Mucoepidermoid carcinoma on the lip is a very rare disease, consisting of epidermoid and mucus-producing cells histologically. A 47 year old female had had an asymptomatic, palpable, bean-sized nodule on the center of her upper lip for 2 years. Histological findings showed multiple oval nests consisting of epidermoid & clear cells and cystic spaces filled with faintly eosinophilic amorphous material in a fibrotic background throughout the dermis, subcutis, and muscular layer After a diagnosis of mucoepidermoid carcinoma, Mohs micrographic surgery was performed for complete removal, and A - T flap with M plasty was designed for appropriate repair.
Carcinoma, Mucoepidermoid* ; Dermis ; Diagnosis ; Eosinophils ; Female ; Humans ; Lip* ; Middle Aged ; Mohs Surgery* ; Rare Diseases

Subependymomas are rare benign tumors located in the ventricular system. Intraparenchymal subependymoma is extremely rare; only 6 cases have been reported, and all were located in the supratentorial region. We describe a case of infratentorial, intraparenchymal subependymoma in a 28-year-old man with intermittent headache. Imaging revealed a well-demarcated cystic and solid cerebellar mass near the fourth ventricle. The mass had a microcystic component and calcification without contrast enhancement. Complete surgical excision was performed, and histopathology confirmed a subependymoma.
Adult ; Calcinosis/diagnosis ; Cerebellar Neoplasms/*diagnosis/surgery ; Fourth Ventricle ; Glioma, Subependymal/*diagnosis/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Rare Diseases/*diagnosis/surgery ; Tomography, X-Ray Computed

The association of intramuscular myxoma and fibrous dysplasia is a rare disease known as Mazabraud's syndrome. We present a case of Mazabraud's syndrome coexisting with a uterine tumor and resembling an ovarian sex cord tumor (UTROSCT). This uterine tumor showed a high mitotic index and cytological atypia. To the best of our knowledge, the coexistence of the two different entities has not been reported in the literature.
Aged ; Biopsy ; Buttocks/pathology/surgery/ultrasonography ; Diagnosis, Differential ; Female ; Fibrous Dysplasia, Monostotic/complications/*diagnosis/surgery ; Humans ; Magnetic Resonance Imaging ; Myxoma/complications/*diagnosis/surgery ; Ovarian Neoplasms/*diagnosis ; Rare Diseases ; Sex Cord-Gonadal Stromal Tumors/*diagnosis ; Syndrome ; Uterine Neoplasms/complications/*diagnosis/surgery

Mesenteric lymphangiomas are rare abdominal masses that are seldom associated with small bowel volvulus, and especially in adult patients. We report here on an unusual case of small bowel volvulus that was induced by a mesenteric lymphangioma in a 43-year-old man who suffered from repeated bouts of abdominal pain. At multidetector CT, we noticed whirling of the cystic mesenteric mass and the adjacent small bowel around the superior mesenteric artery. Small bowel volvulus induced by the rotation of the mesenteric lymphangioma was found on exploratory laparotomy. Lymphangioma should be considered as a rare cause of small bowel volvulus in adult patients.
Abdominal Pain/etiology ; Adult ; Contrast Media/diagnostic use ; Diagnosis, Differential ; Humans ; Intestinal Volvulus/*diagnosis/etiology/surgery ; Intestine, Small/*radiography/surgery ; Lymphangioma/*complications/surgery ; Male ; Mesenteric Artery, Superior/*radiography/surgery ; Radiographic Image Enhancement/methods ; Rare Diseases ; Tomography, X-Ray Computed

We describe the enhancement patterns of myoepithelioma in two patients with a soft palate mass. In the first case, helical CT revealed a faintly enhancing mass. Histologically, the tumor was composed of plasmacytoid cells in a background of rich myxoid stroma. Immunostaining for CD34 showed scanty blood vessels. In the second case, helical CT revealed an intensely enhancing mass. Histologically, the mass was a cellular tumor with fibrous stroma. Immunostaining for CD34 also showed frequent blood vessels.
Aged ; Contrast Media/administration & dosage ; Diagnosis, Differential ; Female ; Humans ; Iohexol/analogs & derivatives/diagnostic use ; Male ; Middle Aged ; Myoepithelioma/*diagnosis/surgery ; Palatal Neoplasms/*diagnosis/surgery ; Palate, Soft/*radiography/surgery ; Radiographic Image Enhancement/methods ; Rare Diseases ; Tomography, Spiral Computed/*methods

Pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma is a rare disease and usually presents as pulmonary masses, mass-like consolidation, or pulmonary nodules on chest images. We report a case of a 43-year-old man with symptoms of chronic cough for 1 year, showing bilateral diffuse bronchovascular bundle thickening and focal ground glass opacities on a chest computed tomography scan. Video-assisted thoracoscopic surgery was performed and the final pathologic diagnosis was pulmonary MALT lymphoma. Concurrent involvement of the pancreas was discovered during staging workup. After diagnosis, he was treated with cytotoxic chemotherapy and rituximab and showed improvements in his lung lesion and pancreas.
Adult ; Cough ; Diagnosis ; Drug Therapy ; Glass* ; Humans ; Lung ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Pancreas* ; Rare Diseases ; Rituximab ; Thoracic Surgery, Video-Assisted ; Thorax

Adenoid cystic carcinoma (ACC) is a malignant neoplasm of glands commonly occurs in salivary glands. Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare form of ACC that primarily presents on the skin. Herein, we represent a rare case of PCACC occurred in the umbilicus in a 66-year-old Korean male patient. The patient visited our center with erythematous indurated patch on the umbilicus diagnosed as ACC by incisional biopsy at another center. The diagnosis of PCACC was confirmed by additional histopathologic examination and imaging study. We proceeded Mohs micrographic surgery and reconstructed umbilicus with tacked purse string suture. Local recurrence and distant metastasis were not observed during 30-month follow-up. We report this rare case of PCACC on the umbilicus so that dermatologist can aware of the rare disease. Furthermore, we recommend MMS and tacked purse string suture as effective methods for treatment of PCACC and immediate umbilical reconstruction.
Adenoids ; Aged ; Biopsy ; Carcinoma, Adenoid Cystic ; Diagnosis ; Follow-Up Studies ; Humans ; Male ; Mohs Surgery ; Neoplasm Metastasis ; Rare Diseases ; Recurrence ; Salivary Glands ; Skin ; Sutures ; Umbilicus

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

Aspergillosis is a rare cause of spondylitis. Moreover, early diagnosis by MR imaging and adequate treatment can prevent the serious complications of fungal infection. To our knowledge, the MR findings of multilevel aspergillus spondylitis in the cervico-thoraco-lumbar spine have not been previously described. Here, we report the MR findings of aspergillus spondylitis involving the cervical, thoracic, and lumbar spine in a liver transplant recipient.
Aspergillosis/*diagnosis ; Aspergillus/isolation & purification ; Bone Transplantation ; Cervical Vertebrae/microbiology/pathology/surgery ; Humans ; *Immunocompromised Host ; Liver Transplantation ; Lumbar Vertebrae/microbiology/pathology/surgery ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Postoperative Complications/diagnosis/microbiology/surgery ; Rare Diseases ; Spondylitis/*microbiology/surgery ; Thoracic Vertebrae/microbiology/pathology/surgery

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