Alveolar soft part sarcoma (ASPS) is a rare epithelial-like soft tissue sarcoma. The two main sites of its occurrence are the lower extremities in adults and the head and neck in children. Primary pulmonary involvement of this sarcoma, without evidence of soft tissue tumor elsewhere, is very exceptional. We present a case of primary ASPS of the lung in a 42-yr-old woman. A computed tomographic scan of the thorax demonstrated a well-circumscribed, solid tumor located in the right upper lobe. The mass was resected by right upper lobectomy. After 5 months, three metastatic lesions, involving lumbar vertebrae and occipital scalp, were found. Histologically, the tumor consisted of alveolar nests of large polygonal tumor cells, the cytoplasm of which frequently revealed periodic acid-Schiff-positive, diastase-resistant intracytoplasmic rod-like structures. On immunohistochemical staining, the tumor cells were positive only for vimentin and alpha-smooth muscle actin. Ultrastuctural study using electron microscopy revealed characteristic electron-dense, rhomboid intracytoplasmic crystals.
Soft Tissue Neoplasms/pathology/radiography ; Sarcoma/*pathology/*radiography ; Rare Diseases/pathology/radiography ; Pulmonary Alveoli/*pathology/*radiography ; Lung Neoplasms/*pathology/*radiography ; Humans ; Female ; Adult

Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder characterized by lymphatic channel proliferation. It is mostly reported in children and young adults. Here, we report a case involving a 52-year-old asymptomatic woman who presented with increased interstitial markings, as seen on a chest radiograph. Diffuse interstitial septal thickening was found on a serial follow-up chest computed tomography scan, and lymphangitic metastasis was the primary radiologic differential diagnosis. However, histologic sections of wedge resected lung revealed diffuse pleural and interlobular septal lymphatic proliferation characteristic of DPL.
Diagnosis, Differential ; Female ; Humans ; Lung Neoplasms/pathology/*radiography ; Lymphangioma/pathology/*radiography ; Middle Aged ; Rare Diseases/pathology/*radiography ; Tomography, X-Ray Computed

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous lymphoma. There have been a few case reports describing the radiologic imaging findings of SPTCL. We report a case of SPTCL, rarely presented with a breast mass. Here, we review her clinical history and radiologic (mammography and ultrasound) findings.
Adult ; Breast Neoplasms/*pathology/radiography/ultrasonography ; Female ; Humans ; Lymphoma, T-Cell/*pathology/radiography/ultrasonography ; Mammography ; Panniculitis/*pathology/radiography/ultrasonography ; Rare Diseases/*pathology/radiography/ultrasonography ; Skin Neoplasms/*pathology/radiography/ultrastructure

We report here on a case of primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas. A 41-year-old man was evaluated by CT to find the cause of his abdominal pain. The CT scans showed a heterogeneously enhancing necrotic mass with numerous areas of coarse calcification, and this was located in the left side of the retroperitoneal space and involved the body and tail of the pancreas. Portal venography via the celiac axis also showed invasion of the splenic vein. Following excision of the mass, it was pathologically confirmed to be primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas.
Abdominal Pain/etiology ; Adult ; Chondrosarcoma, Mesenchymal/complications/*diagnosis/surgery ; Contrast Media/administration & dosage ; Diagnosis, Differential ; Humans ; Iohexol/analogs & derivatives/diagnostic use ; Male ; Necrosis ; Pancreas/pathology/radiography ; Pancreatic Neoplasms/complications/*diagnosis/surgery ; Portal Vein/radiography ; Radiographic Image Enhancement/methods ; Rare Diseases ; Retroperitoneal Space/radiography ; Splenic Vein/radiography ; Tomography, X-Ray Computed/methods

Churg-Strauss syndrome (CSS) is a rare disease characterized by asthma, peripheral eosinophilia and vasculitis. A quarter of CSS patients with clinical remission have experienced a relapse. We report here a case of lung abscess in a patient with relapsed CSS. A 46-year-old man who achieved clinical remission of CSS was confirmed for relapse by the presence of peripheral eosinophillia, pulmonary lesions and perivascular infiltrate of eosinophils in colon pathology. After administration of systemic glucocorticoid and one cycle of cyclophosphamide pulse therapy, he complained of dyspnea, sputum and chest pain. There were necrotic masses containing internal air-fluid levels in the right upper and the left lower lobes of the lung on chest radiography. Percutaneous needle aspiration culture specimens revealed the presence of K. pneumoniae. The patient was successfully treated with a 5-week course of antibiotics.
Anti-Bacterial Agents ; Asthma ; Chest Pain ; Churg-Strauss Syndrome* ; Colon ; Cyclophosphamide ; Dyspnea ; Eosinophilia ; Eosinophils ; Humans ; Lung Abscess* ; Lung* ; Middle Aged ; Needles ; Pathology ; Pneumonia ; Radiography ; Rare Diseases ; Recurrence ; Sputum ; Thorax ; Vasculitis

Chondrosarcomas are malignant tumors of cartilage that rarely involve the sinonasal region, and myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that usually occurs in the soft tissue of extremities. Although several case reports and results of small series of chondrosarcomas in the sinonasal region in children are available, myxoid type chondrosarcoma is extremely rare. We recently experienced a case of low grade myxoid chondrosarcoma involving the sinonasal cavity in a 10-year-old boy, and here we report its radiologic-pathologic findings. In this case, chondroid calcification on CT and septal and marginal enhancement on MRI suggested a chondrosarcoma. Whole body PET-CT demonstrated no definite metastatic lesion and a low peak standardized uptake value primary tumor. However, no definite distinguishing imaging features were observed that distinguished low grade myxoid chondrosarcoma from conventional chondrosarcoma.
Bone Neoplasms/*diagnosis/radiotherapy/surgery ; Child ; Chondrosarcoma/*diagnosis/radiotherapy/surgery ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Myxosarcoma/*diagnosis/radiotherapy/surgery ; Nasal Cavity/*pathology/*radiography/surgery ; Paranasal Sinuses/*pathology/*radiography/surgery ; Positron-Emission Tomography ; Rare Diseases ; Tomography, X-Ray Computed ; Whole Body Imaging

Scurvy is very rare disease in industrialized societies. Nevertheless, it still exists in higher risk groups including economically disadvantaged populations with poor nutrition, such as the elderly and chronic alcoholics. The incidence of scurvy in the pediatric population is very low. This study reports a case of scurvy in a 5-year-old girl with cerebral palsy and developmental delay based on MRI findings.
Ascorbic Acid/blood/therapeutic use ; Bone Diseases, Metabolic/etiology ; Cerebral Palsy/complications ; Child, Preschool ; Cholecalciferol/blood ; Developmental Disabilities/complications ; Drainage ; Female ; Femur/pathology/radionuclide imaging/surgery ; Fever/etiology ; Follow-Up Studies ; Hematoma/diagnosis/etiology/surgery ; Humans ; Knee/radiography ; Magnetic Resonance Imaging/*methods ; Muscle Weakness/etiology ; Rare Diseases ; Scurvy/complications/*diagnosis/drug therapy ; Thigh/pathology ; Vitamins/therapeutic use

Glomerulonephritis and pulmonary hemorrhage are features of Goodpasture's syndrome. Goodpasture's syndrome accompanied with central nervous system (CNS) vasculitis is extremely rare. Herein, we report a rare case of CNS vasculitis associated with Goodpasture's syndrome in a 34-year-old man, who presented with a seizure and sudden onset of right sided weakness. He also had recurrent hemoptysis of one month's duration. Goodpasture's syndrome is histologically diagnosed by intense linear deposits of IgG along the glomerular basement membrane in both renal and lung tissues.
Adult ; Anti-Glomerular Basement Membrane Disease/complications/*diagnosis/therapy ; Anti-Inflammatory Agents/administration & dosage ; Brain/*pathology ; Contrast Media/administration & dosage ; Diagnosis, Differential ; Fluorescent Antibody Technique ; Hemoptysis/etiology ; Humans ; Image Enhancement/methods ; Immunoglobulin G/immunology ; Kidney/ultrasonography ; Lung/pathology/*radiography ; Magnetic Resonance Imaging ; Male ; Methylprednisolone/administration & dosage ; Muscle Weakness/etiology ; Plasmapheresis ; Rare Diseases ; Seizures/etiology ; Tomography, X-Ray Computed ; Vasculitis, Central Nervous System/*diagnosis/etiology/therapy