Aortic Angiosarcoma Manifesting as Multiple Musculoskeletal Metastases: A Case Report.

Aortic angiosarcomas are rare. Due to its rarity and metastatic presentation, it is difficult to diagnose metastatic aortic angiosarcoma. We describe the clinicopathological and radiologic features of a metastatic aortic angiosarcoma presenting as musculoskeletal metastases. A 59-year-old male patient presented with left thigh pain. Plain radiographs revealed multifocal osteolytic lesions in the left femur shaft. Abdominopelvic computed tomography showed a lobulated osteolytic lesion in the left iliac bone. Magnetic resonance images revealed multifocal soft tissue lesions in the thigh musculature. A positron emission tomography/computed tomography (PET/CT) scan demonstrated multiple foci of increased uptake in the left femur bone, pelvis, left thigh, and calf musculature. Focal increased uptake in the lower abdominal aorta was newly detected. Pelvis biopsy showed tumor cell nests of epithelioid cells. The tumor cells showed vasoformative features. Immunohistochemically, the tumor cells showed positivity for vimentin, CD31, and ERG. The pathologic diagnosis of epithelioid angiosarcoma was established. The origin of the tumor was presumed to be the aorta. This case underscores the importance of PET scans in identifying primary lesions. In terms of the histopathologic diagnosis of biopsy samples with tumor cells exhibiting epithelioid neoplastic morphology, employing appropriate ancillary techniques such as immunocytochemistry with vascular markers may assist in accurately diagnosing metastatic angiosarcoma.

Histological findings of regeneration in retrieved pasteurized bone grafts.

BACKGROUND: Despite a number of radiologic evaluations of the incorporation of pasteurized bone (PB) in human and histologic evaluations in animal models, there has been a scarce documentation regarding the histologic evaluation of PB from human. Herein, we present histologic findings of regeneration in retrieved PB graft from pediatric and adult patients. METHODS: PB was retrieved for various reasons in 7 patients (10-52 years old). Two bone pathologists independently counted the number of empty lacunae and lacunae with living cells in up to 10 randomly selected fields on medium-power (H&E, ×200) for each patient. Regeneration of PB was assessed as the ratio of the number of lacunae with nucleated cells to that of whole lacunae, which was defined as the "repair rate (RR)". RESULTS: The mean interval between initial reconstruction and retrieval (graft removal time; GRT) was 47.4 months (range, 11-144 months). The length of original PBs ranged from 5.8 to 20.6 cm. Microscopic examination of PBs showed areas with empty lacunae indicating necrosis and other areas contained lacunae with nucleated osteocytes, indicative of regeneration. Some Haversian canals of the PBs were filled with fibrovascular tissue and surrounded by lamellar bones including living osteocytes. RR varied widely from 21.7 to 62.4% with a mean of 36.8%. It was much higher in adult patients (46.6-62.4%, mean = 55.3%) than in pediatric patients (21.7-28.6%, mean = 25.3%), which was correlated with GRT (pediatric patients; mean of 14 months, adult patients; mean of 72.3 months). In adult patients, RR was higher in a patient with prosthesis composite in the proximal humerus (Case No. 3; 62.4%). CONCLUSIONS: RR was higher in whom GRT was longer, being correlated with GRT in retrieved PBs. In terms of our histological observation, PB is thought to be an acceptable temporary biologic spacer in limb-sparing surgery for malignant bone or soft tissue tumors.

Thymic carcinoma initially presented with geographic destruction of scapula in a child.

As the conventional histopathologic examination of thymic carcinoma (TC) is nonspecific, immunohistochemical studies along with correlative radiographic investigations are needed for its correct diagnosis. TC commonly occurs in the late 5th to early 6th decades of life but is extremely rare in childhood. It may be incidentally detected from chest radiographs taken as routine or for other reasons. However, most patients present with symptoms such as chest pain, cough, shortness of breath, dysphagia and hoarseness, which are directly attributable to the mediastinal mass. Although TC frequently invades the neighboring organs, pleura and pericardium and metastasizes to the lymph nodes, liver and lung at the time of the first diagnosis, initial or late metastasis to the bone has been seldom reported in adults. Indeed, the English literature revealed no earlier report on initial bony metastasis in a child to date. We report a case of TC in a 12-year-old boy who initially presented with scapular osteolysis masquerading as a primary bone tumor to emphasize the usefulness of combined imaging for staging and histologic studies, particularly for such an unexpected case.

Radiologic healing of lateral femoral wall fragments after intramedullary nail fixation for A3.3 intertrochanteric fractures.

INTRODUCTION: Intramedullary nail fixation is a useful treatment option for A3 intertrochanteric fractures. Occasionally, we have encountered displaced lateral femoral wall (LFW) fragment during surgery with intramedullary nail system. We investigated the postoperative spontaneous reduction of displaced LFW fragments without further fixation and the factors that affected the spontaneous reduction of displaced LFW fragments. MATERIALS AND METHODS: Forty-four patients with A3.3 intertrochanteric fracture were treated by surgery using intramedullary nails (PFNA; Synthes, Paoli) between March 2007 and December 2012. All patients had a minimum follow-up period of 12 months. We calculated the amount of spontaneous reduction of the displaced LFW fragments from immediate postoperative and last follow-up anteroposterior radiographs. We measured the tilting angle of the LFW fragment, tip-apex distance (TAD), and telescoping of the blade, and evaluated the quality of postoperative reduction. RESULTS: Twenty-five of the 44 patients had displaced LFW fragments, and the average amount of spontaneous reduction of the displaced LFW fragment was 4.8 mm with statistical significance (p = 0.005). The average tilting angle of all patients was -4.97°, telescoping was 6.83 mm, and TAD was 19.77 mm. Twenty-one patients had good quality of reduction, 21 had acceptable quality, and 2 had poor quality. Multivariate logistic regression analysis for these factors indicated that tilting angle was the only significant factor related to spontaneous reduction of a displaced LFW fragment (p = 0.007, odds ratio = 1.336). CONCLUSIONS: In intramedullary nailing of A3.3 intertrochanteric fractures, the displaced LFW fragments tend to reduce spontaneously without any additional fixation during the postoperative period. We conclude that no additional fixation is needed for the displaced LFW fragment after surgery with intramedullary nail.

Differential diagnostic value of "blue reticulated chondroid-like material" in aneurysmal bone cysts: a classic histopathologic analysis of 215 cases.

OBJECTIVES: Our classic histopathologic study of aneurysmal bone cyst (ABC) revealed that "blue reticulated chondroid-like material" (BRC) is characteristic of ABC. METHODS: The light microscopic findings were retrospectively analyzed in 215 cases of ABC, including 101 primary and 114 secondary cases. In addition, 22 cases of telangiectatic osteosarcoma (TOS) were drawn from the same source and used as a control. RESULTS: We found the presence of typical BRC in 24 (23.8%) of 101 cases of primary ABC and in six (5.3%) of 114 cases of secondary ABC, with an overall incidence of 30 (14%). None of the cases of TOS showed BRC. BRC was significantly more common in primary ABC than in secondary ABC (P < .05) and in patients 19 years or younger than in those 20 years or older (P < .05). CONCLUSIONS: BRC appears to be a unique histopathologic feature of ABC, making it valuable to differentiate benign ABC from TOS. Simple H&E stain can be economically performed anywhere.

A case of pulmonary metastasis of giant cell tumor of bone presenting as pulmonary arteriovenous malformation.

Although giant-cell tumor (GCT) of the bone was originally classified as a benign tumor, metastasis has been reported. The radiographic features usually comprise parenchymal solitary or multiple nodules that are round-to-oval nodular opacities of homogeneous density in patients with GCT. However, the patient described in this case presented with a hypervascular mass with feeding vessels and hemothorax, which are common features of pulmonary arteriovenous malformation. To the best of our knowledge, cases of pulmonary metastases presenting as a pulmonary arteriovenous malformation have not been reported. Here, we report a case of giant-cell tumor of the bone that exhibited histologically benign pulmonary metastases and mimicked an arteriovenous malformation.

Huge tophaceous pseudogout associated with tenosynovial chondromatosis arising from flexor digitorum tendon sheaths of the foot: a case report.

Synovial chondromatosis (SC) is a benign proliferative process of synovial tissue creating multiple cartilaginous nodules in joints. It most commonly occurs in the large joints of the knee, hip, and shoulder, uncommonly in the small joints of the hand and foot, and only rarely in the tenosynovial membrane of tendon sheath, termed tenosynovial chondromatosis (TC). Unlike SC, TC predisposes to the foot or hand. The rarity and unfamiliarity of imagers with TC, as well as the variability of its histologic features often lead to an erroneous diagnosis of extraskeletal chondroma or even chondrosarcoma as in the present case. Calcium pyrophosphate dehydrate (CPPD) crystals are usually deposited in the articular cartilage or periarticular structures such as synovium and capsule, and rarely in other soft tissue structures including bursa, tendon, subcutaneous tissue, and dura mater. CPPD crystals may also be deposited in extraskeletal chondroma and SC. We present an exceptionally rare case of huge tophaceous pseudogout associated with TC that is considered to arise from the flexor digitorum longus tendon sheaths of the foot, initially mistaken for a chondrosarcoma.

Metastatic malignant tumour in the hand.

Skeletal metastasis is a common event during the advanced stage of a malignant tumour, but metastasis to the hand is rare (about 0.1% incidence). We have seen seven cases of metastatic malignant tumour of the hand since 1983, and report them here.

Depiction of Nidi and Fibrovascular Zones of Osteoid Osteomas Using Gamma-Correction Tc-99m HDP Pinhole Bone Scan and Conventional Radiograph, and Correlation with CT, MRI, and PVC Phantom Imaging.

PURPOSE: For the precise imaging diagnosis of osteoid osteoma (OO), the identification of the nidus and fibrovascular zone (FVZ) is essential. However, the latter sign has received little attention because it is difficult to demonstrate. We applied the recently introduced gamma correction (GC) to depict the FVZ on pinhole bone scan (PBS), conventional radiography (CR), and computed tomography (CT). Non-gamma correction MRI was also analyzed for reference. METHODS: Ten patients with histologically proven diagnoses of OO were enrolled in this retrospective study. PBS, CR, and CT were processed by GC to demonstrate the nidi and FVZ as distinct yet integrating components of OO. PBS was performed using a 4-mm pinhole collimator 3 h after iv injection of 925 to 1,110 MBq (25 to 30 mCi) of Tc-99m HDP, and anteroposterior and mediolateral CR and transverse CT were taken according to the standard technique. MRI sequences included T1- and T2-weighted images. For gamma correction, we utilized the Photo Correction Wizard program of ACD Photo Editor v3.1. A team of three qualified nuclear physician-radiologists, two nuclear physicians, and one MRI specialist read bone scans, radiographs, and MRIs of OO according to each specialty, and orthopaedic aspects and histology were reviewed by one qualified orthopedic surgeon and two qualified pathologists, respectively. Each observer first read the images separately with basic information about the aim of the study given and then in concert. Interpretive disagreement was settled by discussion and consensus. RESULTS: On pinhole scan, nidi were presented as areas of intense tracer uptake in all cases, and, importantly after GC, a thin ring-like zone with lower tracer uptake became visible in seven out of ten cases. GCCR also revealed a thin lucent zone that circumscribed the nidi in six out of ten cases and GCCT in two of four cases. MRI, without GC, presented nidi with high signal in the center and a thin ring-like zone with low signal in the periphery in five out of six cases. Ring-like zones were 1-2 mm in thickness and circumscribed the nidus as an integrated part and, hence, were morphologically interpreted as FVZ. Histologically, the presence of a variously mineralized FVZ was confirmed in four cases, but individual locus-by-locus image-histology correlation could not be accompolished because specimens were fragmentary. In the FVZ, tracer uptake was lower than in nidi, presumably reflecting that bone metabolism in the two parts differs as in their histology. Statistically, no significant correlation existed between the duration of symptoms and imaging demonstrability of the FVZ (Spearman's test r = -0.057, p = 0.877), but parallelism existed in the demonstrability of the FVZ among GC PBS, CR, and CT, and non-correction MRI. CONCLUSIONS: GC was useful to enhance the resolution of PBS, CR, and CT in OO so that both the nidi and FVZ were separately imaged. The use of CG PBS and CR in combination is recommended for the specific diagnosis of OO with information about bone metabolism and anatomical characteristics. PBS and CR are economical and widely available.

Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings.

Extraskeletal Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are widely regarded as clinically and histologically identical tumors which consist of small blue round cells. Extraskeletal ESs/PNETs usually occur in the deep soft tissues of the paraspinal region, chest wall, or lower extremities. However, superficially located cases, so-called cutaneous ESs/PNETs, are exceedingly rare, and the vast majority of the reported cases present as a single small mass. We present magnetic resonance imaging (MRI) findings and clinical course of a unique case of primary cutaneous ES/PNET presenting as numerous huge masses with severe ulceration on them.

Kimura's disease involving a long bone.

Kimura's disease is a rare, benign lymphoproliferative disorder of unknown etiology. It occurs most often in Asian men, usually in the second or third decade of life. Most lesions occur in the head and neck followed by the axilla, groin, popliteal region, and arm. The lesions are commonly found in soft tissues. To the best of our knowledge, there has been only one case report of bone involvement in Kimura's disease presented on plain radiography. We report a case of Kimura's disease that involved the proximal meta-diaphysis of the humerus and adjacent soft tissue shown on radiography and MR imaging.

Dysplasia epiphysealis hemimelica: radiographic and magnetic resonance imaging features and clinical outcome of complete and incomplete resection.

OBJECTIVES: The objectives of this communication were to discuss radiographic and magnetic resonance (MR) imaging manifestations and clinical outcome after complete and incomplete resection of the mass of dysplasia epiphysealis hemimelica (DEH). MATERIALS AND METHODS: Clinical records, radiographs, and MR images of eight patients with DEH were retrospectively examined. Six patients were treated by complete excision of the lesional mass, and two patients were treated by incomplete resection at our University Hospitals during the period from 1980 to 2006. RESULTS: We found that, unlike in osteochondroma, DEH was radiographically not clearly separable from the underlying or host bone with preserved cortical bone and marrow continuity. The finding in the talus distinguished DEH from (osteochondroma-like) parosteal osteosarcoma, in which a radiolucent demarcation line clearly separated the tumor from the host bone. The DEH mass had a well-defined low to intermediate signal intensity on T1-weighted images and an intermediate to high signal intensity on T2-weighted images, with irregularity of the articular surface. Simple excision was performed in all patients. The excision was complete in six patients and incomplete in two patients whose lesions was juxta-articular in the ankle and articular in the knee, respectively. The residual mass slowly absorbed and vanished, resulting in mild flaring of the affected portion of the epiphysis. No local recurrence or complication was seen in any of the eight patients. CONCLUSIONS: Although the radiographic signs of DEH are characteristic, (osteochondroma-like) parosteal osteosarcoma should be differentiated from DEH when there is a radiolucent separation line between the mass and host bone in the talus. Simple excision was effective in the management of DEH if the deformity was not complicated. Incompletely excised masses resolved and vanished with time.

Chromomycosis presenting as soft-tissue mass: report of a case with MRI features.

Chromomycosis is primarily a skin disease that superficially presents as slowly growing, verrucous lesions, often warty or cauliflower-like in appearance. It may occasionally create a flat, plaque-like lesion in the skin but deep-seated tumorous presentation has not previously been reported. As the lesion is limited to the cutaneous and superficial subcutaneous tissues, hitherto reported cases have been described from the view point of dermatology and, so, without MRI study. We report a patient with pathologically proven chromomycosis that produced a subcutaneous mass in the dorsum of the hand with an emphasis on MRI features.

Treatment of osteonecrosis of the femoral head using autologous cultured osteoblasts: a case report.

INTRODUCTION: Osteonecrosis of the femoral head is a progressive disease that leads to femoral head collapse and osteoarthritis. Our goal in treating osteonecrosis is to preserve, not to replace, the femoral head. CASE PRESENTATION: We present the case of a patient with bilateral osteonecrosis of the femoral head treated with autologous cultured osteoblast injection. CONCLUSION: Although our experience is limited to one patient, autologous cultured osteoblast transplantation appears to be effective for treating the osteonecrosis of femoral head.

Cystic fibrous dysplasia in the long bone.

Prominent osteolysis associated with "ground glass" density of fibrous dysplasia may indicate cystic change or sarcomatous transformation. This complication has been reported only sporadically in the long bones. This article presents clinical, radiographic, and pathologic findings, and outcome of simple curettage and bone graft observed in a series of 8 patients with prominent cystic fibrous dysplasia of the long bone. Magnetic resonance imaging features provide a basis for separation of benign cystic change from malignant transformation. However, biopsy is necessary to distinguish nonspecific cystic degeneration from secondary aneurysmal bone cyst. Simple curettage with allo-chip-bone graft is an effective treatment for cystic fibrous dysplasia.

Gastric cancer acrometastases to all digits of one hand following closed intramedullary nailing.

Metastatic malignant tumor of the hand is unusual and the most common site of the primary cancer is lung. Acrometastases to the phalanges of the hand usually involve a single bone, while those proximal to the phalanges often involve multiple bones. Metastasis to the hand from gastrointestinal cancer, particularly from gastric cancer, is extremely rare. To our knowledge, only five cases have been reported in the literature to date and there has been no reported case following closed intramedullary nailing for metastatic diaphyseal fracture of an ipsilateral long bone. We present a very unusual case of gastric cancer, which metastasized to all fingertips of the ipsilateral hand after closed interlocking intramedullary nailing of a pathologic fracture of the humerus.