Leukaemic infiltration of an adenocarcinoma effusion.
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Biomedical subjects
Publications and source records attributed to T W Shek.
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Giant cell tumour of the tendon sheath is a soft tissue mass found occasionally in the hand. Its diagnosis can be readily made preoperatively if the characteristic MRI features are appreciated. This pictorial essay demonstrates and describes the imaging findings correlated with histopathological findings in a group of patients with proven giant cell tumour of the tendon sheath.
The rib is an uncommon site of osteosarcoma. With the use of adjuvant chemotherapy and aggressive surgical resection of the metastatic pulmonary lesions, extrapulmonary metastases are becoming more clinically evident. Primary rib osteosarcoma with extrapulmonary metastasis is exceedingly rare. A case is reported, showing that the pattern of metastasis of rib osteosarcoma is similar to that of primary bone osteosarcoma. The liver metastasis occurred after resection of the metastatic pulmonary lesions. A CT scan of the primary rib lesion and liver metastasis both showed a lace-like enhancement pattern, its histological appearance corresponding with neoplastic osteoid. With the increasing use of CT abdomen for localization of extrapulmonary metastases, lace-like enhancement may be seen more readily in the future.
Follicular dendritic cell (FDC) tumors are uncommon neoplasms that can involve lymph nodes or extranodal sites. They can exhibit a broad spectrum of histologic appearances and behavior, but the intra-abdominal ones usually pursue an aggressive course. The purpose of this study was to characterize a distinctive variant of FDC tumor morphologically mimicking inflammatory pseudotumor through analysis of the clinicopathologic features of 11 cases. The patients included 10 women and one man (age range, 19-61 years; median age, 40 years) who presented with abdominal discomfort or pain. Six patients had systemic symptoms such as marked weight loss, fever, or malaise. All tumors occurred in intra-abdominal sites: liver (n = 7), spleen (n = 3), and peripancreatic region (n = 1). Of the nine patients with follow-up data, six were alive and well, one developed recurrence at 9 years, and two had repeated recurrences over many years. Grossly, the tumors were usually solitary and fleshy, punctuated by areas of hemorrhage and necrosis. Histologically, in a background of abundant lymphocytes and plasma cells were dispersed spindle or ovoid cells with vesicular nuclei and distinct nucleoli. The degree of nuclear atypia was variable, and some nuclei could be grotesque or resemble Reed-Sternberg cells. Focally, spindle cell fascicles could be formed. The atypical cells were immunoreactive for FDC markers such as CD21/CD35, CD23, and CNA.42. In situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was positive in all cases, remarkably highlighting the spindle cells and their atypia. EBV-latent membrane protein-1 was expressed commonly, albeit often focally and weakly. Therefore, inflammatory pseudotumor-like FDC tumor represents a distinctive variant of FDC tumor that differs from conventional FDC tumor in the following aspects: marked female predominance; selective localization in intra-abdominal sites, especially the liver and spleen; frequent presence of systemic symptoms; indolent behavior despite an intra-abdominal location; dispersed distribution of tumor cells and prominent lymphoplasmacytic infiltration; and consistent association with EBV.
This report describes a rare case of Ewing sarcoma (ES) of the small intestine. The patient was a 9-year-old girl with progressive abdominal distension. Computed tomography showed a large mass in the small bowel. Histopathologic examination of the resected tumor showed ES with typical histologic, immunohistochemical, and ultrastructural features. The tumor recurred in the pelvic cavity 18 months after the original surgery. Molecular study of the recurrent tumor confirmed a diagnostic EWS-FLI1 gene fusion. This patient illustrates the unique occurrence of ES in the small intestine.
A 46 year old man presented with a growing mass over his wrist. Erosions of the triquetrum and hamate were present radiographically. Magnetic resonance imaging (MRI) showed a solid mass arising from the extensor carpi ulnaris tendon, which was T(1) hypointense and isointense, T(2) hypointense, and bloomed on gradient echo images. The preoperative diagnosis of giant cell tumour of the tendon sheath was confirmed on histopathological examination of the excised specimen. The clinical, pathological, and imaging features, with emphasis on MRI findings, of this condition are reviewed.
This report describes a left adrenal tumour in a 16 year old Chinese girl who presented with fever of unknown origin. The histological and ultrastructural features of the adrenal tumour were those of a primitive small round cell tumour with neuroendocrine differentiation. Cytogenetic analysis of cultured tumour cells showed a reciprocal translocation t(12;22)(q13;q12). This is the first example of such a tumour being reported in the adrenal gland. The adrenal tumour was also the cause of the fever, which subsided after the removal of the tumour.
We present a case of primary nodal plasmacytoma in an elderly Chinese woman that was associated with significant paraproteinaemia and paraproteinuria. Clinical and laboratory features of the patient satisfied Durie's criteria for the diagnosis of multiple myeloma. The present case was unusual in two aspects. Firstly, there was no evidence of clonal plasma cell proliferation elsewhere in the body after extensive radiological investigations, repeated bone marrow examinations, and polymerase chain reaction for immunoglobulin gene rearrangement study. Secondly, the clinical behaviour was indolent despite the large amount of paraprotein production, and showed satisfactory disease control with local radiotherapy. The differential diagnoses of plasmacytosis in the lymph node are also discussed.
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Chondroblastoma is typically located in the epiphysis. Predominant metadiaphyseal location is very rare, as is involvement of the digits. We describe a case of chondroblastoma involving the metadiaphysis of the thumb. The patient was a 13-year-old boy who presented with pain and swelling of his left thumb. Radiographs showed an expanded lytic lesion involving the whole metaphysis and diaphysis of the proximal phalanx, which subsequently progressed to involve the epiphysis. Curettage and bone grafting were done.
Post-transplantation lymphoproliferative disorders (PTLD) are a group of heterogeneous diseases that occur after organ transplantation. Determination of the origin of the tumor cells not only provides clues to its possible pathogenetic mechanism, but also gives prognostic guidance in the clinical management of patients. We reviewed the clinicopathological features of four cases of PTLD that developed after solid organ transplantation. Using microsatellite analysis performed on paraffin-embedded tissue and using multiple, highly polymorphic markers, we have successfully determined the recipient/donor origin of the tumor cells in all of them. The time of onset of the PTLD ranged from 5 to 11 mo. All cases were diffuse large cell lymphomas of B-cell lineage, and the two cases that have been tested for EBV by in situ hybridization were positive. Three of the 4 PTLD were of donor origin and these three patients died of diseases unrelated to PTLD. The single patient with PTLD of recipient origin died of disseminated PTLD. The mean survival length of the three patients with donor origin was 26.3 mo, whereas that of the patient with recipient origin was 12 mo. Our results indicate a relatively high incidence of PTLD of donor origin among our patients with solid organ transplantation, as compared to other reported series. Moreover, the finding of the relatively indolent nature of PTLD of donor origin supports that determination of the donor/recipient origin of PTLD is of prognostic significance.
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A 16-year-old Chinese girl presented with AML-M5a. A bone marrow examination showed that the myeloblasts which were overwhelming the marrow contained giant granules (pseudo-Chediak-Higashi anomaly). Her karyotype showed a rare translocation t(10; 11)(p13; q14). Molecular delineation of the translocation breakpoints was not possible. Nonetheless, this case further demonstrates the morphological and phenotypic heterogeneity of acute leukaemia with this translocation. In this girl it was associated with disseminated intravascular coagulation.
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