Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult.
Adult ; Diagnosis, Differential ; Human ; Immunohistochemistry ; Lung Neoplasms/*diagnosis/radiography/surgery ; Male ; Pulmonary Blastoma/*diagnosis/radiography/surgery ; Teratoma/*diagnosis/*radiography/surgery ; Tomography, X-Ray Computed ; Vimentin/biosynthesis

Thoracic paragangliomas comprise only 1-2% of all paragangliomas, including the adrenal pheochromocytomas, and these tumors are mostly found in the mediastinal compartments (1). To the best of our knowledge, there is only one case report in the pathology literature of endobronchial involvement by a primary pulmonary paraganglioma (2). We report here on the CT and bronchoscopic findings of a case of pathologically proven endobronchial paraganglioma in a 37-year-old woman. In our case, bronchoscopy and CT demonstrated an endobronchial hypervascular mass, which indicated the presence of carcinoid or hypervascular metastasis based on the known incidence of such tumors.
Adult ; Bronchial Neoplasms/*radiography/surgery ; Bronchoscopy ; Contrast Media ; Diagnosis, Differential ; Female ; Humans ; Lung Neoplasms/*radiography/surgery ; Paraganglioma/*radiography/surgery ; Tomography, X-Ray Computed

Clear cell tumor of the lung is a rare and very unusual benign pulmonary tumor. As clear cell tumor of the lung contains abundant cytoplasmic glycogen, this tumor is called "sugar tumor". We report a case of sugar tumor in a 64-yr-old man presenting as a round pulmonary nodule. On dynamic computed tomography (CT) scans, the solitary pulmonary nodule showed early wash-in enhancement with an early washout pattern like a lung malignancy. The patient underwent wedge resection for the tumor. Pathologic examination, including immunohistochemical studies, revealed that the nodule was a benign clear cell tumor, so-called "sugar tumor". Because only a small number of cases have been reported previously, clinical aspects, radiological characteristics on dynamic contrast-enhanced CT, and differential diagnosis of the tumor are not well established. Herein we present a clear cell tumor of the lung and discuss the clinical, radiological, and pathological features of the tumor.
Antigens, Neoplasm/metabolism ; Diagnosis, Differential ; Humans ; Lung/radiography/surgery ; Lung Neoplasms/diagnosis/pathology/*radiography ; Male ; Middle Aged ; Neoplasm Proteins/metabolism ; Perivascular Epithelioid Cell Neoplasms/diagnosis/pathology/*radiography ; Solitary Pulmonary Nodule/diagnosis/pathology/*radiography ; *Tomography, X-Ray Computed

Aggressive angiomyxoma is an uncommon mesenchymal myxoid tumor that is characterized by slow growth and frequent local recurrence. It is currently regarded as a nonmetastasizing tumor. We describe a case of recurrent aggressive angiomyxoma with invasion into the veins including the inferior vena cava and the right atrium and with pulmonary metastases. Our case, together with those unusual cases documented in previous reports, may lead to a reappraisal of the nature of aggressive angiomyxoma.
Adult ; Biopsy ; Contrast Media/diagnostic use ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Lung Neoplasms/*secondary/surgery ; Myxoma/*diagnosis/pathology/surgery ; Radiography, Interventional ; Tomography, X-Ray Computed ; Vascular Neoplasms/*diagnosis/pathology/surgery ; Vena Cava, Inferior/pathology/surgery

We report the dynamic contrast-enhanced CT and histopathologic findings of a rare case of recurrent pulmonary capillary hemangiomas. The findings consisted of peripheral nodular enhancement at the early arterial phase and a subsequent "central filling-in" enhancement pattern on the delayed scans, which was identical to the well-known enhancement pattern of hemangiomas of the liver. Although there was no evidence of histological malignancy, pulmonary capillary hemangiomas manifested as multiple nodular lesions and showed postoperative recurrence.
Contrast Media/diagnostic use ; Diagnosis, Differential ; Female ; Hemangioma, Capillary/pathology/*radiography/surgery ; Humans ; Iopamidol/diagnostic use ; Lung/*blood supply ; Lung Neoplasms/pathology/*radiography/surgery ; Recurrence ; Tomography, X-Ray Computed/*methods ; Young Adult

Basaloid squamous cell carcinoma is a rare and aggressive variant of squamous cell carcinoma, which mostly occurs in the upper aerodigestive tracts. Basaloid squamous cell carcinoma also typically arises in the anal canal, but is extremely rare in the lower gastrointestinal tract. A 70-year-old man presented with loose stool and intermittent hematochezia 2 months ago. Colonoscopy showed an ulceroinfiltrative mass on the rectosigmoid colon from 16 cm to 18 cm above the anal verge. Conventional colonoscope could not pass through the lesion but it was possible with pediatric colonoscope. Abdominal CT scan showed 1.6 cm sized wall thickening with circumferential luminal narrowing in the rectosigmoid colon and multiple ill-defined low density masses in both lobes of the liver. Therefore, colon cancer with liver metastasis was suspected. However, basaloid cells were noted on histologic examination, and they were weakly positive for synaptophysin on immunohistochemical study. After palliative lower anterior resection, histologic examination of the resected specimen revealed basaloid differentiation with keratin pearls, and tumor cells were positively stained with high molecular weighted cytokeratin (34BE12) and CK 5/6. Thus, the patient was finally diagnosed with basaloid squamous cell carcinoma of rectosigmoid colon with distant metastases.
Aged ; Carcinoma, Squamous Cell/*diagnosis/pathology/surgery ; Colonoscopy ; Colorectal Neoplasms/*diagnosis/pathology/surgery ; Humans ; Immunohistochemistry ; Keratins/metabolism ; Liver Neoplasms/radiography/secondary ; Lung Neoplasms/radionuclide imaging/secondary ; Male ; Positron-Emission Tomography ; Synaptophysin/metabolism ; Tomography, X-Ray Computed

We report an unusual case of 9.5-cm-sized embryonal rhabdomyosarcoma arose from a mediastinal mature teratoma in a 46-yr-old man. A man presented with chest trauma as a result of an accident at 10 September 2011. On chest X-ray, an anterior mediastinal mass was detected. To obtain further information, chest computed tomography (CT) with contrast enhancement was performed, revealing an anterior mediastinal mass. Complete surgical excision was performed and entire specimen was evaluated. Pathologic diagnosis was embryonal rhabdomyosarcoma arising in mature cystic teratoma. After surgical excision, two cycles of dactinomycin-based chemotherapy were performed. Lung metastasis was detected on follow up CT in September 2012, and wedge resection was performed. Pathological finding of the lung lesion showed same feature with that of primary rhabdomyosarcoma.
Antibiotics, Antineoplastic/therapeutic use ; Dactinomycin/therapeutic use ; Desmin/metabolism ; Humans ; Immunohistochemistry ; Lung Neoplasms/radiography/secondary/surgery ; Male ; Mediastinal Neoplasms/*diagnosis/pathology ; Middle Aged ; Neoplasms, Germ Cell and Embryonal/drug therapy/*radiography/surgery ; Rhabdomyosarcoma, Embryonal/drug therapy/*radiography/surgery ; Teratoma/*diagnosis/pathology ; Tomography, X-Ray Computed

A pulmonary mucinous cystadenocarcinoma is an extremely rare tumor that is considered to be a cystic variant of mucin-producing lung adenocarcinoma. We present a case of pulmonary mucinous cystadenocarcinoma in a 54-year-old woman. Chest CT scans showed a 4.3-cm-sized, lobulated, well-defined, and homogeneous mass in the right middle lobe with peripheral stippled calcifications that demonstrated low-attenuation with no enhancement after contrast administration; 18F-fluorodeoxyglucose (FDG) PET/CT demonstrated mild heterogeneous FDG uptake. The mass was diagnosed as adenocarcinoma with mucin production by transbronchial lung biopsy. Right middle lobectomy was performed, and the pathologic examination disclosed a pulmonary mucinous cystadenocarcinoma.
Cystadenocarcinoma, Mucinous/pathology/*radiography/surgery ; Diagnosis, Differential ; Female ; Fluorodeoxyglucose F18/diagnostic use ; Humans ; Lung Neoplasms/pathology/*radiography/surgery ; Middle Aged ; Positron-Emission Tomography and Computed Tomography ; Radiopharmaceuticals/diagnostic use ; Tomography, X-Ray Computed/*methods

OBJECTIVETo study the clinical manifestations, radiologic findings, pathologic diagnosis and differential diagnosis of primary osteosarcoma in elderly patients.

METHODSTwelve cases of primary osteosarcoma occurring in patients older than 60 years were encountered during the period from 1985 to 2010. The clinical manifestations, radiologic features and pathologic findings were studied and the follow-up data were analyzed.

RESULTSThe sites of involvement included long bones (number = 7), ilium (number = 1), craniofacial bones (number = 2) and soft tissue (number = 2). Radiologic examination showed a mixture of osteosclerotic and osteolytic lesions in 10 patients, soft tissue lesions with high-density areas in 2 patients and soft tissue lesions with periosteal reaction in 8 patients. Histologically, most cases showed features of conventional osteosarcoma. There were 2 cases of malignant fibrous histiocytoma-like osteosarcoma, 2 cases of chondroblastic osteosarcoma and 1 case of well-differentiated intraosseous osteosarcoma. Immunohistochemical study played little role in pathologic diagnosis. Ten patients had undergone amputation, including one patient who had received adjuvant chemotherapy beforehand. Nine patients had follow-up information available. Three of them died of lung metastasis and 1 died of cardiovascular disease.

CONCLUSIONSPrimary osteosarcoma rarely occurs in elderly patients and can easily be missed. Correlation with clinical, radiologic and histologic features is important for arriving at a correct diagnosis.

12E7 Antigen ; Aged ; Antigens, CD ; metabolism ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Cell Adhesion Molecules ; metabolism ; Chondrosarcoma ; pathology ; Diagnosis, Differential ; Female ; Femoral Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Follow-Up Studies ; Humans ; Ilium ; Lung Neoplasms ; secondary ; Lymphoma ; pathology ; Male ; Middle Aged ; Osteitis Deformans ; pathology ; Osteosarcoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Radiography ; Soft Tissue Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

OBJECTIVETo study the clinicopathologic features and differential diagnosis of myxoid dermafibrosarcoma protuberans (DFSP).

METHODSThe clinical and pathologic features of 16 cases of myxoid DFSP were reviewed.

RESULTSThere were altogether 15 males and 1 female. The age of the patients ranged from 11 to 73 years (mean = 47 years and median = 48 years). The commonest site of involvement was trunk (number = 11), followed by shoulder (number = 2), head and neck (number = 2), and extremity (number = 1). Similar to conventional DFSP, most patients presented with a slowly enlarging subcutaneous nodule which showed a rapid recent growth in some cases. Amongst the 16 cases studied, 12 occurred de novo and 4 represented local recurrence. The tumors ranged from 2 to 10 cm in greatest dimension (mean = 5 cm and median = 4 cm). Histologically, they were poorly circumscribed and located in the dermis, with focal infiltration into the underlying subcutaneous tissue. Seven cases were purely myxoid and composed of spindly and stellate cells with delicate arborizing vascular meshwork. The remaining 9 cases were predominantly myxoid (> 50%), with 5 cases containing cellular areas resembling conventional DFSP and 4 cases showing fibrosarcomatous transformation. In addition, foci of giant cell fibroblastoma-like areas were noted in 1 case. Immunohistochemical study showed that the tumors cells were positive for CD34. The staining was weak in the myxoid areas, as compared with conventional DFSP. Of the 4 recurrent cases, one patient developed lung metastases.

CONCLUSIONSMyxoid DFSP represents a rare variant of DFSP and may pose important diagnostic pitfalls. It is especially so if the tumor purely consists of myxoid element. Familiarity with the histologic features helps to avoid misdiagnosis.

Abdominal Wall ; Adolescent ; Adult ; Aged ; Antigens, CD34 ; metabolism ; Child ; Dermatofibrosarcoma ; diagnostic imaging ; immunology ; pathology ; surgery ; Diagnosis, Differential ; Female ; Head and Neck Neoplasms ; diagnostic imaging ; immunology ; pathology ; surgery ; Humans ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Radiography ; Shoulder ; Skin Neoplasms ; diagnostic imaging ; immunology ; pathology ; surgery ; Young Adult