiographic and histopathologic features of the biopsy and initial resected tumor are virtually indistinguishable from conventional giant cell tumor. The tumor rapidly recurred 7 months after resection with metastasis to the groin lymph nodes, was resistant to first-line chemotherapy and pursued an aggressive course, developing disseminated metastasis to the lung, liver, pelvis, scapula and clavicle, and resulted in the death of the patient 21 months after initial presentation. The subtle features alerting one to the possibility of giant cell-rich osteosarcoma are retrospectively evaluated in comparison with cases of metaphyseal conventional giant cell tumors, four from our records and those from literature review. We conclude that the occurrence of a giant cell-rich lesion in the metaphysis of a skeletally immature individual merits careful assessment for the presence of periosteal reaction, permeative infiltrative margins, lacelike osteoid formation, high mitotic activity or Ki67 proliferative index, and extra-tumoral lymphovascular permeation, since the possibility of an aggressive lesion notably giant cell-rich osteosarcoma probably increases with the number of such features.

US assessment of medial arterial calcification: a sensitive marker of diabetes-related microvascular and macrovascular complications.

PURPOSE: To examine the sensitivity of ultrasonography (US) compared with conventional radiography in detection of lower limb (thigh) medial arterial calcification (MAC) in type 2 diabetic patients and evaluate its association with diabetes-related complications. MATERIALS AND METHODS: The study was approved by the local research ethics committee, and informed written consent was obtained. US was performed in 289 patients with type 2 diabetes mellitus, and MAC severity was assigned a score from 0 to 8. Among the patients, 263 underwent radiographic examinations. All subjects underwent clinical evaluation to detect the presence of diabetes-related complications. RESULTS: US helped detect MAC in more subjects compared with radiography (65.8% vs 12.2%). US helped detect MAC from mild (scores 1-4) to severe (scores 5-8) degrees, while mild degree of MAC was poorly demonstrated with radiography. The incidence of nephropathy, retinopathy, sensory neuropathy, and macrovascular complications increased with the severity of MAC (based on US scoring). With univariate analysis, the presence of MAC was associated with nephropathy (P<.001), retinopathy (P<.001), sensory neuropathy (P=.004), and macrovascular complications (P<.001). After adjustment for potential confounders, the presence of severe MAC was associated with nephropathy, retinopathy, and macrovascular complications, with the odds ratios of 3.4 (95% confidence interval [CI]: 1.53, 7.43; P=.003), 2.6 (95% CI: 1.22, 5.32; P=.013), and 3.8 (95% CI: 1.37, 10.6; P=.01), respectively. CONCLUSION: In type 2 diabetic Chinese patients, US was more sensitive than conventional radiography in the detection of MAC, particularly when the MAC was mild. The presence of severe MAC was associated with diabetic nephropathy, retinopathy, and macrovascular complications. US detection of MAC was a potential early marker to identify diabetes-related complications.

Primary pleomorphic liposarcoma of bone in an adolescent: imaging features of a rare entity.

We report a rare case of a primary osseous pleomorphic liposarcoma of the distal femur in a 13-year-old boy. Primary liposarcoma of bone is a very rare malignant tumour originating from primitive mesenchymal cells rather than mature adipose tissue. Pleomorphic liposarcoma is the least common of the eight known histological subtypes in the WHO classification. The entity is not previously reported in children. This is the third reported case of pleomorphic liposarcoma of the bone, and the first reported case presenting in adolescence. We describe the computed tomography and magnetic resonance imaging features of this rare bony tumour.

Intraosseous glomus tumor treated by toe-to-finger transfer: case report.

We report a case of intraosseous glomus tumor that developed in the right ring finger distal phalanx of a 19-year-old man. Clinical and radiographic findings were atypical. The tumor was excised en bloc because of the extensive involvement. The left second toe was transferred to the right ring finger to reconstruct the excised finger part. Symptom relief, function, and cosmetic outcome were satisfactory during 2-year follow-up.

Differential, time-dependent effects of perivenous application of fibrin glue on medial thickening in porcine saphenous vein grafts.

OBJECTIVE: Neointimal and medial thickening play a critical role in late vein graft failure following CABG. Previous ex vivo experiment suggested that perivenous application of fibrin glue may reduce the damage in the circular smooth muscle cell layer of the media of the vein graft shortly after exposing to arterial pressure. However, the in vivo as well as the longer term impact of this intervention remain unknown. METHODS: Bilateral saphenous vein-carotid artery interposition grafting was performed in eight large white pigs (35-45 kg). In each pig, one of the grafts was randomly selected to receive perivenous fibrin glue support while the contralateral graft served as control. At 1 and 4 months following surgery (n=4 pigs in each group), all 16 patent vein grafts were removed and pressure-fixed. Multiple histological sections from each graft were prepared. Proliferating cell nuclear antigen (PCNA) was detected by immunocytochemistry. Vein graft morphology was assessed using computer-aided planimetry. RESULTS: Although perivenous application of fibrin glue had little effects either on medial thickness 1 month after implantation or on PCNA index, it significantly increased medial thickness (control: 0.37+/-0.02 mm; treated: 0.55+/-0.02 mm, p<0.001) and total wall thickness (control: 0.75+/-0.04 mm; treated: 0.92+/-0.04 mm, p=0.008) at 4 months (mean+/-SEM; n=4 in each group). CONCLUSIONS: Our data indicated that perivenous application of fibrin glue enhances graft thickening and as such does not constitute a strategy for preventing late vein graft failure after CABG.

Primary osteochondrorhabdomyosarcoma (malignant mesenchymoma) of the fibula: a rare tumour in an unusual site -- case report and review of the literature.

Malignant mesenchymoma, defined by Stout as sarcomas comprising two or more unrelated differentiated tissue elements other than a fibrosarcoma component, is rare. We report a case of primary malignant mesenchymoma of the proximal fibula in a 10-year-old female student who presented with pain and swelling of the right knee for 2 months. Initial biopsy showed features of rhabdomyosarcoma only, but the resected specimen revealed additional osteosarcomatous and chondrosarcomatous elements. The patient remained well more than 5 years after initial presentation. Including our present patient, 16 cases of primary malignant mesenchymoma of bone are found in the English literature, affecting mainly adolescents and young adults, with a slight male predominance and predilection for the metaphysis of long bones, especially around the knee. More than 60% of the patients develop metastasis, almost invariably to the lung, but occasionally to the brain. About 60% of the patients, all with metastasis, died mostly within one year of diagnosis. The clinical features of primary malignant mesenchymoma of bone thus resemble those of conventional osteosarcoma. Moreover, our case illustrates that, with combination chemotherapy targeted for individual elements, the prognosis of this rare tumour might be much improved, as in osteosarcoma.

Papillary endothelial hyperplasia within synovial haemangioma of the flexor tendon sheath of the wrist.

A 60-year-old housewife presented with a painful and slowly enlarging swelling in the left wrist for 3 months. Plain X-ray showed mild soft tissue swelling and ultrasonography tenosynovitis of the flexor tendons. Exploration revealed a vascular growth involving the synovium of the flexor tendon sheath of the left little finger. Synovectomy and excision of the entire growth led to the diagnosis of synovial haemangioma with areas of recent haemorrhage and florid papillary endothelial hyperplasia. The recent haemorrhage corresponded to the sudden increase in size, while the papillary endothelial hyperplasia accounted for the persistence and gradual enlargement of the lesion. The patient made an uneventful recovery and remained well more than 2 1/2 years after the operation.

Cystic pulmonary metastases from epithelioid cell sarcoma.

A patient with cystic pulmonary metastases, from epithelioid sarcoma of the thigh, resulting in bilateral pneumothoraces is presented. The pulmonary metastases superficially resembled pneumomatoceles and were not visible on radiography. Computed tomography examinations over a 10-month period showed no change in the size or number of the cystic metastases, though air fluid levels and pericystic nodular consolidation (probably due to pulmonary hemorrhage) did occur and regress. This case report serves to emphasize that bullous type lesions on thoracic computed tomography in patients with a known sarcoma should be interpreted with particular caution.

Massive intestinal hemorrhage resulting from a polypoid tumor in the sigmoid colon: an unusual complication of a giant cell tumor of the fifth lumbar vertebra.

STUDY DESIGN: Clinicopathologic study of a case of giant cell tumor of the spine. SUMMARY OF BACKGROUND DATA: Giant cell tumors of the spine are uncommon, accounting for 1.3-6.5% of all cases in various series. Because of their location, they may cause neurologic deficits. The treatment consists of excision or curettage and has been claimed to give good results. METHODS: A 33-year-old woman presented with low back pain in 1995; radiologic investigation and biopsy showed features of giant cell tumor involving the fifth lumbar and first sacral vertebrae. Wide excision was performed, but the tumor recurred in 1996 and was curetted. She developed massive intestinal bleeding in 1997 resulting from infiltration of the sigmoid colon by giant cell tumor in the form of a polypoid intraluminal mass. The involved segment of colon was resected, and the patient remained alive, although debilitated, 7 years after initial presentation. RESULTS: Examination of the tumor in the spine and the colon showed typical histology of giant cell tumor with no evidence of malignant transformation. The involved colon was freely mobile and away from the tumor of the spine. CONCLUSION: Giant cell tumor of the spine can result in unusual complication, massive intestinal hemorrhage in our case, which causes considerable morbidity.

A new technological approach to nonanatomical pulmonary resection: saline enhanced thermal sealing.

BACKGROUND: This is the first clinical report on the feasibility study of two new devices (monopolar Floating Ball and bipolar Sealing Forceps; TissueLink Medical Inc, Dover NH) that incorporated the novel technology of saline enhanced thermal sealing. METHODS: From December 2000 to December 2001, 25 patients (mean age, 54.8 years) with peripheral lung nodules planned for either diagnostic or therapeutic wedge resection were recruited for the study. When the nodule lay deep to a flat lung surface, video-assisted thoracic surgical resection using the modified Perelman technique with the Floating Ball (TissueLink Medical Inc) was preferred. In other patients, the Sealing Forceps (TissueLink Medical Inc) were used for video-assisted thoracic surgical wedge resection. RESULTS: There were no mortality or major intraoperative complications. The Floating Ball was used exclusively in 11 patients; the Sealing Forceps were used in 9 patients; and a combination of the two devices was used in 5 patients. The mean operation time was 70.3 minutes. Average chest drain duration was 3.9 days, and postoperative hospital stay was 5.2 days. There were 2 patients with persistent air leak more than 1 week, one who resolved spontaneously, and the other who required reoperation for control. One patient had pulmonary embolism after a technically uneventful procedure. There have been no late complications after an average follow-up of 10 months. CONCLUSIONS: The devices appear to be technically safe. The Floating Ball has definite advantages over the conventional diathermy and can be adapted to the Perelman procedure using the video-assisted thoracic surgical approach. The Sealing Forceps hold promise to reduce overall consumable costs compared with conventional staplers. These devices should complement the surgeon's existing armamentarium. Comparative studies with conventional instruments are warranted to further define the role of these new devices in thoracic operations.

Imaging of musculoskeletal tuberculosis: a new look at an old disease.

There are certain imaging features that help to differentiate tuberculosis from other bone and joint disorders with a similar presentation. The current authors discuss these distinguishing imaging features particularly with respect to ultrasound, computed tomography, and magnetic resonance imaging. The judicious and appropriate use of these newer imaging modalities coupled with aspiration or biopsy can lead to earlier recognition of musculoskeletal tuberculosis before the onset of debilitating disease.

Closed retrograde nailing of pathological humeral fractures.

We treated 19 patients with 21 pathological humeral fractures secondary to metastatic disease. All patients were stabilized using retrograde Ender's nails. Open reduction was required in one patient and mean operative time was 40 min (range 30-65 min). All patients retained satisfactory elbow and shoulder function while 16 patients reported good pain relief. Functional recovery was rated as good in 15 patients, fair in three, and poor in one. In our experience the retrograde technique using Ender's nails provides secure stabilization with minimal morbidity, minimal blood loss, and short operative time, and is well suited for this category of patients.

Scope and limitations of limb-sparing surgery in childhood sarcomas.

The authors reviewed 43 children with high-grade sarcomas of bone and soft tissue who underwent surgery during the past 9 years. Twenty-six patients had osteosarcoma, 7 had Ewing sarcoma, and 10 had soft tissue sarcomas. Patients ranged in age from 4 months to 13 years. Mean follow-up period was 6 years. The feasibility of resection, results of local resection, and failure of local disease control were reviewed. Limb salvage was feasible in 81% of patients. Of the 35 patients undergoing limb salvage, margins were considered adequate in 88% and inadequate in 12%. Limb-sparing resection was not feasible in 19% of patients. Five patients (four with metastatic disease at presentation) did not undergo surgery and were given palliative care; three other patients underwent amputation. The decision of whether to proceed with limb salvage surgery must depend on the aggressiveness of the underlying tumor, its stage, the feasibility of obtaining tumor-free resection margins, and the response to neoadjuvant therapy.