Neoplastic degeneration in Paget's disease is a rare complication (approximately 1% of cases) and, despite the treatment, presents a poor prognosis. The authors report a case of a male, 82-year-old patient with long standing Paget's disease who presented imaging findings of malignant degeneration in the calcaneus histopathologically diagnosed as sarcomatous degeneration.

Keywords: Paget's disease, Sarcoma, Sarcomatous degeneration, Calcaneus, Osteitis deformans

Kaposi sarcoma is a neoplasm associated with immunosuppressive conditions, and involving blood and lymphatic vessels. It is the most frequent intrahepatic neoplasm in patients with acquired immunodeficiency syndrome. Computed tomography and magnetic resonance imaging demonstrate multiple small nodules, prominence and contrast-enhancement of periportal branches due to the presence of the neoplastic tissue. The authors report a case of a 47-year-old male patient with acquired immunodeficiency syndrome presenting disseminated Kaposi sarcoma.

Keywords: Kaposi sarcoma, Acquired immunodeficiency syndrome, Liver disease, Cancer, Computed tomography, Magnetic resonance imaging

OBJECTIVE: To investigate and describe clinical, radiologic and pathologic findings of gastrointestinal stromal tumors. MATERIALS AND METHODS: In the period between December 2000 and March 2006, 16 patients were submitted to surgery for gastrointestinal stromal tumors in our institution. The following variables were taken into consideration: sex and age, signs and symptoms at presentation, tumor site and size, radiological and pathological features, and presence of metastasis. RESULTS: The study population was constituted by nine men and seven women. The primary tumor sites of origin were: stomach (n = 5), rectum (n = 4), small bowel (n = 3), mesentery (n = 3), and colon (n = 1). Mean primary tumor size was 9 cm. Computed tomography was the main radiological method utilized. Circumscribed, lobulated and heterogeneously contrast-enhanced mass was the main image finding. Metastasis was found in nine patients (56% of cases) at presentation or tumor recurrence was observed during the follow-up period (mean = 32 months). CONCLUSION: Gastrointestinal stromal tumor occurs in middle-age adults and the elderly, and must be taken into consideration as differential diagnosis for abdominal masses. Early diagnosis, adequate therapy, and rigorous follow-up are essential, considering the high probability of malignancy of these neoplasms as demonstrated by the present study.

Keywords: Gastrointestinal stromal tumor, Gastrointestinal neoplasms, Gastrointestinal diseases, Sarcoma

OBJECTIVE: To review the clinical and radiographic findings in patients with histologically confirmed Ewing's sarcoma. MATERIALS AND METHODS: Clinical and radiological (whenever available) data of 226 patients with Ewing's sarcoma of the bone were analyzed. RESULTS: Of the evaluated cases, 61.5% (139) were male and 83.7% (189) were white. The average age was 14 years old and the most common site of the lesions was the iliac bone, seen in 13.7% (31) of the cases. The most common radiological findings were lytic lesion with periosteal reaction, seen in 32.7% (74) of the patients. CONCLUSION: Ewing's sarcoma of bone is an aggressive neoplasm, occurring mainly in the second decade of life, which main radiological findings are lytic lesions with periosteal reaction, typical characteristic of aggressive lesions.

Keywords: Conventional radiology, Ewing's sarcoma, Bone neoplasms

We report the case of a 23-year-old female patient with a low-grade fibromyxoid sarcoma involving the chest wall. The chest radiography and computed tomography scan showed a heterogeneous mass in the lower right hemithorax, with necrosis and calcification foci. Histological examination was suggestive of a leiomyoma but the immunohistochemical study proved to be a low-grade fibromyxoid sarcoma. The clinical outcome of this patient was good, although the computed tomography scan showed signs of malignancy.

Keywords: Computed tomography, Chest wall, Fibromyxoid sarcoma

The melanotic neuroectodermal tumor of infancy, also known as progonoma, is a rare benign disease of neural crest origin that occurs within the first year of life and affects mainly the maxilla. The authors report a case of a 10-month-old child presenting with this uncommon tumor in the maxilla, emphasizing the diagnostic findings on computed tomography, and present a literature review.

Keywords: Neoplasia, Neural crest, Maxilla, Infancy, Computed tomography

PURPOSE: To evaluate the influence on local control in adult patients with soft tissue sarcomas of the limbs that underwent limb-sparing surgery and high-dose rate brachytherapy (HDRB) in association with teletherapy. MATERIAL AND METHODS: Sixteen patients treated from 1993 to 1999 were reviewed. Teletherapy was used pre- or postoperatively (30--55 Gy) in association with HDRB in a dose range of 18--36 Gy (fx 3--6 Gy BID). The linear quadratic model was used to calculate the biological effective dose (BED) and the values obtained were compared with data from the literature, of studies that employed teletherapy and low-dose rate brachytherapy (LDRB). RESULTS: Soft tissue sarcomas BED mean and median values were 78.5 Gy7 and 80 Gy7, respectively. Univariated analysis showed that BED for patients with soft tissue sarcoma of the limbs submitted to HDRB was similar to the value of 83 Gy7 of patients submitted to LDRB (p = 0.008). Actuarial local control, disease free and overall survival rates at 5-years were 83.2%, 75% and 93.7%, respectively. CONCLUSIONS: HDRB appears to be an effective complementary method to teletherapy in the treatment of soft tissue sarcomas. The local control rates are comparable to those reported in the literature. However, studies with longer follow-up and a larger number of patients are still necessary in order to determine the true potential of HDRB as a substitute for LDBR.

Keywords: Soft tissue sarcoma, Brachytherapy, Limb-sparing surgery, Biological effective dose

Ewing's sarcoma and Triton's tumor are two uncommon bone tumors of the skull that have nonspecific clinical and imaging features. However, imaging methods are important in the detection of the lesions during the diagnostic investigation in order to evaluate the extent of the bone lesions, involvement of soft tissues and brain, and to determine the presence of local recurrence and metastases. The confirmatory diagnosis relies on histological studies and immunohistochemistry. The authors report two cases of patients with these tumors and present the radiological findings.

Keywords: Ewing's sarcoma, Triton's tumor, Bone tumors, Skull, Computed tomography

Undifferentiated embryonal sarcoma of the liver is a rare disease that affects predominantly children over five years of age. The most common macroscopic finding is a large hepatic mass with a greater solid component and smaller cystic areas. However, computed tomography tends to overestimate the cystic components of the lesion and consequently ultrasonography is considered a more reliable technique for evaluating the texture of the tumor. Imaging methods can help to rule out nonneoplastic diseases such as liver abscesses and hematomas, and to establish the extent of the lesions. The diagnosis of undifferentiated embryonal sarcoma can be correctly inferred when imaging findings, the age of the patient and serum alpha protein levels are concurrently analyzed. The authors report a case of undifferentiated embryonal sarcoma in a 10-year-old girl and discuss the imaging findings features and differential diagnosis.

Keywords: Sarcoma, Tumor, Liver

OBJECTIVE: To evaluate the efficacy of percutaneous computed tomography (CT)-guided core needle biopsy of softtissue tumors in obtaining appropriate samples for histological analysis, and compare its diagnosis with the results of the surgical pathology as available. MATERIALS AND METHODS: The authors reviewed medical records, imaging and histological reports of 262 patients with soft-tissue tumors submitted to CT-guided core needle biopsy in an oncologic reference center between 2003 and 2009. RESULTS: Appropriate samples were obtained in 215 (82.1%) out of the 262 patients. The most prevalent tumors were sarcomas (38.6%), metastatic carcinomas (28.8%), benign mesenchymal tumors (20.5%) and lymphomas (9.3%). Histological grading was feasible in 92.8% of sarcoma patients, with the majority of them (77.9%) being classified as high grade tumors. Out of the total sample, 116 patients (44.3%) underwent surgical excision and diagnosis confirmation. Core biopsy demonstrated 94.6% accuracy in the identification of sarcomas, with 96.4% sensitivity and 89.5% specificity. A significant intermethod agreement about histological grading was observed between core biopsy and surgical resection (p < 0.001; kappa = 0.75). CONCLUSION: CT-guided core needle biopsy demonstrated a high diagnostic accuracy in the evaluation of soft tissue tumors as well as in the histological grading of sarcomas, allowing an appropriate therapeutic planning.

Keywords: Sarcoma; Needle biopsy; Computed tomography.

The effective evaluation for the treatment of patients with Ewing tumors depends on the accuracy in the determination of the primary tumor extent and the presence of metastatic disease. Currently, no universally accepted staging system is available to assess Ewing tumors. The present study aimed at discussing the use of PET/CT as a tool for staging, restaging and assessment of therapeutic response in patients with Ewing tumors. In spite of some limitations of PET/CT as compared with anatomical imaging methods, its relevance in the assessment of these patients is related to the capacity of the method to provide further physiological information, which often generates important clinical implications. Currently, the assessment of patients with Ewing tumor should comprise a study with PET/CT combined with other anatomical imaging modalities, such as radiography, computed tomography and magnetic resonance imaging.

Keywords: Ewing sarcoma; Imaging diagnosis; Diagnosis; Evaluation.

OBJECTIVE: To evaluate the performance of magnetic resonance imaging (MRI) in detecting periosteal reactions and to compare MRI and conventional radiography (CR) in terms of the classification of periosteal reactions. MATERIALS AND METHODS: Retrospective study of 42 consecutive patients (mean age, 22 years; 20 men) with a confirmed diagnosis of osteosarcoma or Ewing's sarcoma, MRI and CR images having been acquired pretreatment. Three blinded radiologists detected periosteal reactions and evaluated each periosteal reaction subtype in CR and MRI images: Codman's triangle; laminated; and spiculated. The CR was used as a benchmark to calculate the diagnostic performance. We used the kappa coefficient to assess interobserver reproducibility. A two-tailed Fisher's exact test was used in order to assess contingency between CR and MRI classifications. RESULTS: In the detection of periosteal reactions, MRI showed high specificity, a high negative predictive value, and low-to-moderate sensitivity. For CR and for MRI, the interobserver agreement for periosteal reaction was almost perfect, whereas, for the classification of different subtypes of periosteal reaction, it was higher for the Codman's triangle subtype and lower for the spiculated subtype. There was no significant difference between MRI and CR in terms of the classifications (p < 0.05). CONCLUSION: We found no difference between MRI and CR in terms of their ability to classify periosteal reactions. MRI showed high specificity and almost perfect interobserver agreement for the detection of periosteal reactions. The interobserver agreement was variable for the different subtypes of periosteal reaction.

Keywords: Periosteum; Osteosarcoma; Sarcoma, Ewing; Magnetic resonance imaging; Radiography; Reproducibility of results.

Sarcomas of the breast belong to a heterogeneous group of breast tumors of mesenchymal origin, without epithelial components. These tumors can be primary or secondary (after previous treatment for breast cancer), are rare, present aggressive behavior, and have a poor prognosis. They occur mainly in women between 45 and 50 years of age, with the exception of angiosarcomas, which can occur in younger patients. Clinically, breast sarcomas manifest as palpable, mobile, rapidly growing masses, without skin thickening, axillary lymphadenopathy, or nipple discharge. Although the imaging findings are non specific, they can be suggestive of sarcoma. For instance, a solitary mass showing rapid growth, with circumscribed or indistinct margins and, a complex (solid-cystic) or heterogeneous echotexture, without axillary lymph node involvement, can raise the suspicion of sarcoma. The treatment is not well established, because of the rarity and heterogeneity of this type of neoplasm. The principles of treatment for sarcoma of the breast have been addressed only in small cohort studies. In most cases, the treatment of choice is surgery without axillary lymphadenectomy.

Keywords: Breast cancer; Sarcoma; Mammography; Ultrasonography; Magnetic resonance imaging.

Os nervos cranianos representam extensões das estruturas funcionais do cérebro que cruzam a cabeça e o pescoço. Eles estão ligados a diferentes estruturas cranianas e se relacionam com diversas doenças. Uma compreensão profunda de sua complexa anatomia e aparência normal de imagem permite ao examinador identificar e caracterizar com mais precisão as anormalidades. A ressonância magnética com contraste é uma importante ferramenta para avaliar essas estruturas, especialmente as sequências tridimensionais de precessão livre de estado estacionário, que proporcionam alta resolução espacial e de partes moles, apesar da fina espessura dos nervos cranianos. Na maioria dos casos, os achados de imagem são inespecíficos, e uma anamnese completa, exame físico dirigido e testes laboratoriais são necessários para estreitar o diagnóstico diferencial. Este ensaio iconográfico ilustra e discute doenças congênitas, traumáticas e vasculares dos nervos cranianos, mostrando imagens em uma abordagem fisiopatológica.

Keywords: Neuroimagem/métodos; Radiologia/métodos; Ressonância magnética/métodos; Nervos cranianos/diagnóstico por imagem.

OBJECTIVE: To evaluate the degree of similarity between manual and semiautomatic segmentation of soft-tissue sarcomas on magnetic resonance imaging (MRI). MATERIALS AND METHODS: This was a retrospective study of 15 MRI examinations of patients with histopathologically confirmed soft-tissue sarcomas acquired before therapeutic intervention. Manual and semiautomatic segmentations were performed by three radiologists, working independently, using the software 3D Slicer. The Dice similarity coefficient (DSC) and the Hausdorf distance were calculated in order to evaluate the similarity between manual and semiautomatic segmentation. To compare the two modalities in terms of the tumor volumes obtained, we also calculated descriptive statistics and intraclass correlation coef ficients (ICCs). RESULTS: In the comparison between manual and semiautomatic segmentation, the DSC values ranged from 0.871 to 0.973. The comparison of the volumes segmented by the two modalities resulted in ICCs between 0.9927 and 0.9990. The DSC values ranged from 0.849 to 0.979 for intraobserver variability and from 0.741 to 0.972 for interobserver variability. There was no significant difference between the semiautomatic and manual modalities in terms of the segmentation times (p > 0.05). CONCLUSION: There appears to be a high degree of similarity between manual and semiautomatic segmentation, with no significant difference between the two modalities in terms of the time required for segmentation.

Keywords: Image processing, computer-assisted; Sarcoma/diagnostic imaging; Soft tissue neoplasms/diagnostic imaging; Magnetic resonance imaging; Reproducibility of results.