Correction: Pleuropulmonary blastoma, not rhabdomyosarcoma in a congenital lung cyst.
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Biomedical subjects
Publications and source records attributed to Wan-Ting Huang.
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We report a rare case of primary synovial sarcoma of the lung. A57-year-old man had a well-defined tumor in the right middle lobe seen on chest computed tomography, and underwent lobectomy. Grossly, the nonencapsulated tumor measured 4.5 cm in greatest diameter, with a solid and tan-white cut surface. Histologically, the tumor was mainly composed of a dense proliferation of spindle cells. Immunohistochemical studies were focally positive for epithelial membrane antigen, and diffusely positive for CD99 and Bcl-2. Cytokeratin, S-100 protein, desmin, smooth muscle act in, and CD34 were absent. SYT-SSX1 gene fusion transcript was detected by reverse transcription-polymerase chain reaction, which is diagnostic of primary synovial sarcoma of the lung. We also review the literature with regard to the clinicopathologic, immunohistochemical, and molecular studies of primary pulmonary synovial sarcoma.
Mantle cell lymphoma (MCL) is a rare B-cell lymphoma that has never been characterized in Taiwan. The purpose of the present paper was to retrospectively identify 21 cases in male patients, with a median age of 61, involving lymph node (91%), marrow (71%), and peripheral blood (23%). Eighteen (86%) were in stages III/IV with 1 and 5 year survival rates of 78% and 17%, respectively. Mixed nodular and diffuse pattern (45%) was most common while interstitial pattern (92%) predominated in marrow. Eighteen (86%) were of classical morphology, two were pleomorphic and one was blastic. The tumors expressed IgM and bcl-2 (100%), cyclin D1 (95%), CD5 (86%), CD43 and IgD (62%), CD52 (60%), and bcl-6 (5%). Ki-67 index>or=30% (P=0.1834) was associated with a trend toward poorer survival while p21, p27, or p53 expression was not statistically significant for survival. Real-time polymerase chain reaction for cyclin D1 (CCND1) gene mRNA expression showed high levels in nine cyclin D1-positive patients and a low level in the single cyclin D1-negative patient. The latter patient was cyclin D2 positive and negative for immunoglubuin heavy chain gene and CCND1 gene translocation by locus-specific interphase fluorescent in situ hybridization. In conclusion, it is confirmed that the usual morphological variants and aberrant immunophenotype of MCL in the West occur in Taiwan and that this disease carries a poor prognosis.
Adrenal cortical tumour-induced malignant hypertension is rare, except for some documented aldosterone-producing adenomas. Hypertension is a common feature of Cushing's syndrome, whereas malignant hypertension is rarely seen. This case in Taiwan is only the second reported case with malignant hypertension secondary to a cortisol-secreting adrenal tumour. The immunohistochemical study of the excised tumour showed strong positive staining of interleukin (IL)-6. IL-6 can trigger inflammatory vascular damage, which is typical in the malignant phase of hypertension. We hypothesise that IL-6 may have played an important role in this case.
Rhabdomyosarcoma arising within a congenital cystic adenomatoid malformation (CCAM) is an unusual entity. The patient underwent a lobectomy of his right lower lobe of lung due to a CCAM at the age of two. One year later, he developed a solid embryonal rhabdomyosarcoma at the same location. He received 1-year period chemotherapy and when the tumor reduced to a resectable size, surgical excision was done. The tumor cells appeared more differentiated after chemotherapy. The patient remains disease free to date, 16 months after surgery.
Primary cervical lymphoma is rare, with less than 60 cases reported in the English literature, including a few cases of synchronous cervical intraepithelial neoplasia (CIN) with cervical lymphoma. We describe a 42-year-old woman with CIN 3 and malignant lymphoma of the cervix. The Papanicolaou cytology only revealed dysplastic cells of CIN 3, but not the malignant lymphoid cells. The histology of the cervix revealed diffuse infiltration of monotonous medium-sized lymphoid cells with dispersing tingible bodies, and a Burkitt's lymphoma in combination with a small CIN 3 lesion was diagnosed. Neither human papillomavirus nor Epstein-Barr virus was identified by polymerase chain reaction or in situ hybridization. The patient developed leukemic spreading and died soon after surgery.
Columnar-cell carcinoma is a rare form of papillary thyroid carcinoma. It is a biologically aggressive variant, according to the World Health Organization classification, due to its rapid growth, high local recurrence rate, and frequent lung, brain, and bone metastases. However, recent reports indicate encapsulated tumors confined to the thyroid gland are associated with a more favorable prognosis. We report a case of encapsulated columnar-cell carcinoma occurring in the right lobe of the thyroid in a 25-year-old female and discuss the histopathologic features, differential diagnosis, and prognostic factors.
BACKGROUND: In Taiwan, lung cancer is the second most common cause of cancer death and its incidence has been rising for the last 50 years. Shifts in histological types and differences in gender distribution have also accompanied the changed incidence of lung malignancies. METHODS: A total of 590 lung cancer patients were interviewed at Kaohsiung Chang-Gung Memorial Hospital, a medical center of Southern Taiwan, in 1997 and 2002. A retrospective investigation confirmed the age-adjusted incident rate in the hospital and demographic variations by different histological types for both genders. The statistical differences were evaluated using the heterogeneity chi-squared test and Cox regression. RESULTS: Results indicated that from 1997 to 2002, the age-adjusted rates of lung cancer decreased by 3.64% at the hospital. The largest percentage of increases in the age-adjusted rate was observed for small cell lung cancer (approximately 8.18%), whereas it decreased by 31.2% for squamous cell carcinoma and increased by 1.62% for adenocarcinoma. Female patients were found to be younger and had longer survival duration. The frequency was the highest for lesions in the upper lobe and patients had more advanced stage in all histological types. The 6-month relative survival rate between the two time-periods did not change appreciably. CONCLUSIONS: The age-adjusted incidence rate of adenocarcinoma at the hospital has increased, as well as small cell lung carcinoma. During the study period, early-staging diagnosis and 6-months survival rate did not change appreciably for the different histological lung cancer patients, suggesting that therapeutic and diagnostic advances, prevention or screening procedures had mild effects in southern Taiwan. Further studies are needed for confirmation of our results.
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Cellular schwannoma is a variant of classical schwannoma that is characterized by high cellularity. As it is accompanied by mitotic figures, it is easily mistaken for a malignant neoplasm. However, hyalinized thick-walled blood vessels, an alternating growth pattern of spindle-shaped neoplastic cells, and even Verocay bodies can be found. It is most commonly seen in the paravertebral area, particularly in the mediastinum and retroperitoneum. Retrobulbar cellular schwannoma is uncommon, and only one case has been reported to date. In this study, we report two additional cases of retrobulbar cellular schwannoma that recurred two years after surgery. We suggest that the patient be given an aggressive postoperative treatment because a successful, complete excision cannot be guaranteed at such an anatomic site.
We describe an unusual case of pneumatosis cystoides intestinalis (PCI) in a patient with chronic pulmonary disease. A 79-year-old woman was hospitalized due to abdominal fullness and bowel habit change. Colonoscopy revealed numerous round cystic lesions in the sigmoid colon, which bled easily on contact. Due to persistent local peritonitis, a left hemicolectomy with primary anastomosis was performed. The patient has done well in the 12 months following surgery.
Collagenous fibroma (desmoplastic fibroblastoma) is an extremely rare benign soft tissue tumor of fibroblastic origin. The majority of reported cases have been located in the deep subcutis, fascia, aponeurosis, or skeletal muscle of the extremities, limb girdles, or head and neck regions. There has been no mention of underlying diseases in patients who developed this tumor. We here report an additional three cases of superficial collagenous fibroma, one of which was a dermal lesion occurring in the abdomen of a 26-year-old male patient with a 5-year history of pemphigus vulgaris prior to development of the tumor. To the best of our knowledge, an association between collagenous fibroma and pemphigus vulgaris has not previously been reported. The remaining two tumors were located in the superficial subcutaneous tissue of the infrascapular area and right foot, respectively. There was no tumor recurrence or metastasis during follow up of 18, 25, and 47 months, respectively. All three tumors were well-circumscribed and unencapsulated without infiltrating borders. Histologically, the common denominator of all three cases was paucicellular proliferation of spindle or stellate fibroblasts enmeshed within an extensively collagenous background. Immunohistochemically, there was diffuse strong staining for vimentin and intense focal reaction for smooth muscle actin in two tumors tested. Electron microscopy revealed features consistent with a fibroblastic or myofibroblastic lineage. Flow cytometry in two cases demonstrated a diploid DNA content with low S-phase fractions, which correlated with minimal MIB-1 nuclear labeling (less than 1%) and benign behavior of this entity.