Biomedical subjects

A G Nascimento

Publications and source records attributed to A G Nascimento.

At least 19 recordsLinked to original sources

PubMed2002

Lipoblastoma-like tumour of the vulva: report of three cases of a distinctive mesenchymal neoplasm of adipocytic differentiation.

AIMS: We sought to delineate and describe three cases of a distinctive mesenchymal neoplasm of the vulva showing adipocytic differentiation and affecting young patients. METHODS AND RESULTS: In three patients between 13 and 38 years of age, the vulvar tumours had well-circumscribed borders and ranged in size from 35 to 100 mm. Histologically, they were well circumscribed and lobulated. The lobules were separated by thin fibroconnective tissue septa and were composed of slender spindle cells showing slightly eosinophilic cytoplasm with indistinct boundaries, uniform nuclei with finely granular chromatin, and no nucleoli. The cells were embedded in a richly myxoid stroma. The background in all three tumours was a 'chicken-wire', capillary vascular network resembling that seen in myxoid liposarcomas. Two tumours had scattered signet-ring-type lipoblasts and the third a large number of such lipoblasts. Clusters of mature adipocytes were entrapped in the tumours. None had mitotic figures, necrosis, or pleomorphism. The neoplastic cells stained positively for vimentin and were negative for other immunohistochemical markers. Treatment for all three tumours was enucleation alone. After follow-up of 10 years, 7 years, and 1 year, all patients are well with no evidence of disease. CONCLUSIONS: The benign behaviour of these neoplasms militates against the diagnosis of liposarcoma. We believe these are benign lesions of adipocytic differentiation akin to infantile lipoblastomas.

Adipocytes↗

PubMed2002

Wide spectrum screening keratin as a marker of metaplastic spindle cell carcinoma of the breast: an immunohistochemical study of 24 patients.

AIMS: Metaplastic spindle cell carcinomas may be difficult to distinguish histologically from other spindle cell lesions in the breast. Variable staining with cytokeratin immunomarkers has been reported for metaplastic carcinomas. We evaluated the diagnostic utility of anti-cytokeratin polyclonal antibody, wide spectrum screening keratin, to assess spindle cell breast lesions. METHODS AND RESULTS: Twenty-four patients with spindle cell breast carcinoma and 31 patients with benign or malignant spindle cell tumours were studied using a panel of antibodies directed against multiple cytokeratins (AE1/AE3, CAM5.2, wide spectrum screening keratin), epithelial membrane antigen (EMA), and vimentin. Sites of origin for the 31 controls included breast, bone, and soft tissue. All but one (95.8%) metaplastic carcinomas stained positively with wide spectrum screening keratin. Only rare or focal immunoreactivity was observed with AE1/AE3 in four cases; however, sensitivity of AE1/AE3 was improved in 13 cases using steam EDTA as an antigen retrieval technique. Three cases were immunoreactive with CAM5.2 and eight cases were immunoreactive with EMA. All control cases lacked immunoreactivity with the cytokeratin panel and EMA. The spindle cells in the metaplastic breast tumours (88%) and in the controls (97%) stained with vimentin. CONCLUSIONS: Wide spectrum screening keratin may be the most useful and convenient antibody in differentiating metaplastic spindle cell carcinoma from other spindle cell lesions in the breast.

Aged↗

PubMed2001

Solitary fibrous tumor: a study of cytologic features of six cases diagnosed by fine-needle aspiration.

Solitary fibrous tumor (SFT) is a spindle-cell neoplasm most often presenting as a pleural-based tumor but increasingly recognized in other locations. Few reports have described the cytologic features of SFTs. Six cases of SFT diagnosed by fine-needle aspiration (3 pleura, 2 retroperitoneum, and 1 orbit) were identified in the Mayo Clinic files. The smears (Papanicolaou-stained) and corresponding histologic specimens were reviewed. Immunohistochemical staining for CD34 was performed in all cases. The cytologic findings were similar in all cases. The tumor cells were oval to polygonal, with cellularity ranging from scant to moderate. The background contained irregular ropy fragments of collagen and a few inflammatory cells. Most cells were dispersed singly, but all cases contained irregular, loose aggregates of cells enmeshed in a collagenous matrix. The nuclei were uniformly bland, with evenly distributed, finely granular chromatin. All cases were immunoreactive for CD34. SFT has distinctive cytologic features that allow diagnosis in cytologic specimens with the help of appropriate immunocytochemical stains on accompanying tissue biopsy specimens. Distinctive cytologic findings predictive of clinical behavior were not identified.

Adolescent↗

PubMed2001

Grading in soft tissue tumors: principles and problems.

Histologic grading has been considered the most important prognostic factor for soft tissue sarcomas. Several grading systems have been proposed based on the assessment of morphologic features in heterogeneous groups of sarcomas. Currently, the French Federation of Cancer Centers (FNCLCC) and the National Cancer Institute (NCI) grading systems are the most commonly used. These systems are based on a few morphologic predictors of biologic behavior, which is justifiable because of the rarity of soft tissue sarcomas. Nonetheless, over- or underestimation of prognosis may occur because of an uneven representation of specific sarcomas with rather distinct biologic behaviors among studies of grading systems. In addition, lack of standardization of morphologic criteria and frequent omission of the influence of clinical factors on the final survival analyses preclude universal acceptance of a particular grading system. New advances in diagnostic imaging, quantitative morphometric technologies, cytogenetics, and molecular genetics, allied with alternative analytic data systems, may provide better validation, reproducibility, and prognostic capabilities for current and future grading systems. This article summarizes and critically analyzes the various important grading systems that have thus far been proposed and suggests alternatives for the elaboration of more reproducible systems with higher predictive capabilities.

Adolescent↗

PubMed2001

Leptin and leptin receptor mRNA are widely expressed in tumors of adipocytic differentiation.

Adipose tissue is the principal source of leptin, a cytokine-like peptide with many biologic functions. Leptin binds to the leptin receptor, present in the hypothalamus and in many other tissues, and modulates energy balance and maintenance of body weight. The expression of leptin and leptin receptor in tumors of adipocytic differentiation has not been previously examined. Because normal adipose tissue is the principal source of leptin and expresses leptin receptor, we hypothesized that tumors of adipose tissue differentiation may also express leptin and/or the long functional form of the leptin receptor (OB-Rb). Leptin and OB-Rb were analyzed by immunohistochemistry, in situ hybridization, RT-PCR, and western blotting in 21 lipomas, 2 hibernomas, and 16 liposarcomas. Immunostaining and in situ hybridization showed leptin and OB-Rb mRNA expression in all cases of lipomas, hibernomas, and liposarcomas, including dedifferentiated and pleomorphic liposarcomas. RT-PCR analysis showed leptin and OB-Rb mRNA in both lipomas (n = 5) and liposarcomas (n = 5). Western blotting identified the 16 kDa leptin protein in a lipoma and a liposarcoma. No important difference in the expression of leptin and OB-Rb mRNA was found between lipomas and liposarcomas, although the level of leptin protein was higher in a lipoma than a liposarcoma by western blotting. These results show for the first time that leptin and OB-Rb mRNA are expressed in lipomas, hibernomas, and liposarcomas. The presence of leptin and its receptor may provide new insights into the pathobiology of these tumors.

Adult↗

PubMed2001

Inflammatory pseudotumor and sarcoma of urinary bladder: differential diagnosis and outcome in thirty-eight spindle cell neoplasms.

We assessed diagnostic criteria among 38 spindle cell tumors of the urinary bladder and obtained follow-up in 36 patients. Patients comprised 28 males and 10 females aged 2.5 months to 87 years. Hematuria was the commonest presenting symptom (27 patients). After review and immunohistochemical workup, 17 patients had inflammatory pseudotumor (myofibroblastic tumor), 4 postoperative spindle cell nodule, 1 leiomyoma, 13 sarcoma (7 low-grade; 6 high-grade), and 3 carcinoma. Mean age was 38 years for pseudotumor (range 15 to 74), 65 for postoperative spindle cell nodule, 51 for sarcoma, and 76 for carcinoma. Size of pseudotumor averaged 4.4 +/- 0.7 cm (range 1.5 to 13.0), similar to sarcoma, 4.0 +/- 0.6 cm (range 0.5 to 7.0). Similar proportions of benign tumors and sarcomas had muscularis propria invasion. The criteria that best differentiated sarcoma from inflammatory pseudotumor were presence of necrosis at the tumor-detrusor muscle interface in muscle-invasive cases, and nuclear atypia. Sarcoma also had less prominent microvasculature, less variable cellularity, consistently > or =1 mitotic figure per 10 high-power fields, and predominant acute inflammation without plasma cells. p53 protein nuclear immunostaining was moderate, unlike the rare to absent staining in pseudotumors. Because all 12 sarcomas were desmin-negative, we did not call them leiomyosarcoma; they overlapped with benign tumor in epithelial, mesenchymal, and actin immunostaining. Among 12 sarcoma patients, 2 died of tumor (at 3 months). Two of four experienced tumor recurrence after partial cystectomy (2 and 26 months). No pseudotumors recurred after transurethral resection or partial cystectomy, although one patient, 5 months after transurethral resection, had histologically identical pseudotumor that the surgeon considered residual. Another patient with pseudotumor, not a candidate for tumor ablation after transurethral resection, had continued tumor growth and he died of urosepsis. In conclusion, inflammatory pseudotumor, although overlapping with sarcoma in presentation, age range, and size, does not metastasize and remains histologically distinct from low-grade sarcoma.

Actins↗

PubMed2001

ETV6 rearrangements in patients with infantile fibrosarcomas and congenital mesoblastic nephromas by fluorescence in situ hybridization.

Congenital mesoblastic nephroma (CMN) and infantile fibrosarcoma (IFS) are two pediatric tumors arising in the kidneys and soft tissues of infants, respectively. Recently, a t(12;15)(p13;q25) resulting in ETV6-NTRK3 gene fusion was detected in patients with IFS and in patients with the cellular type of CMN, suggesting a common pathogenetic pathway. We investigated the presence or absence of ETV6 rearrangements and numerical abnormalities of chromosome 11 by using fluorescence in situ hybridization on paraffin-embedded material from five cases of IFS, two of CMN, and one of mixed type (CMN and IFS) found in our files. In three cases of IFS, we found ETV6 gene rearrangement but a normal copy number of chromosome 11. One case each of IFS, the cellular type of CMN, and the mixed type (CMN and IFS) had both abnormalities. In a case of classic CMN, neither trisomy 11 nor gene rearrangement was found. It is possible that trisomy 11 is a later, nonessential event in the pathogenetic process or that this secondary aberration is associated with still-unrecognized clinical or biological characteristics. We confirmed that IFS and the cellular type of CMN are cytogenetically related and can occur synchronously in the same organ.

Child, Preschool↗

PubMed2001

Extraskeletal myxoid chondrosarcoma: a light microscopic, immunohistochemical, ultrastructural and immuno-ultrastructural study indicating neuroendocrine differentiation.

AIMS: Extraskeletal myxoid chondrosarcoma is a rare low-grade soft-tissue sarcoma with locally aggressive and metastasizing potential. Extraskeletal myxoid chondrosarcoma has distinctive clinical, light microscopic, immunophenotypic, cytogenetic and ultrastructural features. Evidence that extraskeletal myxoid chondrosarcoma often shows neuroendocrine features was first provided by Chhieng et al. on the basis of an immunohistochemical and ultrastructural study of seven cases. Our study aims to further confirm by immunohistochemistry and ultrastructural studies, including immunoelectron microscopy, that extraskeletal myxoid chondrosarcoma indeed may show neuroendocrine differentiation. METHODS AND RESULTS: Fifteen cases of extraskeletal myxoid chondrosarcoma and seven control cases of skeletal chondrosarcomas were studied. Extensive immunohistochemical analysis was performed in all cases and ultrastructural studies were done in 11 extraskeletal myxoid chondrosarcomas and three skeletal chondrosarcomas. Immunoelectron microscopy was performed on one case each of extraskeletal myxoid chondrosarcoma and skeletal chondrosarcoma. Extraskeletal myxoid chondrosarcomas expressed neuron-specific enolase (100%), synaptophysin (87%), S100 (50%), PGP 9.5 (40%), and epithelial membrane antigen (25%). Co-expression of synaptophysin and PGP 9.5 was observed in six tumours. Skeletal chondrosarcomas showed expression of S100 protein, vimentin and neuron-specific enolase in all cases. Synaptophysin, chromogranin and PGP 9.5 were not expressed in any skeletal chondrosarcoma case. Ultrastructurally, extraskeletal myxoid chondrosarcoma was characterized by distinct cords of cells immersed in a glycosaminoglycan-rich matrix. The cells were rich in mitochondria, had well-developed Golgi apparatus and there were numerous smooth vesicles. In three cases there were easily found 140-180 nm diameter membrane-bound dense-core granules in cell bodies and in processes, unrelated to the Golgi, compatible with neurosecretory granules. Fewer such granules were present in the remaining extraskeletal myxoid chondrosarcoma cases, three of which also contained intracisternal tubules typical of extraskeletal myxoid chondrosarcoma. The skeletal chondrosarcomas had scalloped cell surfaces, prominent rough endoplasmic reticulum focally distended with secretory product, and lacked neurosecretory granules. Intermediate filaments were prominent in both extraskeletal myxoid chondrosarcoma and skeletal chondrosarcomas. Immunoelectron microscopy showed synaptophysin expression in the extraskeletal myxoid chondrosarcoma but not in the skeletal chondrosarcoma case. CONCLUSIONS: This study confirms that a substantial proportion of extraskeletal myxoid chondrosarcomas show immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation, and are unlikely to be related to conventional skeletal chondrosarcomas.

Adult↗

PubMed2001

Oligodendrogliomas: reproducibility and prognostic value of histologic diagnosis and grading.

Prognostic value of histological grading of oligodendroglial tumors is controversial and interobserver reproducibility in grading of these tumors is unknown. Seven neuropathologists and 6 surgical pathologists experienced in brain tumor-diagnosis assessed 124 oligodendroglial tumors operated at the Mayo Clinic (1960-1990). Among histologic parameters upon which current oligodendroglioma grading systems are based, only high cellularity, presence of mitoses, microcalcifications, endothelial hypertrophy, endothelial proliferation, and necrosis appeared to be reproducible. Reproducible histologic features, based on consensus ratings among neuropathologists (defined as > 60%), were evaluated for the association with cause-specific survival by fitting Cox regression models. By univariate analysis, a significant association with survival was found for age, high cellularity, presence of mitoses, endothelial hypertrophy and proliferation and necrosis. On multivariable analysis with a stepwise variable selection method, only age and presence of endothelial proliferation were found to be independently associated with survival with a discriminatory index of the model of 0.68. Mitotic index was significantly associated with survival based on the grading from each separate neuropathologist, but it was not based on consensus, most likely because this was classified as indeterminate in 54% of cases. Alternatively, "models fit" considering the assessment of single neuropathologists, identified a model based on age and on mitotic index with similar discriminatory indices of 0.69-0.7. Our study found few factors independently associated with cause specific-survival among morphological parameters. These findings are consistent with the present WHO stratification of oligodendrogliomas into low- and high-grade variants.

Adult↗

PubMed2001

Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of a clinicopathologically distinct tumor.

Sclerosing epithelioid fibrosarcoma (SEF) is an uncommon tumor of deep soft tissues, originally described in 1995 by Meis-Kindblom et al. In the current study, the authors identified 16 cases of SEF in the pathology files of their institutions and studied their pathologic features and disease course. The group consisted of six male and 10 female patients (age range, 14-55 years; mean age, 40 years), and the tumors were located in a limb or limb girdle (n = 7), base of the penis (n = 1), back or chest wall (n = 3), and head and neck (n = 5). Tumor size ranged from 3.7 to 22 cm (mean, 8.9 cm). Histologically, the SEFs were composed predominantly of small to moderate-size round to ovoid, relatively uniform cells, often with clear cytoplasm, embedded in a hyalinized fibrous stroma. The only consistent immunohistochemical finding was a strong, diffuse reactivity of tumor cells for vimentin. Ultrastructural analysis performed in eight cases confirmed their fibroblastic nature. Bone invasion and tumor necrosis, features not reported before, were found in six cases each. Treatment consisted of intralesional excision (n = 2), attempted wide local excision (n = 11), and amputation (n = 3), with either adjuvant radiation therapy (n = 9) or chemotherapy (n = 3). Follow-up of at least 1 year in 14 cases revealed persistent disease or local recurrence in seven patients (50%), and distant metastasis in 12 patients (86%). Eight patients (57%) died of disease 16 to 86 months after diagnosis. Five patients were alive with disease as of last follow-up. SEF shares some pathologic features with two other fibrosing fibrosarcomas, low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes, but in the authors' experience behaves clinically as a fully malignant sarcoma.

Adolescent↗

PubMed2001

Extraskeletal myxoid chondrosarcoma.

The medical records and histologic material of 16 patients with extraskeletal myxoid chondrosarcoma were reviewed. The mean age of the patients was 52 years. Thirteen tumors arose in the lower extremity. Thirteen patients presented with primary, localized disease, whereas three presented with pulmonary metastases. Treatment of the primary site included wide excision or amputation in 13 patients and marginal or intralesional resections with radiation in three patients. The mean followup was 7.4 years. Five patients were continuously disease free (5- and 10-year event free survival 43% and 14%, respectively). Local recurrence developed in four, and metastases developed in six of 13 patients presenting with localized disease. Of six patients who received chemotherapy for systemic disease, four had disease progression and died, and two had a response to chemotherapy (one partial, one complete). The mean survival after onset of metastases was 45 months. Overall 5- and 10-year survival was 87% and 63%, respectively. The current series suggests that extraskeletal myxoid chondrosarcoma is an intermediate-grade neoplasm with a tendency toward recurrence and metastasis. Survival after relapse may be prolonged. More effective therapy for systemic disease is needed.

Adult↗

PubMed2001

Is radiotherapy important for low-grade soft tissue sarcoma of the extremity?

Radiotherapy and limb-preserving surgery has replaced amputation and compartmental resection for treatment of soft tissue sarcomas. However, the role of radiotherapy in low-grade tumors remains unclear. This study reviews the outcomes of 132 patients who received multimodality treatment for low-grade soft tissue sarcoma. Large primary tumors (> 5 cm) and the absence of radiotherapy correlated with local recurrence. Radiotherapy was most effective in patients operated on with marginal margins. Patients who were treated with wide surgical margins or had small tumors (< or =5 cm) showed no benefit with adjuvant radiotherapy. Size greater than 5 cm and local recurrence correlated with metastasis. Radiotherapy appears to be important in the management of low-grade soft tissue sarcoma. The principles of local treatment for low-grade soft tissue sarcoma should be the same as for high-grade tumors with a combination of surgery and adjuvant radiotherapy. In a subset of patients with small and widely excised tumors, consideration may be given to withholding radiotherapy. Local recurrence and metastasis from low-grade soft tissue sarcoma may occur as long as 1 decade after primary tumor resection. Long-term review of patients with low-grade tumors may be indicated.

Adolescent↗

PubMed2001

Subcutaneous granuloma annulare in childhood: clinicopathologic features in 34 cases.

OBJECTIVE: To gather clinicopathologic data on subcutaneous granuloma annulare (SGA), a subtype of granuloma annulare that occurs exclusively in children and is histologically similar to rheumatoid nodules. DESIGN: Retrospective record review. PATIENTS: Children <10 years old in whom SGA, deep granuloma annulare, or necrobiotic granuloma was diagnosed at the Mayo Clinic (Rochester, MN) from 1983 to 1998. RESULTS: Thirty-four patients (21 girls and 13 boys; average age at diagnosis: 4.6 years) were found to have SGA. The lesions predominantly occurred in the lower extremity, especially in a pretibial location. Local recurrence within 1 month to 7 years was documented in 38.2%; recurrence at other locations was documented in 14.7%. Average follow-up was 60 months; during follow-up, no patients developed signs or symptoms of rheumatologic disease. Insulin-dependent diabetes mellitus was diagnosed in 2 patients, 1 before the development of SGA and 1 after it by 1 month. CONCLUSIONS: SGA is a lesion that presents as subcutaneous nodules on the lower extremities, hands, or scalp in young children. Recurrence is common but usually does not warrant additional biopsy.

Child↗

PubMed2001

Dedifferentiated liposarcoma.

Dedifferentiated liposarcoma is a high-grade nonlipogenic sarcoma that arises in a background of a preexisting well-differentiated liposarcoma. The phenomenon of dedifferentiation is time dependent, and primary or de novo tumors exceed secondary neoplasms in a ratio of 9:1. The tumor occurs most frequently in adults beyond the 6th decade of life, slightly predominates in men, and involves the abdominal cavity most often. Pleomorphic malignant fibrous histiocytoma-like histologic features are the most commonly observed phenotype, although other sarcomatous phenotypes have been described less frequently. Surgical treatment is the main form of therapy for dedifferentiated liposarcoma, which is associated with a reported local recurrence rate of 41% to 52%, 15% distant metastatic rate, and 30% disease-related mortality rate. On a chromosomal level, dedifferentiated liposarcoma frequently displays the same chromosomal abnormality associated with well-differentiated liposarcomas--ie, the presence of a supernumerary ring or giant chromosome derived from the 12q(13-15) region.

Chromosome Aberrations↗

PubMed2001

Pleomorphic liposarcoma.

Pleomorphic liposarcoma (PLS) is defined as a high-grade pleomorphic sarcoma containing multivacuolated lipoblasts. PLS arises most frequently in the extremities of older individuals and often metastasizes to the lungs. The prognosis of PLS parallels that of many other high-grade sarcomas. In a small analysis combining clinicopathologic data available from 16 previously reported and 8 additional cases of PLS arising in the extremities, the 5-year overall survival rate for the 24 patients was 40% (median survival, 48 months). By univariate analysis, patients with tumors located in the upper extremities (P = .021) and greater than 10 cm (P = .047) had decreased overall survival. Older age, female sex, epithelioid phenotype, and radiotherapy or chemotherapy administration were not significantly associated with an adverse outcome.

Adult↗

PubMed2000

Paraganglioma of the urinary bladder: can biologic potential be predicted?

BACKGROUND: Paraganglioma of the urinary bladder is rarely encountered and its biologic behavior is uncertain. The authors sought to determine the prognostic factors that would predict patient outcome. METHODS: The Mayo Clinic experience over 53 years with paraganglioma of the bladder was reviewed. All histologic slides from 16 patients were reviewed by the authors. Eight cases were examined immunohistochemically with cytokeratin (AE1/3, cytokeratin 7, and cytokeratin 20), vimentin, S-100 protein, neuroendocrine markers (chromogranin, synaptophysin, and neuron specific enolase), p53 protein, and MIB-1. DNA ploidy was determined by digital image analysis in formalin fixed, paraffin embedded tissue. The mean follow-up was 6.3 years (range, 0.4-16.4 years). RESULTS: Paraganglioma usually occurred in young adult women (mean age, 45 years; range, 16-74 years). The male-to-female ratio was 1 to 3. The common symptoms and signs were hypertension and hematuria. The tumors were usually located intramurally in the lateral and posterior wall of the bladder and were multifocal in 3 cases (18%). Seven patients were treated by transurethral resection, eight by partial cystectomy, and one by radical cystectomy. T classification was T1 (1 patient), T2 (9 patients), T3 (2 patients), and T4b (4 patients). At the time of diagnosis, one patient had distant metastasis and one had regional lymph node metastasis. One patient developed metastasis 1 year after diagnosis and died of the disease 1.5 years later. None of the patients with T1 or T2 tumors had recurrence or tumor progression. All tumors were aneuploid. The mean MIB-1 labeling index was 1.5% (range, 0.03-7.0%). The tumor cells displayed immunoreactivity for S-100 protein and neuroendocrine markers and were negative for p53 (except 1 case) and cytokeratin. CONCLUSIONS: Paraganglioma of the urinary bladder occurs mostly in young adult women. Patients with tumor of advanced classification (>/=T3) are at risk of recurrence, metastasis, and dying of the disease, whereas patients in this study with T1 or T2 disease had favorable outcomes after complete tumor resection.

Adolescent↗

PubMed2000

Surgical management of desmoid tumors of the female pelvis.

BACKGROUND: Desmoid tumors are neoplasms characterized by proliferation of fibroblasts without cytologic features of malignancy. Although desmoid tumors are not uncommon, pelvic desmoid tumors are rare and therapeutically challenging. We describe the surgical management of seven patients with histologically confirmed desmoid tumors arising in the pelvis. STUDY DESIGN: All patients, six with primary and one with recurrent desmoid tumors of the female pelvis, were treated at Mayo Clinic Rochester between 1976 and 1997. The mean age of one African-American and six Caucasian patients was 29.8 years (range 17 to 59 years). No history of previous relevant pelvic trauma was identified; six patients experienced pelvic or leg pain (or both). All patients presented with isolated masses; five of seven were located left of the midline, generally involving the obturator internis or levator muscles (or both). The surgical approach was transabdominal, with a vaginal component in one patient. In addition to this retrospective review of these seven patients, literature addressing the management of desmoid tumors of the female pelvis is reviewed. RESULTS: Extirpative surgery necessitated resection of musculoskeletal, vascular, or adjacent soft tissue structures. Mean tumor diameter was 13.9cm (range 5 to 27 cm). Although the median recorded blood loss was 1,425mL, one patient who refused blood products died secondary to surgical hemorrhage. The median followup for the remaining evaluable patients was 57 months (range 13 to 249 months). Recurrent disease developed in 3 of 6 patients at 3, 11, and 13 months after surgical resection and was successfully managed with subsequent reoperations, with or without adjuvant radiotherapy. CONCLUSIONS: Although recent reports demonstrate selective regression of desmoid tumors with antiestrogen therapy, management of symptomatic pelvic desmoid tumors in young women may necessitate an operation. In our series, all primary and recurrent tumors were managed with resection of involved bone and other adjacent soft tissues. The role of adjunctive radiotherapy or hormonal therapy remains to be defined.

Adolescent↗

PubMed2000

Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and ploidy analysis of 23 cases.

Twenty-three cases of extraskeletal myxoid chondrosarcoma, evaluated at the Mayo Clinic between 1968 and 1996, were studied for clinicopathologic features, immunohistochemical profile, Ki-67 activity, and ploidy status to identify adverse prognostic factors. Females and males were equally affected, and the median age at diagnosis was 50 years. The tumors were located mainly in the lower extremities (83%), and the median tumor size was 9.5 cm. Sixteen tumors showed low cellularity (70%), and eight tumors had high mitotic activity (more than two per 10 high-power fields). The tumors were immunoreactive for vimentin (89%), synaptophysin (72%), epithelial membrane antigen (28%), and S-100 protein (17%). Nine tumors were diploid, three aneuploid, and one tetraploid. Mean Ki-67 activity was 11% (range, 1 to 45%). The 10-year overall survival rate was 78%. On univariate analysis, tumor size > or = 10 cm, high cellularity, presence of anaplasia or rhabdoid features, mitotic activity more than two per 10 high-power fields, Ki-67 > or = 10%, and Ki-67 "hot spot" > or = 25% were associated with decreased metastasis-free or overall survival. Ploidy status was not associated with any adverse outcome. The presence of any of these adverse prognostic factors can indicate the possibility of a more aggressive behavior in extraskeletal myxoid chondrosarcoma, and a closer follow-up is suggested.

Adult↗