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Diagnosis of thymolipoma with fine needle aspiration biopsy. Report of a case initially misdiagnosed as liposarcoma.

BACKGROUND: Thymolipomas are the most outstanding mesenchymal mediastinal tumors although they represent only a small percentage of thymic neoplasms. Their histogenesis remains unsolved, and they are usually benign. Fine needle aspiration biopsy (FNAB) has become the method of choice for the study of mediastinal tumors, including thymolipomas. Making the correct diagnosis depends on the availability of all thymic and lipomatous components as well as on the correct application of a differential diagnosis with other lipomatous tumors. CASE: A 24-year-old woman had a 3-month history of coughing with sputum, fever, asthenia, adynamia, headaches and anemia. Physical examination revealed a bilateral pulmonary condensation syndrome. Imaging studies showed a well-defined, large mass occupying both hemithoraxes and the mediastinum. FNAB revealed an admixture of atypical adipocytes, nonneoplasic lymphoid tissue and capillaries with prominent endothelium. An initial diagnosis of well-differentiated liposarcoma was made. A 2.8-kg, encapsulated, yellowish gray mediastinal tumor was surgically removed. Mature adipose tissue intermingled with abundant thymic tissue containing numerous Hassall's corpuscles were seen histologically. Immunohistochemical markers for thymic epithelium, lymphoid tissue and adipocytes were all positive. Thymolipoma was the final diagnosis. The anemia subsided with resection of the tumor, and the patient was disease free 2.5 years after surgery. CONCLUSION: To the best of our knowledge, this is the second report of a thymolipoma studied with FNAB. The criteria for the differential diagnosis between thymolipomas and other lipomatous tumours should be kept in mind.

Adult↗

Liposarcoma of the maxilla.

A clinically appearing, well-encapsulated lipoma was locally excised from the right posterior upper buccal gingival sulcus of a 24-year-old man and diagnosed histologically as pleomorphic liposarcoma. A right maxillectomy with preservation of orbital function was followed by 5,000 rads of planned postoperative radiation therapy. Four months later the tumor recurred outside of the original site, which has been controlled by further radiation and chemotherapy to these areas.

Adult↗

Liposarcoma of the thyroid gland.

The presumed first case of primary liposarcoma of the thyroid gland is presented, and the possible origin of this rare tumor from either mature fat cells or primitive mesenchymal cells is discussed.

Aged↗

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↗

Clinicopathologic prognostic factors in myxoid liposarcoma: a retrospective study of 49 patients with long-term follow-up.

BACKGROUND: The main goal of this retrospective study was to investigate prognostic factors influencing the survival of myxoid liposarcoma (MLS) with emphasis on the role of transitional areas (TLS) and round cell morphology (RCLS). METHODS: From 1977 to 2004, 49 patients-28 men (57%) and 21 women (43%) with a median age of 44 years (range, 7-83 years)-were diagnosed with an MLS. In 42 patients, the histology could be reviewed, and tumors were classified as MLS, TLS, or RCLS. Clinicopathologic factors were analyzed for influence on survival by univariate and multivariate methods. RESULTS: The median follow-up of 49 patients was 101 months (range, 4-550 months). Of the 42 patients for whom histology was reviewed, 16 tumors were classified as MLS (38%), 19 as TLS (45%), and 7 as RCLS (17%). Sixteen patients (33%) developed a local recurrence after a median follow-up of 21 months (range, 2-108 months). Thirteen patients (27%) developed metastases. The median interval between diagnosis and metastasis was 41 months (range, 0-222 months). Median survival after metastasis was 18 months (range, 1-179 months). The 5- and 10-year disease-specific survival rates were 85% and 72%, whereas the 5- and 10-year overall survival rates were 83% and 68%, respectively. Age at presentation (P = .02), tumor grade (P = .01), and tumor size (P = .005) were significant prognostic factors associated with survival. Tumor grade was the only independent prognostic variable that remained significant with multivariate analysis. A TLS presentation had no negative influence on patient survival. CONCLUSIONS: Age at presentation, tumor grade, and tumor size had a negative influence on survival by univariate analysis, whereas tumor grade was the only independent prognostic factor by multivariate analysis. TLS was not associated with poor outcome.

Adolescent↗

Radiological findings in myxoid liposarcoma of the anterior mediastinum.

CT and MR findings of a rare myxoid liposarcoma involving the anterior mediastinum are reported. The mass was a low density lesion with calcific septations and some peripheral frond-like enhancement on CT. MRI showed heterogeneous intermediate to high signal intensity on T1 weighted images and high signal intensity on T2 weighted images. The signal of the mass was not suppressed on fat suppressed images.

Adult↗

Pulmonary metastasis from liposarcoma: a clinicopathologic and immunohistochemical study of 24 cases.

A review of the histologic features of pulmonary metastasis and clinical implications of liposarcoma (LS) is given for 24 cases (8 each) of the 3 types of LS: myxoid LS (ML), pleomorphic LS (PL), and dedifferentiated LS (DDL). Most patients were men. Metastatic ML and PL were distributed almost equally among the lung lobes, whereas DDL was more common in the left lower lobe. The metastatic MLs had variable cellularity ranging from singly scattered cells in a hyalinized stroma (treatment-related effect) to hypercellular ML. Most PLs (6/8) were nonlipogenic and resembled an undifferentiated pleomorphic sarcoma. All metastatic DDLs had high-grade histologic features and were predictably nonlipogenic. After pulmonary metastasectomy, 2 patients with ML and 1 with PL were disease-free. The other 6 patients with ML, 7 with PL, and all with DDL had progressive disease. The morphologic features of LS metastatic to the lungs seem diverse but within the spectrum of the histologic type expected from the primary tumor. Overall, the general trend for these LS subsets is progressive disease, metastatic disease for ML and PL with a much shorter interval for PL, and metastatic disease and local recurrence for DDL.

Adolescent↗

Blockade of interleukin-6 (IL-6) signaling in dedifferentiated liposarcoma (DDLPS) decreases mouse double minute 2 (MDM2) oncogenicity via alternative splicing.

Effective therapies for retroperitoneal (RP) dedifferentiated liposarcoma (DDLPS) remain unavailable. Loco-regional recurrence occurs in >80% of cases; 5-year disease-specific survival is only 20%. DDLPS is especially prevalent in the retroperitoneum and abdomen; evaluation of the DDLPS microenvironment in these high-fat compartments appears pertinent. Adipose is a main supplier of interleukin-6 (IL6); excessive activation of IL6 signal transducer glycoprotein 130 (GP130) underlies the development of some diseases. The role of GP130 pathway activation remains unstudied in DDLPS, so we examined the role of microenvironment fat cell activation of the IL6/GP130 signaling cascade in DDLPS. All DDLPS tumors and cell lines studied expressed elevated levels of the GP130-encoding gene IL6ST and GP130 protein compared to normal tissue and cell line controls. IL6 increased DDLPS cell growth and migration, possibly through increased signal transducer and activator of transcription 1 (STAT1) and 3 (STAT3) activation, and upregulated mouse double minute 2 (MDM2). GP130 loss conveyed opposite effects; pharmacological blockade of GP130 by SC144 produced the MDM2 splice variant MDM2-ALT1, known to inhibit full length MDM2 (MDM2-FL). Although genomic MDM2 amplification is pathognomonic for DDLPS, mechanisms driving MDM2 expression, regulation, and function beyond the MDM2:p53 negative feedback loop are poorly understood. Our findings suggest a novel preadipocyte DDLPS-promoting role due to IL6 release, via upregulation of DDLPS MDM2 expression. Pharmacological GP130 blockade reduced the IL6-induced increase in DDLPS MDM2 mRNA and protein levels, possibly through enhanced expression of MDM2-ALT1, a possibly targetable pathway with potential as future DDLPS patient therapy.

Proto-Oncogene Proteins c-mdm2↗

Botryomycosis mimicking a liposarcoma.

Botryomycosis is an atypical reaction of the host to a common bacterial infection. A case of botryomycosis mimicking a liposarcoma as suspected on clinical and radiological grounds is described. The diagnosis was made by biopsy and culture of the lesion. A review of the literature is given.

Abdomen↗

Liposarcoma of the spermatic cord. Case report.

We report a case of a 78-year-old man with liposarcoma of the spermatic cord. This rare tumour, which is slow growing and tends to recur locally, must be considered in the differential diagnosis of solid tumours between the testis and the internal ring. The surgical treatment is radical orchiectomy with wide local excision and high ligation of the spermatic cord. Retroperitoneal lymph node dissection is debatable and the value of chemo- and radiotherapy is uncertain.

Aged↗

A 6 kg retroperitoneal liposarcoma in a 42-year-old woman. Case report.

A large retroperitoneal liposarcoma (6 kg) is described in a 42-year-old woman. Retroperitoneal tumours are rare, produce nonspecific symptoms, and are often extensive when diagnosed. The main treatment of these tumours is radical excision, but the tendency to local recurrence makes the prognosis poor.

Adult↗

Maximal displacement of the ureter by a liposarcoma.

We describe a patient with displacement of both ureters to the right side caused by a liposarcoma. The left ureter was displaced beyond the right ureter. The risk of intraoperative trauma to the ureter could be minimized by preoperative placement of ureteral stents.

Humans↗

Liposarcoma of the spermatic cord.

In a 57-year-old man, a suspected incarcerated left inguinal hernia proved to be multifocal dedifferentiated liposarcoma of the spermatic cord. Radical orchiectomy and wide excision were performed, but marginal biopsies were positive for tumour. Radiotherapy was given.

Genital Neoplasms, Male↗

Primary liposarcoma of bone.

A case is presented of a rare primary liposarcoma of bone localized to the major trochanter of the left femur of a 52-year-old female. Despite combined treatment with curettage and irradiation with a total dose of 4,500 rad the neoplasm showed rapid invasive growth with destruction of the bone, spread to the iliac fossa and outgrowth through the operation wound. Approximately 5 months after admission the patient succumbed due to widespread metastases in the lungs, liver and left kidney. The histopathology, clinical course and treatment of this rare neoplasm of the bone are discussed.

Biopsy↗

Primary liposarcoma of gallbladder diagnosed by preoperative imagings: a case report and review of literature.

A 49-year-old Japanese woman was referred to our department because of high fever and a huge abdominal mass. Computed tomography (CT) and magnetic resonance (MR) imagings revealed a tumor, about 30 cm in diameter,occupied the right hepatic lobe and the peritoneal cavity. Abdominal angiography showed that the tumor was fed mainly by the cystic artery. We preoperatively diagnosed angiosarcoma of the gallbladder and performed tumor resection with cholecystectomy because the tumor was almost capsulated,however the posterior wall of the gallbladder attached to the tumor firmly. Histologically,the tumor was composed of spindle cells including lipoblasts with cellular pleomorphism, which were also detected in the muscular layer of the gallbladder. We finally diagnosed pleomorphic liposarcoma of the gallbladder. At 10 mo and 29 mo after the first operation, she underwent two more operations because of recurrence. Now she has a good quality of life 3 yr and 6 mo after the first operation.

Angiography↗