Cutaneous myoepithelioma?
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Biomarkers, Tumor↗
Biomedical subjects
C D Fletcher
Publications and source records attributed to C D Fletcher.
Intradermal spindle cell/pleomorphic lipoma: a distinct subset.
Spindle cell/pleomorphic lipomas are a group of benign lipogenic tumors composed of primitive spindle cells, multinucleated giant cells, and mature adipocytes. These tumors have rarely been reported to arise in the dermis and may be misdiagnosed in this location. Twenty (12.7%) intradermal lesions identified among 157 spindle cell/pleomorphic lipomas in the authors' files were studied clinicopathologically and immunohistochemically. The patients' ages ranged from 20 to 85 years (median: 42 years); 14 of 20 patients were female (70%). Anatomical sites were the head/neck region (7 cases, 4 of which arose on the face), shoulder/upper back (4 cases), lower limbs (4 cases), trunk (3 cases), and upper limbs (2 cases). Most lesions presented as a soft and slowly enlarging cutaneous nodule, usually measuring less than 2.5 cm. Histologically, these dermal lesions differed from usual spindle cell/pleomorphic lipoma, being unencapsulated with poorly defined infiltrative margins, although the cytomorphologic findings, ropy collagen, and mast cells were as seen in usual subcutaneous lesions. Six cases showed features of pleomorphic lipoma. Immunohistochemically, lesional cells stained positively for CD34 and were negative for S-100 protein. One case recurred locally after 21 years. Dermal spindle cell/pleomorphic lipomas are distinctive in their apparent female predilection, wider anatomical distribution than subcutaneous lesions, and lack of circumscription. These unusual features can cause problems in differential diagnosis.
Adult↗
Cellular pseudosarcomatous fibroepithelial stromal polyps of the lower female genital tract: an underrecognized lesion often misdiagnosed as sarcoma.
Fibroepithelial stromal polyps of the vulvovaginal region are benign lesions that, when bland or hypocellular, are readily recognized. However those that exhibit bizarre cytomorphology, atypical mitoses, or hypercellularity, raising the possibility of malignancy, continue to be underrecognized. The authors reviewed a series of fibroepithelial stromal polyps to characterize further the morphologic features that can lead to a misdiagnosis of sarcoma. A total of 33 of 65 consecutive cases of fibroepithelial stromal polyps retrieved from the authors' consultation files were remarkable for marked hypercellularity (33 of 33), marked cytologic pleomorphism (21 of 33), mitotic counts of more than 10 mitoses per 10 high-power fields (12 of 33), and the presence of atypical mitoses (14 of 33). A total of 16 of 33 lesions had three or more of these features. Important morphologic clues to the diagnosis (shared with usual polyps at this site) were lack of an identifiable lesional margin, extension of abnormal stromal tissue up to the mucosal-submucosal interface, and the frequent presence of individually scattered multinucleate stromal cells, most often located close to the surface epithelium. Immunohistochemically, seven of 12 cases were desmin positive and one of 11 cases were smooth muscle actin positive. The age range of patients was 16 to 75 years (median, 32 years), and 21 patients (64%) were premenopausal. Sites included the vagina (18 of 33), cervix (seven of 33), and vulva (eight of 33). A total of 14 of 33 patients were pregnant, three patients were taking Tamoxifen, and one patient was on oral progesterone. Eight of 33 patients had multiple lesions at the time of presentation, of whom five were pregnant. Clinical follow-up was available in 21 of 33 patients. Three of 21 patients with follow-up had local, nondestructive recurrence. Two of these patients had multiple recurrences. None of the patients followed developed metastases. Cytologic atypia has been a previously recognized feature in these lesions; however, the occurrence of marked stromal cellularity and a mitotic rate of more than 10 mitoses per 10 high-power fields have not been emphasized previously. Moreover, the combination of these features has only rarely been documented. Awareness of the spectrum of histologic features that these lesions can exhibit is crucial in their accurate recognition, thus avoiding potential overtreatment.
Adolescent↗
Primary giant cell tumor of soft tissues: a study of 22 cases.
Twenty-two cases of giant cell tumor of soft tissues (GCT-ST) identified in the Mayo Clinic files and the consultation files of two of the authors (A.G.N., C.D.M.F.) were analyzed clinicopathologically. Age at presentation ranged from 5 to 80 years (median, 43 years), and there was no sex predilection (12 male, 10 female). Duration of symptoms ranged from 2 to 12 months (median, 4.5 months), and a painless growing mass was the most common complaint. The lower limbs were the most frequent location (50%), followed by the trunk (31.8%) and the upper limbs (13.6%). The size of the tumors ranged from 1 to 10 cm, and they tended to be superficial (86.4%), forming well-circumscribed (72.7%), multinodular (86.4%) masses. Histologically, all tumors consisted of a mixture of mononuclear cells showing vesicular, round to oval nuclei and osteoclastlike, multinucleated giant cells distributed uniformly throughout the tumors. Foci of stromal hemorrhage were observed in 11 tumors (50%); nine tumors (40.1%) showed metaplastic bone formation and six (27.2%) showed aneurysmal bone cystlike areas. Necrosis was absent in all but one tumor. Mitotic figures were present in all but one tumor, ranging from two to more than 30 mitoses per 10 high-power fields (HPFs; median, 9.5 mitoses per 10 HPFs) and were typical in aspect. Vascular invasion was identified in seven tumors (31.8%), and none of the tumors showed marked cellular atypia or pleomorphism. The tumors were treated surgically, and follow-up information was available for 16 patients (duration of follow-up, 2 to 130 months; median, 51 months). Only one of the 16 patients (6.2%) had local recurrence and lung metastases; this patient died of the tumor. In conclusion, GCT-ST occurs as a primary soft-tissue neoplasm and is identical clinically and morphologically to giant cell tumor of bone. Provided that GCT-ST is treated adequately by complete excision, a benign clinical course is expected because episodes of distant metastasis and tumor-associated death seem to be exceedingly rare.
Adolescent↗
Composite hemangioendothelioma: a complex, low-grade vascular lesion mimicking angiosarcoma.
Eight cases of a previously uncharacterized vascular neoplasm, showing varying combinations of benign, low-grade malignant, and malignant vascular components are described. Seven tumors occurred in the dermis and/or subcutis and one occurred in the oral submucosa. The patients were all adults with a median age of 39.5 years (range, 21-71 years). Five patients were men. The tumors arose predominantly in the hands and feet, and the lesions were usually of several years duration. The tumors were composed of a complex admixture of histologic components that varied from tumor to tumor, such that no two tumors looked precisely the same. This was due to variation in the proportions of each component as well as the manner in which each component was distributed throughout each lesion. The predominant histologic components were epithelioid hemangioendothelioma (HE) and retiform HE, which were each present in seven of the tumors. Areas of spindle cell HE were identified in four lesions. Angiosarcoma-like elements were identified in seven tumors. One of the tumors was associated with an arteriovenous malformation and one was associated with an area of lymphangioma circumscriptum. Of six cases with follow up (median duration, 6.5 years), three have recurred locally and, to date, only one has metastasized. We think composite HE is best regarded as a low-grade malignant vascular neoplasm, and the available data suggest that it behaves more favorably than conventional angiosarcoma. The existence of these composite lesions has led to careful reexamination of the concept of HE. The term HE, in that it is currently synonymous with a low-grade malignant vascular tumor, should be reserved for lesions that have true metastatic potential, albeit with low frequency.
Adult↗
Diffuse-type giant cell tumor: clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease.
The clinical and pathologic features of 50 cases of diffuse-type tenosynovial giant cell tumor (D-TGCT), also known as extraarticular pigmented villonodular tenosynovitis (PVNTS), are presented. Patients' ages ranged from 4 to 76 years (median, 41 yrs), with a slight female predominance (28 women, 22 men). By definition, all lesions presented as predominant soft tissue masses, with or without an associated articular component. Tumor sites included the wrist (9 cases), knee (8 cases), thigh and foot (6 cases each), finger (5 cases), ankle (3 cases), hand, elbow, toes, buttock, paravertebral region (2 cases each), lower leg, sacrococcygeal area, and retroperitoneum; 27 cases were described as entirely extraarticular. Tumors showed infiltrative margins and, in most cases, a sheet-like growth pattern. Striking variation in the number of osteoclast-like giant cells, foamy cells, amount of hemosiderin, and in the degree of stromal hyalinization were responsible for a wide morphologic spectrum. In addition to the predominant histiocyte-like cells, we identified in most cases a subpopulation of large dendritic, desmin-positive cells showing characteristic, but potentially misleading, cytologic features, including abundant eosinophilic cytoplasm, large vesicular nuclei, paranuclear eosinophilic inclusions, and occasional nuclear inclusions. Follow-up information was available for 24 patients, with a duration ranging from 6 months to 30 years (mean, 55 mos). Local recurrence occurred in eight cases (33%), between 4 months and 6 months after surgery (median, 15 mos) and was repeated in five cases; recurrence did not appear to correlate with morphologic parameters. Six cases showed atypical histologic features and four of these contained areas of sarcomatous change. Among the latter, one of three cases with available follow up developed pulmonary metastases and died after 35 months. In addition, one histologically benign lesion gave rise, after two local recurrences, to inguinal and iliac lymph node metastases. Despite this exceedingly uncommon event, we think most cases of D-TGCT are best regarded as benign but locally aggressive neoplasms with significant recurrent potential and should be treated, when possible, by wide excision. Atypical features such as increased mitotic activity, necrosis, spindling of the mononucleate cells, and cytologic atypia are not indicative of malignancy when present individually. This study also confirms the existence of malignant tenosynovial giant cell tumors, some of which are characterized by aggressive behavior.
Adolescent↗
Myxoid leiomyosarcoma of soft tissue, an underrecognized variant.
Myxoid leiomyosarcoma is an uncommon tumor which, although previously well described in the uterus, is recognized to a lesser extent at other sites. We describe 18 cases of soft tissue leiomyosarcoma in which myxoid stroma occupied >50% of the tissue examined. Patients ranged in age from 22 to 84 years old (median, 57.5 yrs) and female patients outnumbered male patients 14 to 4. Tumor locations included the limbs (6 cases), female external genitalia (4 cases), head and neck region (3 cases), chest (2 cases), nipple, paratesticular soft tissue, and perineum (one case each). The tumors had a grossly gelatinous appearance and adopted three major histologic architectures: fascicular, reticular/microcystic, and "myxofibrosarcoma-like." The tumor cells were predominantly spindled in all cases with typical features of smooth muscle differentiation; there was a mixture of spindle and epithelioid cells in one case. No cases with pure epithelioid cytology were seen. All tumors displayed immunoreactivity for smooth muscle markers (smooth muscle actin 16/17, desmin 8/18) and, in addition, four cases were positive for keratin CAM 5.2 and three for epithelial membrane antigen. The tumors had a tendency to be morphologically lower grade (9 tumors were grade I, 8 were grade II, and only 1 was grade III). Follow up was available in 13 patients with a duration of 8 months to 41 years (median, 39 mos), and revealed local recurrences (often repeated) in five cases and metastases in two cases. There were three tumor-related deaths, of which two were the result of uncontrolled local disease. The differential diagnosis of myxoid leiomyosarcoma is broad and encompasses both benign and malignant lesions. Accurate diagnosis is critical because therapies may differ widely for entities in the differential diagnosis of myxoid leiomyosarcoma.
Adult↗
Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series.
The clinicopathologic features of 12 cases of benign lymphangioendothelioma (acquired progressive lymphangioma) are reported. There were five male and seven female patients. Age at diagnosis ranged from 17 to 90 years (median age, 54 yrs). Development of a single macular/papular hemangiomatous or pigmented lesion was the main presenting symptom. Symptom duration before diagnosis ranged from 2 months to 20 years (median, 5.5 yrs). Tumor size ranged from 0.3 cm to 10 cm (median. 1.5 cm). Location included skin of the head and neck (n = 5), back (n = 1), breast (n = 1), shoulder (n = 1), forearm (n = 1), plantar aspect of the foot (n = 2), and oral mucosa (n = 1). No patient had any other concomitant vascular anomaly (for example, lymphangiomatosis) or was suspected to have acquired immunodeficiency syndrome. Treatment consisted of excisional biopsy in nine patients, incisional biopsy in two, and wide excision in one. Follow-up information on nine patients (range, 4-40 mos; median, 12 mos) showed two local recurrences in one patient. Microscopically, the lesions consisted of anastomosing, often widely dilated vascular structures developing in the superficial dermis. As the lesion grew within deeper dermis, the vascular spaces collapsed and dissected the dermal collagen in an angiosarcoma-like pattern. The lining endothelium was flat and monolayered, with little or no cytologic atypia and no evident mitoses. Some vascular structures contained stromal papillary projections resembling papillary endothelial hyperplasia, and intravascular red blood cells were present occasionally. Immunohistochemistry performed in eight specimens showed variable endothelial cell reactivity for CD31 (7 of 8), CD34 (7 of 7), and factor VIII-related antigen (4 of 6). A smooth muscle cell layer was observed focally around the vascular spaces in six lesions. Benign lymphangioendothelioma (acquired progressive lymphangioma) is an uncommon benign lesion that, in view of major differences in treatment and prognosis, should be distinguished from well-differentiated angiosarcoma and Kaposi's sarcoma, especially the patch stage and lymphangioma-like variants of the latter.
Adolescent↗
The role of megestrol acetate as an alternative to conventional hormone replacement therapy.
OBJECTIVE: To investigate the effect of megestrol acetate on menopausal symptoms, lipid metabolism, bone metabolism and coagulation. METHODS: In a prospective observational study, 71 postmenopausal women, for whom conventional hormone replacement therapy (HRT) was unsuitable, were treated with megestrol acetate 40 mg per day. At 0, 3, 6 and 12 months, fasting lipoproteins, bone biochemistry and thrombophilia profiles were measured and symptom score cards (Greene climacteric scale) completed. Bone mineral density measurement was performed at 0 and 12 months. RESULTS: Forty-one women completed the study. Treatment produced significant decreases in psychological (p < 0.001), vasomotor (p < 0.001) and somatic (p < 0.01) symptoms. There were significant reductions in triglycerides (p < 0.001), total cholesterol (p < 0.001), very-low-density lipoprotein (VLDL) (p < 0.05), low-density lipoprotein (LDL) (p < 0.001), high-density lipoprotein (HDL) cholesterol (p < 0.001) and lipoprotein(a) (p < 0.001). Levels of protein C were reduced (p < 0.05) and fibrinogen increased (p < 0.05). Protein S, plasminogen and antithrombin III levels showed an upward trend, which did not reach statistical significance. Biochemical markers of bone turnover did not change, apart from a significant decrease in alkaline phosphatase. Spinal bone density decreased significantly after 12 months, while femoral neck density remained unchanged. CONCLUSIONS: Megestrol acetate controls menopausal symptoms, has equivocal effects on cardiovascular risk markers and does not increase bone density. It is useful where estrogen is contraindicated.
Adult↗
Uptake of radiolabeled somatostatin analog is detectable in patients with metastatic foci of sarcoma.
BACKGROUND: Somatostatin receptors are present on many types of epithelial tumors, and ligands targeting these receptors are used to treat patients with neuroendocrine malignancies. Preclinical studies have demonstrated the presence of somatostatin receptors on a variety of mesenchymal tumors by in vitro receptor autoradiography. The use of radiolabeled somatostatin analogs to assess the presence of somatostatin receptors in vivo has been established, but use of this technique to evaluate human sarcomas has not been reported previously. METHODS: Seventeen patients (13 females and 4 males) with metastatic sarcoma underwent imaging via somatostatin-receptor scintigraphy. Scans were performed using indium -111 pentetreotide. Planar studies and single photon emission computed tomography imaging were performed at 4 and 24 hours, and results of scintigraphy were correlated with computed tomography findings. RESULTS: Twelve of 17 scans showed increased uptake in regions of known metastatic disease. There was no apparent correlation with scan positivity and patient age, histology, site of disease, or duration of diagnosis. CONCLUSIONS: Seventy-one percent of patients with advanced soft-tissue sarcomas had positive scintigraphy scans demonstrating tumor expression of somatostatin receptors subtype 2 in vivo. Imaging with indium-111 pentetreotide could be studied as an adjunct to conventional imaging modalities for assessment of sarcoma patients. Further research is needed to determine the prognostic implications of somatostatin receptor subtype 2 positivity, including larger studies to evaluate any potential correlation with metastatic behavior and other clinical outcomes.
Abdominal Neoplasms↗
The T-cell activation markers CD30 and OX40/CD134 are expressed in nonoverlapping subsets of peripheral T-cell lymphoma.
The tumor necrosis factor (TNF) receptor family includes several important markers of activation in T cells. We examined expression patterns of two T-cell-associated members of these receptors, namely CD30 and OX40/CD134, in 148 cases of T-cell lymphoma to identify possible objective immunohistochemical criteria for subclassification of these tumors. CD30 expression was characteristic of tumors with an anaplastic (46/47 cases [98%]) or large-cell (10/21 [48%]) morphology and was seen in only scattered cells in other tumor types. In contrast, large numbers of OX40/CD134(+) tumors cells were typical of angioimmunoblastic lymphoma (15/16 [94%]), angiocentric lymphoma (4/4), a subset of large-cell lymphomas (10/21 [48%]), and lymphomas with a prominent histiocytic component (6/7 [86%]). Strong OX40/CD134 and CD30 coexpression was seen in only 4% of tumors, typically those with an anaplastic/Hodgkin's-like appearance. OX40/CD134 expression was characteristic of tumors composed of activated CD4(+) T cells and was not seen in small-cell T-cell lymphomas, lymphoblastic lymphomas, or other tumor types, including B-cell lymphomas or carcinomas. These results suggest that immunostaining for OX40/CD134 may be helpful in subclassification of peripheral T-cell lymphomas and that the patterns of TNF receptor family expression in these tumors may parallel those seen within nonneoplastic helper T-cell subsets.
Antigens, CD↗
Induction of solid tumor differentiation by the peroxisome proliferator-activated receptor-gamma ligand troglitazone in patients with liposarcoma.
Agonist ligands for the nuclear receptor peroxisome proliferator-activated receptor-gamma have been shown to induce terminal differentiation of normal preadipocytes and human liposarcoma cells in vitro. Because the differentiation status of liposarcoma is predictive of clinical outcomes, modulation of the differentiation status of a tumor may favorably impact clinical behavior. We have conducted a clinical trial for treatment of patients with advanced liposarcoma by using the peroxisome proliferator-activated receptor-gamma ligand troglitazone, in which extensive correlative laboratory studies of tumor differentiation were performed. We report here the results of three patients with intermediate to high-grade liposarcomas in whom troglitazone administration induced histologic and biochemical differentiation in vivo. Biopsies of tumors from each of these patients while on troglitazone demonstrated histologic evidence of extensive lipid accumulation by tumor cells and substantial increases in NMR-detectable tumor triglycerides compared with pretreatment biopsies. In addition, expression of several mRNA transcripts characteristic of differentiation in the adipocyte lineage was induced. There was also a marked reduction in immunohistochemical expression of Ki-67, a marker of cell proliferation. Together, these data indicate that terminal adipocytic differentiation was induced in these malignant tumors by troglitazone. These results indicate that lineage-appropriate differentiation can be induced pharmacologically in a human solid tumor.
Adult↗
How shall we apply the new biology to diagnostics in surgical pathology?
Molecular technology plays an ever-increasing role in academic pathology and frequent claims are made that these technical developments will revolutionize diagnostic histopathology. In reviewing the role and potential utility of molecular genetics in the clinical practice of tumour pathology, it is evident that claims have outweighed facts, at least to date, and while molecular data have helped to clarify specific small areas of tumour classification and have facilitated the diagnosis of certain relatively rare tumours (especially on limited tissue), the overall impact on cancer management has been small. In particular, molecular markers of prognosis or tumour response have yet to impact substantially on the role of conventional light microscopic examination. We believe that these shortcomings have more to do with the generally different goals of basic medical science and clinical practice. In order to optimize the benefit to patients of modern molecular pathology, these techniques need to be rigorously appraised in the clinical setting and properly analysed in terms of cost-benefit. This requires close collaboration between the basic research community and histopathologists. Suggestions that 'H&E' pathology will be superceded in the near future are both premature and unrealistic.
Biomarkers, Tumor↗
Classification of human liposarcoma and lipoma using ex vivo proton NMR spectroscopy.
Prognostication in patients with liposarcoma is a complex and controversial subject based on recognition of lipoblasts, adipocyte nuclear atypia, and qualitative estimations of cellularity and cell size. We show here that for 30 patients with liposarcoma and 5 patients with lipoma, spectral differences on high-resolution, magic angle spinning proton nuclear magnetic resonance (hr-MAS 1H-NMR) spectroscopy relate to known biochemical changes and correlate with adipocyte tissue differentiation, histologic cell type, and cellularity. The NMR-visible level of triglyceride is shown to correlate with liposarcoma differentiation, since the triglyceride level in well-differentiated liposarcoma is 33-fold higher on average than for myxoid/round cell liposarcoma, which in turn is 6-fold higher than the dedifferentiated and/or pleomorphic subtypes. The NMR-visible phosphatidylcholine level serves as an estimate of total tissue cell membrane phospholipid mass and was found to correlate with liposarcoma subtype. Pleomorphic liposarcoma, the most aggressive and metastatic subtype, was found to have a threefold increase in NMR-visible phosphatidylcholine level compared with dedifferentiated liposarcoma. The level of NMR-visible phosphatidylcholine was twofold greater in well-differentiated liposarcoma compared with lipoma and was threefold larger for the hypercellular myxoid/round cell subtype compared with the pure myxoid histology. Thus, NMR-derived parameters of tissue lipid may be used for objective distinction of liposarcoma histologic subtype/grade and lipoma from liposarcoma. These biochemical parameters may ultimately improve prognostication in patients with liposarcoma.
Humans↗
Correlation between clinicopathological features and karyotype in spindle cell sarcomas. A report of 130 cases from the CHAMP study group.
Soft-tissue tumors have proved to be a fruitful area for the identification of reproducible cytogenetic aberrations, especially among pediatric round-cell sarcomas and lipomatous tumors. Thus far, however, data regarding sarcomas of monomorphic spindle cell type have been limited and somewhat disappointing, with the notable exception of synovial sarcoma. As part of an ongoing international collaborative study, 130 karyotyped spindle-cell sarcomas were reviewed and classified histologically, without knowledge of the clinical and karyotypic data, with the aim of identifying objective correlations between morphology, karyotype, and clinical parameters. Clonal chromosomal abnormalities were identified in 82 cases studied (63%), but only in the group of synovial sarcomas was there clear correlation between the cytogenetic findings, in the form of a consistent t(X;18)(p11;q11), and morphology. Among leiomyosarcomas (41 cases) and malignant peripheral nerve sheath tumors (MPNSTs; 27 cases) as well as in individual examples of rarer entities, there was a general tendency for karyotypic complexity associated with frequent loss or rearrangement of chromosome arms 1p, 10p, 11q, 12q, 17p, and 22q. Rearrangements of 17q (the region of the NF1 gene) were seen in 9/27 (33%) of MPNSTs. Among nine cases of solitary fibrous tumor (in which previous cytogenetic data are very limited) no consistent aberrations were identified. We conclude that, with the exception of synovial sarcoma, most spindle-cell sarcomas share with pleomorphic sarcomas the tendency for karyotypic complexity. There was no indication (in most of these lesions) that detectable cytogenetic aberrations could either facilitate their diagnosis or help to determine prognosis. There is a clear need to further study and understand the significance of multiple chromosomal abnormalities in this group of mesenchymal neoplasms with the particular goal of determining their role in the process of tumor development.
Adolescent↗
Primary juxtaarticular soft tissue lymphoma arising in the vicinity of inflamed joints in patients with rheumatoid arthritis.
AIMS: Primary soft tissue lymphoma is uncommon and little is known regarding its aetiology and pathogenesis. In a review of 37 soft tissue lymphomas we uncovered three cases associated with rheumatoid arthritis which we report herein. METHODS AND RESULTS: The clinical records and pathology of the cases are described together with the results of in situ hybridization studies with oligonucleotide probes to Epstein-Barr virus (EBV) encoded RNA (EBER). All three patients were females with a long-standing history of rheumatoid arthritis ranging from 9 to 17 years. Each presented with a soft tissue mass in the vicinity of a joint affected by rheumatoid disease. All had received prior treatment with nonsteroidal anti-inflammatory drugs and one also received gold, penicillamine and intra-articular steroids to affected joints. None had received methotrexate. Histologically, the juxtaarticular soft tissue masses were all B-cell lymphomas. None were associated with EBV as determined by in situ hybridization. CONCLUSIONS: These cases document an association between rheumatoid arthritis and soft tissue lymphoma of B-cell type, arising in the vicinity of an affected joint. Chronic local immune stimulation may have played a significant role in the genesis of these lymphomas, unlike the frequently reversible and EBV-positive lymphomas that occur in rheumatoid patients on immunosuppressive therapy.
Adult↗
High-grade extraskeletal myxoid chondrosarcoma: a high-grade epithelioid malignancy.
AIMS: Extraskeletal myxoid chondrosarcoma is typically a low-to-intermediate grade sarcoma that is associated with a prolonged clinical course. High-grade forms are rare and not well characterized. In this series we report the clinicopathological, immunohistochemical and ultrastructural findings in four cases of high-grade extraskeletal myxoid chondrosarcoma. METHODS AND RESULTS: The patients were three men and one woman (ages 34-73 years) with tumours located in the thigh (two cases), paraspinal soft tissue and perineum. Three patients had metastases, one at 12 weeks, one at 10 months, and one at presentation of recurrent tumour. In the latter case the original tumour was low grade and became high grade when it recurred 3.5 years later. All three patients died of disease. One patient was lost to follow-up. The most striking histological feature in all four tumours was the presence of numerous large epithelioid cells. These cells were arranged in cords within myxoid matrix and in sheets devoid of matrix. Two tumours had areas of conventional extraskeletal myxoid chondrosarcoma intermixed with the high-grade areas. One tumour showed transition to high-grade spindle cell sarcoma. One tumour had cells with rhabdoid features. Immunohistochemically, two tumours focally expressed S100 protein, and one focally expressed EMA. All were negative with cytokeratin, desmin, smooth muscle actin, HMB45, CD31 and CD34. Ultrastructural features in three cases were compatible with chondrosarcoma; one tumour had aggregates of microtubules within rough endoplasmic reticulum, a characteristic feature of this tumour. CONCLUSIONS: High-grade extraskeletal myxoid chondrosaroma is a rare and aggressive soft tissue sarcoma, and should be included in the differential diagnosis of other epithelioid malignancies.
Adult↗
Myxoid tumours of soft tissue.
Myxoid tumours of soft tissue encompass a heterogeneous group of lesions characterized by a marked abundance of extracellular mucoid (myxoid) matrix. This group of tumours demonstrate significant variability in their biological behaviour thus including tumours which are entirely harmless, tumours with a tendency to recur locally but not metastasize, and malignant tumours. There appears to be a considerable degree of overlap clinically and morphologically between the various tumour types in this group, generating potential diagnostic problems for the clinician and pathologist alike. While diligent microscopy remains the basis of diagnostic pathology, the continuous developments and refinements within the fields of immunohistochemistry and molecular cytogenetics are providing substantial new information, allowing the development of new diagnostic criteria and hence facilitating an accurate diagnosis. It is the aim of this short review to highlight the most prevalent soft tissue tumours with predominantly myxoid morphology, to describe the features by which the majority of these myxoid lesions may be identified, and to discuss the differential diagnosis where appropriate.
Chondrosarcoma↗