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Lipoblastic nerve sheath tumors: report of a distinctive variant of neural soft tissue neoplasm with adipocytic differentiation.

Benign nerve sheath tumors of soft tissue can occasionally adopt unusual or unfamiliar morphologic appearances that may introduce difficulties for diagnosis, such as multinucleation, bizarre nuclei, intranuclear vacuoles, and other degenerative changes. Tumor cells adopting a signet-ring or lipoblast-like configuration, however, are mostly associated with epithelial malignancies, liposarcoma and melanoma, and have been only rarely observed in spindle cell tumors of soft tissue. We report 5 cases of benign nerve sheath neoplasms that displayed prominent signet-ring cells with lipoblast-like features. The cases presented as solitary soft tissue masses in the groin, thigh, retroperitoneum, and shoulder in 4 men and 1 woman between the ages of 31 to 57 years. Four tumors predominantly showed features of schwannoma and one of neurofibroma; however, intimately admixed with the spindle cell population, there were also numerous scattered mature adipocytes as well as lipoblast-like cells displaying a signet-ring cell appearance. Immunohistochemical studies showed strong S-100 protein positivity in the spindle cells as well as in the signet-ring lipoblast-like cells and the mature adipocytes. The signet-ring cells were negative for mucin stains, cytokeratin, EMA, CEA, and several other differentiation markers. Ultrastructural examination was performed in 2 cases. The signet-ring cells contained large cytoplasmic lipid droplets that displaced the nuclei to the periphery, consistent with lipoblastic differentiation, whereas complex, interdigitating cytoplasmic processes covered by basal lamina material characteristic of nerve sheath differentiation could be identified in the spindle cells. Four patients for whom follow-up was available were alive and well with no evidence of recurrence over a period of 28 to 116 months (median follow-up, 50 months). The presence of mature fat and signet-ring lipoblast-like cells within a nerve sheath neoplasm is quite rare and may signify a process of aberrant differentiation. Neurogenic tumors should be added in the differential diagnosis of spindle cell tumors capable of displaying prominent signet-ring cell features.

Adenocarcinoma↗

Sonographically guided core needle biopsy of soft tissue neoplasms.

PURPOSE: This study was conducted to evaluate the usefulness of sonography (US)-guided needle biopsy in the diagnosis of soft tissue masses. PATIENTS AND METHODS: Thirty-seven patients with a mean age of 49 years were enrolled in the study. The size of the biopsy needle was selected according to the kind of tumor suspected (ie, primary or metastatic). In patients with suspected metastases, smaller biopsy needles were used; in patients with suspected primary tumors, larger needles were used so that larger specimens could be obtained. Prebiopsy color Doppler sonography (CDUS) was routinely used to guide the cutting needle to areas of the lesion showing sufficient vascularity. From 3 to 6 cores were obtained, depending on their quality. We compared the diagnoses yielded by the core biopsy and the final histopathologic analysis of the resected tumor by classifying the results as "concordant" or "discordant." RESULTS: A total of 37 tumors were examined. Final diagnoses were 24 malignant tumors (6 metastases and 18 primary tumors) and 13 benign tumors. The lesions were diagnosed correctly as either benign or malignant in 35 of the 36 cases for which needle biopsy specimens were adequate, with only 1 misdiagnosis. The diagnoses were concordant in 33 cases (17 primary malignant tumors, 6 metastatic tumors, and 10 benign tumors) and were discordant diagnosis in the other 4 cases (1 primary malignant tumor and 3 benign tumors). No complications were attributable to the needle biopsy. CONCLUSIONS: US-guided percutaneous core needle biopsy of soft tissue neoplasms is an easy, safe, and useful procedure. It can be considered a first-line procedure for the acquisition of tissue specimens adequate for histopathologic diagnosis.

Adolescent↗

Malignant soft tissue neoplasms with the histologic features of renal rhabdoid tumors: an ultrastructural and immunohistochemical study.

Five round cell neoplasms of the soft parts that histologically resembled malignant rhabdoid tumors of the kidney were studied. The tumors were composed mainly of poorly differentiated round or, sometimes, polygonal cells, with a minority of elongated cells; the cytoplasm of many of the cells contained filament-laden acidophilic inclusions. Ultrastructurally, the intracytoplasmic structures were seen to consist of aggregates of 10-nm intermediate filaments, and immunohistochemical staining revealed the presence of cytokeratin and vimentin. All five patients with this tumor had an aggressive clinical course; three of the patients died shortly after the initial diagnosis. As this tumor does not seem to be linked to any known entity, it is referred to as malignant rhabdoid tumor of the soft parts and could be a heterogeneous entity.

Adult↗

Preoperative staging techniques for soft-tissue neoplasms.

Preoperative staging studies need not be limited to one of the techniques discussed since each has its own specific advantages and disadvantages in the overall evaluation of soft-tissue neoplasms. Among orthopaedic oncologists, however, it is apparent that MRI is becoming the preoperative staging study of choice. It is by the careful, judicial use of one or all of these techniques that optimal diagnostic information is obtained and the patient given the best treatment alternatives.

Angiography↗

[Sacral anesthesia in surgical treatment of soft tissues neoplasms of the lower extremities].

The experience with sacral anesthesia in surgical treatment of soft tissues' benign and malignant neoplasms of the lower extremities was analyzed. Benign tumors were the indications for surgical treatment in 19 patients, malignant tumors - in 11 patients. Various methods of sacral anesthesia are described, indications for this type of anesthesia and obtained results are discussed. It is concluded that sacral anesthesia in surgical treatment of soft tissues neoplasms of the lower extremities provides adequate anesthesia even without other types of anesthetic protection.

Anesthesia, Caudal↗

[Treatment of patients with soft tissue neoplasms].

The methods of treatment of 1,410 cases of soft tissue sarcoma were compared. Tumor was less than 5 cm only in 19.4% of patients admitted to the hospital by the beginning of treatment. Radical surgery was performed in 65.3% of cases, it still remaining the major procedure of treatment. Application of combined (surgery + radiation) treatment is increasingly used, every fourth patient (25.1%) being given this therapy. The percentage of radically-treated cases of soft tissue sarcoma may be raised by 15% unless patients refuse to be operated on.

Adolescent↗

Ancient hematoma: a unifying concept for a post-traumatic lesion mimicking an aggressive soft tissue neoplasm.

We describe the clinicopathologic features of four cases of ancient hematoma occurring in the tensor fascia lata and perifascial tissue that clinically and radiologically mimicked aggressive soft tissue neoplasms. The lesions arose in three men and one woman, aged between 31 and 61 years. In three cases, an injury to the affected site had been noted 18 months, 19 years, and 20 years beforehand, respectively. At presentation, these deep-seated and enlarging lesions were highly suggestive of soft tissue sarcomas. Three lesions were treated by excision of an intramuscular or perifascial mass; in one case, repeated incomplete excisions were performed. Grossly, in three cases a well-circumscribed, fusiform mass with a soft cystic center and a fibrous pseudocapsule was described. Histologically, blood clot, fibrin, and predominant amorphous debris were seen in the central cavities; in three cases, the fibrous pseudocapsule contained clumped, eosinophilic, amorphous debris surrounded by a prominent xanthogranulomatous inflammatory infiltrate with numerous, multinucleated, foreign body-type, giant cells. Additionally, cholesterol clefts, areas of hemorrhage and dystrophic calcification, hemosiderin deposits, and cellular scar tissue were seen. Follow-up information revealed no evidence of recurrence or secondary infection in the three cases treated by local or wide excision; in the case in which incomplete excision was performed, continuous symptomatic growth was noted. Pathologic features of ancient hematomas are similar, if not identical, to those hematomas described previously as calcific myonecrosis, chronic expanding hematoma or posttraumatic cyst of soft tissues; we propose instead the term ancient hematoma. The reason for the commonly very long delay between the initiating trauma and presentation is uncertain.

Adult↗

[Magnetic resonance computerized tomography in the diagnosis of soft tissue neoplasms].

Sixty-three patients with malignant and benign tumors of soft tissues were examined making use of magnetic resonance computer-aided tomography (MRT). This method was found effective in the diagnosis of soft tissue tumors, permitting the detection of tumor connection with the adjacent structures. In some cases MRT helped differentiate between malignant and benign soft tissue tumor growth, e.g. liposarcoma, malignant fibrous histiocytoma, lipoma, desmoid, hematoma. MRT may be effectively used in the diagnosis of soft tissue formations.

Adult↗

Cytomorphological study of soft tissue neoplasms: role of fluorescent immunocytochemistry in diagnosis.

OBJECTIVES: Exact categorization of soft tissue tumours (STTs) on smears requires application of various ancillary techniques. This study was aimed at evaluating the role of fluorescent immunocytochemistry (FICC) in cyto-diagnosis of 30 STT cases. METHODS: Thirty cases of soft tissue tumours were included in the present study. All cases were subjected to routine Giemsa and Papanicolaou stain. Extra smears were made and kept for fluorescent immunostaining. A panel of cytoskeletal antibodies, tagged with FITC (Fluorescein isothyocynate), was employed in all these cases. Fluorescent immunostained smears were examined under Zeiss Confocal Laser scanning microscope, using double immunofluorescence (red-green). Finally, all cases were subjected to biopsy and again immunoperoxidase staining. RESULTS: Among the 30 cases in the present study, unaided cytological diagnoses ranged from 'spindle cell' tumour in four (13.3%) cases, benign and malignant spindle cell tumour in 17 (56.6%) cases, to malignant mesenchymal tumour in nine (30%) cases. FICC helped in further correct categorization of 25/30 (83.3%) cases viz. leiomyoma (three), benign neurogenic tumour (six), schwannoma (one), dermatofibrosarcoma protuberans (three), synovial sarcoma (two), rhabdomyosarcoma (two), malignant fibrous histiocytoma (five) and malignant peripheral nerve sheath tumour (three). Aggressive fibromatosis was found to be a missed diagnosis in two cases. Overall concordance between cyto-diagnosis with FICC, and histopathology results was 83.3% (P < 0.05). CONCLUSION: Fluorescent immunocytochemistry is a significant ancillary technique for making a rapid and specific diagnosis of STT, as required for their timely management. Incorporation of a wide panel of antibody markers with clinico-cytological correlation is recommended in forming an exact diagnosis in these cases.

Biopsy, Fine-Needle↗

Divergent differentiation in malignant soft tissue neoplasms: the paradigm of liposarcoma and malignant peripheral nerve sheath tumor.

In tumors clonality does not always translate into morphologic uniformity. While most sarcomas exhibit only one line of histologic differentiation, a minority may display a strikingly diverse phenotype in addition to the main lineage. This phenomenon not only presents a diagnostic problem but also raises questions about the commitment of tumor cells toward a specific phenotype. Among sarcomas, malignant peripheral nerve sheath tumor (MPNST) and dedifferentiated liposarcoma are two entities in which divergent differentiation is a relatively frequent event. Diagnostically it is crucial to recognize the "primary" sarcoma in such tumors correctly and distinguish it from the "secondary" divergent elements. The presence of the latter could be the first morphologic clue to a specific sarcoma type. Even though it may be difficult to explain the pathogenesis of divergent differentiation, divergence still illustrates that the phenotype of a tumor cell is not set in stone but can be modulated or switched by a number of factors.

Cell Transformation, Neoplastic↗

[Soft tissue neoplasms--progress and limits of pathologico-anatomic diagnosis].

Current classifications of soft tissue tumors are based principally on the histogenetic type of the tumor thereby abandoning descriptive terms such as spindle cell sarcoma, round cell sarcoma etc. Insight into the histogenesis of a number of soft tissue tumor types has been gained from electron microscopical studies. In addition, these studies provided valuable data to establish certain neoplasms as tumor entities. Thus, the histogenesis of clear cell sarcoma of tendons and aponeuroses has been traced to melanocytes, and it is now universally accepted that biphasic synovial sarcoma consists of epithelial and fibroblastic components. Despite these advances, about 10% of all soft tissue sarcomas remain unclassified when light- and electron microscopy are used in combination. Further improvement in the classification of soft tissue malignancies has been achieved applying immuno-histological techniques. As in other types of malignant tumors mono- and polyclonal antibodies against intermediate filament proteins are especially useful. Other "markers" include protein S-100, neuronspecific enolase (NSE), epithelial membrane antigen (EMA) and panleucocyte antigen (LCA). Regular use of all these markers enabled us to reduce the percentage of unclassified soft tissue sarcomas from 17.6% in the "pre-immune era" to 6.1%.

Antibodies, Monoclonal↗

Management of vascular soft tissue neoplasms using transcatheter embolization and surgical excision.

Preoperative embolization of highly vascular soft tissue tumors was performed in four patients. Selective catheterization of branch vessels supplying the tumors successfully avoided inadvertent embolization of adjacent normal tissue. Operative blood loss was significantly less than anticipated, permitting an easier, more precise, and quicker operation. Two of the lesions were benign intramuscular hemangiomas; one was a fibrosarcoma, and one, a malignant schwannoma. Three tumors were resected completely. A small portion of one hemangioma could not be removed. All patients were free of recurrence during follow-up periods of 6 to 14 months.

Adolescent↗

Radiation-induced sarcoma of bone: clinical and radiographic findings in 43 patients irradiated for soft tissue neoplasms.

Sarcomas rarely follow irradiation of benign lesions of bone as the dangers of this form of therapy is well recognised. Forty-three patients with soft tissue neoplasms - mainly carcinoma - were irradiated and developed sarcomas in the radiation field. Twenty-four of these patients were seen at this centre in the last decade, a considerable increase compared to earlier years. Radiation osteitis may indicate the nature of the tumour but was only present in 50%. A purely lytic or purely sclerotic pattern was commoner than a mixed lytic and sclerotic appearance. CT scan, bone scan and angiography were helpful in delineating disease but did not assist in diagnosing the type of tumour. A tumour arising within the irradiated field is an indication for biopsy. Prognosis of radiation-induced sarcoma (RIS) remains poor, only five of the 43 patients survived 5 years.

Adolescent↗

[Post-traumatic ossifications simulating soft tissue neoplasms].

Roentgenologic data on 59 patients in whom posttraumatic calcification and ossification simulated soft tissue tumor were evaluated. X-ray diagnostic and differential diagnostic features are discussed. Soft tissue X-ray proved to be best suited in ossification identification.

Adolescent↗

[Potential uses of color Doppler in periskeletal soft tissue neoplasms].

Integrated imaging plays a fundamental role in the study of periskeletal soft tissue tumors, for both diagnosis and treatment planning. The steady and progressive technologic progress of color Doppler US equipment now permits the integration of conventional morphostructural parameters with the biofunctional data of lesion flow patterns and relative qualitative features. To assess color Doppler capabilities in differentiating benign from malignant soft tissue tumors, we reviewed the B-mode and color Doppler findings of 43 consecutive patients with a palpable periskeletal soft tissue mass. All patients were examined with a real time unit (Ultramark 9 HDI), with a broadband (5-10 MHz) linear transducer operating at 6.5 MHz for Doppler measurements. The PRF was set at 1500 to 800 Hz with 70% color gain; a 100 Hz wall filter was used. We kept the color box in the area of interest as small as possible to keep the frame rate high; pulsed Doppler studies were performed with a small sample volume and 2000 Hz PRF. The following signs were considered: morphostructural features; the presence/absence of color signals; the (peripheral/internal) site of vascular branches, their caliber and course; the number of afferent vascular poles; resistance index. As a rule, malignant masses tend to differ from benign masses for the presence of multiple vascular afferent branches, especially if they have an irregular pattern and caliber, and for the variability of the resistance index measured in different parts of the same mass. Further examinations, performed with second level imaging (CT and MRI) and microhistologic tests, respectively after biopsy and surgical resection, confirmed the high predictive value of color Doppler US, with only 1 false negative and 2 false positives; color Doppler sensitivity and specificity were 94.7% and 91.6%, respectively, which are higher values than those obtained with US alone (63% and 66.6%). Therefore, we believe that color Doppler US can be systematically applied to the study of periskeletal soft tissue masses, integrating conventional US for the correct selection of the patients to be submitted to second level investigations.

Adolescent↗