OBJECTIVE: To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. MATERIALS AND METHODS: We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for > or = 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. RESULTS: Among the 70 patients (mean age, 35.8 +/- 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). CONCLUSION: Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites.
Adolescent ; Adult ; Aged ; Asian Continental Ancestry Group ; Bone Neoplasms/*pathology/radiography ; Brain Neoplasms/pathology/radiography ; Female ; Humans ; Lymph Nodes/pathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive/pathology/radiography ; Positron-Emission Tomography ; Sarcoma, Ewing/*pathology/radiography ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVE: To determine whether histogram values of the normalized apparent diffusion coefficient (nADC) and normalized cerebral blood volume (nCBV) maps obtained in contrast-enhancing lesions detected on immediate post-operative MR imaging can be used to predict the patient response to concurrent chemoradiotherapy (CCRT) with temozolomide (TMZ). MATERIALS AND METHODS: Twenty-four patients with GBM who had shown measurable contrast enhancement on immediate post-operative MR imaging and had subsequently undergone CCRT with TMZ were retrospectively analyzed. The corresponding histogram parameters of nCBV and nADC maps for measurable contrast-enhancing lesions were calculated. Patient groups with progression (n = 11) and non-progression (n = 13) at one year after the operation were identified, and the histogram parameters were compared between the two groups. Receiver operating characteristic (ROC) analysis was used to determine the best cutoff value for predicting progression. Progression-free survival (PFS) was determined with the Kaplan-Meier method and the log-rank test. RESULTS: The 99th percentile of the cumulative nCBV histogram (nCBV C99) on immediate post-operative MR imaging was a significant predictor of one-year progression (p = 0.033). ROC analysis showed that the best cutoff value for predicting progression after CCRT was 5.537 (sensitivity and specificity were 72.7% and 76.9%, respectively). The patients with an nCBV C99 of < 5.537 had a significantly longer PFS than those with an nCBV C99 of ≥ 5.537 (p = 0.026). CONCLUSION: The nCBV C99 from the cumulative histogram analysis of the nCBV from immediate post-operative MR imaging may be feasible for predicting glioblastoma response to CCRT with TMZ.
Adult ; Aged ; Antineoplastic Agents, Alkylating/*therapeutic use ; Brain/pathology/radiography ; Brain Neoplasms/*drug therapy/mortality/radiography ; Chemoradiotherapy ; Dacarbazine/*analogs & derivatives/therapeutic use ; Diffusion Magnetic Resonance Imaging ; Disease Progression ; Disease-Free Survival ; Female ; Glioblastoma/*drug therapy/mortality/radiography ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Proportional Hazards Models ; ROC Curve ; Retrospective Studies

A 45-yr-old female patient was admitted with one-month history of headache and progressive left hemiparesis. Brain magnetic resonance imaging (MRI) demonstrated a mass lesion in her right frontal lobe. Her brain tumor was confirmed as a small cell glioblastoma. Her follow-up brain MRI, taken at 8 months after her initial surgery demonstrated tumor recurrence in the right frontal lobe. Contrast-enhanced 7.0T brain magnetic resonance imaging (MRI) was safely performed before surgery and at the time of recurrence. Compared with 1.5T and 3.0T brain MRI, 7.0T MRI showed sharpened images of the brain tumor contexture with detailed anatomical information. The fused images of 7.0T and 1.5T brain MRI taken at the time of recurrence demonstrated no significant discrepancy in the positions of the anterior and the posterior commissures. It is suggested that 7.0T MRI can be safely utilized for better images of the maligant gliomas before and after surgery.
Brain Neoplasms/pathology/*radiography/surgery ; Female ; Frontal Lobe/radiography ; Glioblastoma/pathology/*radiography/surgery ; Humans ; In Situ Hybridization, Fluorescence ; *Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Recurrence, Local ; Receptor, Epidermal Growth Factor/genetics

We report an extremely rare case of metastatic common bile duct cancer from pulmonary adenocarcinoma presenting as obstructive jaundice. The patient was a 76-year-old male, who presented with generalized weakness and right upper quadrant pain. Plain chest X-ray noted multiple small nodules in both lung fields. Abdominal computed tomography scan showed a stricture of the mid common bile duct along with ductal wall enhancement. Endoscopic retrograde cholangiography revealed a concentric, abrupt narrowing of the mid-common bile duct suggestive of primary bile duct cancer. However, pathology comfirmed metastatic common bile duct cancer arising from pulmonary adenocarcinoma with immunohistochemical study with thyroid transcriptional factor-1 (TTF-1).
Adenocarcinoma/*diagnosis/pathology/radiography ; Aged ; Brain Neoplasms/radiography/secondary ; Bronchoscopy ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct Neoplasms/*diagnosis/secondary ; DNA-Binding Proteins/metabolism ; Humans ; Immunohistochemistry ; Jaundice, Obstructive/*etiology ; Lung Neoplasms/*diagnosis/pathology/radiography ; Male ; Positron-Emission Tomography ; Tomography, X-Ray Computed

In the study we assessed the distortion of 7.0 T magnetic resonance (MR) images in reference to 1.5 T MR images in the radiosurgery of metastatic brain tumors. Radiosurgery with Gamma Knife Perfexion(R) was performed for the treatment of a 54-yr-old female patient with multiple brain metastases by the co-registered images of the 7.0 T and 1.5 T magnetic resonance images (MRI). There was no significant discrepancy in the positions of anterior and posterior commissures as well as the locations of four metastatic brain tumors in the co-registered images between 7.0 T and 1.5 T MRI with better visualization of the anatomical details in 7.0 T MR images. This study demonstrates for the first time that 7.0 T MR images can be safely utilized in Perfexion(R) Gamma Knife radiosurgery for the treatment of metastatic brain tumors. Furthermore 7.0 T MR images provide better visualization of brain tumors without image distortion in comparison to 1.5 T MR images.
Adenocarcinoma/pathology/radiography ; Brain Neoplasms/pathology/secondary/*surgery ; Female ; Humans ; Lung Neoplasms/pathology/radiography ; *Magnetic Resonance Imaging ; Middle Aged ; *Radiosurgery ; Tomography, X-Ray Computed

We report a case of a 48-year-old man who developed metastatic angiosarcoma in her left orbit. A 48-year-old man was first sent to us for a check up of proptosis of the left eye. A left orbital tumor was recognized on orbital computed tomography scans. The open biopsy showed angiosarcoma. Chest X-ray films and thoracic computed tomography showed an abnormal mass in the left inferior lung field. Angiosarcoma was confirmed by transbronchial lung biopsy. In summary, we believed that the orbital tumour was an initial symptom of the metastasis ensuing from the lung angiosarcoma.
Antineoplastic Agents/administration & dosage ; Antineoplastic Agents, Phytogenic/administration & dosage ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Brain Neoplasms/pathology/secondary ; Cisplatin/administration & dosage ; Etoposide/administration & dosage ; Fatal Outcome ; Hemangiosarcoma/pathology/radiography/radiotherapy/*secondary/therapy ; Humans ; Lung Neoplasms/drug therapy/*pathology/radiography ; Male ; Middle Aged ; Orbital Neoplasms/pathology/*radiography/*secondary ; Tomography, X-Ray Computed

Syphilis, along with the recent increase of human immunodeficiency virus (HIV) patients, has also been on the rise. It has a broad spectrum of clinical manifestations, among which cerebral gumma is, a kind of neurosyphilis, however, it is rare and can be cured by penicillin. Thus, cerebral gumma needs to be differentially diagnosed from other brain masses that may be present in syphilis patients. We have experienced a case where the patient was first suspected of brain tumor, but confirmed by surgery to be cerebral gumma due to neurosyphilis. This is the first such case encountered in Korea, therefore, we report it here in. A 40-year old woman complaining of headaches was found to have a brain mass on her CT scans and MRI. Suspecting a brain Tumor, a resection was performed on the patient, and histological results revealed that the central portion of the mass contained necrotic material and the peripheral region was infiltrated with plasma cells. Warthin-Starry staining of the region revealed spirochetes, and the patient was thus diagnosed as brain gumma. Venereal Disease Research Laboratory (VDRL) of cerebrospinal fluid (CSF) was reactive. After an operation, penicillin-G at a daily dose of 24x10(6) U was given for 10 days from post-operative day 10, and thereafter, the mass disappeared.
Adult ; Brain Neoplasms/*diagnosis/pathology/radiography ; Female ; HIV Infections/*diagnosis/radiography ; Humans ; Magnetic Resonance Imaging ; Neurosyphilis/*diagnosis/pathology/radiography ; Tomography, X-Ray Computed

Extramedullary plasmacytoma is a rare malignant plasma cell tumour. We report an extremely aggressive case of extramedullary plasmacytoma of the right maxillary sinus, which had metastasised to the brain and rib. A 56-year-old man presented with recurrent epistaxis and acute anaemia. Nasendoscopy revealed a medialised medial wall of the right maxilla and a mass occupying the whole nasopharynx. Magnetic resonance imaging revealed a right maxillary tumour with extension to the ipsilateral nasal cavity, nasopharynx, right sphenoid and ethmoidal sinuses. There was an extra-axial brain metastasis. There were metastases to the right parietal region and left eighth rib. Histopathology examination of the maxillary mass revealed abundant plasma cells with kappa-chain restriction. He was planned for four cycles of chemotherapy. Unfortunately, in view of the advanced stage of disease, he succumbed to his disease during the first cycle of chemotherapy.
Anemia ; complications ; Antineoplastic Agents ; therapeutic use ; Brain Neoplasms ; diagnosis ; pathology ; Fatal Outcome ; Humans ; Magnetic Resonance Imaging ; methods ; Male ; Maxillary Sinus ; diagnostic imaging ; pathology ; Maxillary Sinus Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Middle Aged ; Nasopharynx ; pathology ; Neoplasm Metastasis ; Plasmacytoma ; diagnosis ; diagnostic imaging ; pathology ; Radiography

A myeloid sarcoma (MS) is an extramedullary tumor consisting of primitive granulocytic precursor cells. Although most such tumors have been reported in patients with acute myelogenous leukemia, MS is rarely recognized as an isolated tumor without any evidence of leukemia. However, in such cases, the initial diagnosis of MS can be difficult, so initial misdiagnosis rates of up to 75% have been reported. This report describes an unusual case of MS in a 3-year 5-month-old girl presenting as bilateral renal enlargements, and brain masses, with multiple bone involvements, but no hematological abnormalities.
Bone Neoplasms/pathology/*radiography ; Brain Neoplasms/pathology/*radiography ; Child, Preschool ; Female ; Human ; Kidney Neoplasms/pathology/*radiography ; Sarcoma, Granulocytic/pathology/*radiography ; Tomography, X-Ray Computed

Trilateral retinoblastoma is a rare, but well recognized syndrome. These tumors usually occur in the pineal, parasellar, or suprasellar regions several years after successful management of ocular retinoblastomas without evidence of direct extension or distant metastasis. Here we report a case of trilateral retinoblastoma presenting initially with a sellar tumor and with concurrent unilateral retinoblastoma. The patient was a 5-month-old baby girl showing poor eye contact and nystagmus for several days. She had no family history of retinoblastoma. Brain MRI revealed a midline suprasellar tumor without evidence of cerebrospinal fluid seeding or extracranial metastasis. A pathologic diagnosis of retinoblastoma was made for her brain tumor, and a small, intraocular retinoblastoma was detected in the left eye by thorough examination of the fundus. If a retinoblastoma occurs in the midline of the brain, including the pineal and sellar regions, a careful screening to detect any additional retinal tumors should be performed. Moreover, since these tumors are often hereditary and harbor a worse prognosis, the diagnosis has implications for genetic counseling. This is the first report on a case of trilateral retinoblastoma in Korea presented with a sellar mass.
Brain/*pathology/radiography ; Brain Neoplasms/pathology/radiography/*secondary/surgery ; Female ; Humans ; Infant ; Retinal Neoplasms/*pathology/radiography/surgery ; Retinoblastoma/pathology/radiography/*secondary/surgery