Liposarcoma in the epidural space

STUDY DESIGN: A patient with myxoid liposarcoma in the lumbar epidural space is reported. OBJECTIVE: The subject was treated with marginal resection and posterior instrumentation. SUMMARY OF THE BACKGROUND DATA: Liposarcoma is a malignant tumor of the soft tissues. It is commonly seen in the thigh. Lumbar extradural space is an unusual localization. Two cases have been reported with this localization in the literature. METHODS: The authors have treated a female patient with myxoid liposarcoma in lumbar extradural space with marginal resection and posterior instrumentation. RESULTS: The histopathologic examination showed myxoid liposarcoma. Two cases have been reported in the literature. CONCLUSION: Myxoid liposarcoma is a malignant tumor of the soft tissues. The extradural tumor was probably originated from the epidural fat tissue. Although wide resection is advised in the thigh localization, extradural localization of the tumor can be treated with marginal resection if there is no invasion to the surrounding tissue.     

Limb salvage operation using intraoperative extracorporeal autogenous irradiated bone and tendon graft for myxoid liposarcoma on dorsum of foot

Foot and ankle sarcomas are conventionally treated with amputation. In this article, we report a case of myxoid liposarcoma on the dorsum of the foot treated by limb salvage operation with intraoperative extracorporeal autogenous irradiated bone and tendon grafts. The patient was a 76-year-old woman with a soft tissue tumor beneath the extensor tendons with attachment to the tarsal and metatarsal bones. The histological diagnosis was myxoid liposarcoma. Wide margin was achieved by splitting the tarsal and metatarsal bones into dorsal and plantar parts. The dorsal part of the bones and tendons was isolated from the resected material, irradiated ex-vivo and re-implanted into the host's bones and tendons. No local recurrence was detected around the irradiated bones and tendons during the follow-up at 36 months. The functional rating was 74% according to the ISOLS rating system. This method may be a better procedure for limb salvage operation of sarcomas on the dorsum of the foot.     

Feasibility of whole-body MRI for detecting metastatic myxoid liposarcoma: a case series

No feasible method currently exists to evaluate systemic metastasis in patients with myxoid liposarcoma. The purpose of this study was to determine the feasibility of performing whole-body magnetic resonance imaging (MRI) to detect metastatic myxoid liposarcoma. From June 2008 to May 2010, all patients who were newly diagnosed with myxoid liposarcomas at our institution underwent whole-body MRI along with other conventional imaging methods. We divided the whole body into 38 sections (7 soft tissue sections and 31 bone tissue sections). In total, there were 570 regions (105 soft tissue regions and 465 bony regions) in 15 patients (10 men and 5 women) who underwent whole-body MRI.Of 105 soft tissue regions, there were 4 true positives, 3 false positives, 1 false negative, and 97 true positives. Of 465 bone tissue regions, there were 11 true positives, 5 false positives, 2 false negatives, and 447 true negatives. In soft tissue, whole-body MRI for the detection of metastatic lesion showed a sensitivity of 80%, a specificity of 97.0%, a positive predictive value of 57.1%, and a negative predictive value of 99.0%. In bone tissue, whole-body MRI had a sensitivity of 84.6%, a specificity of 98.9%, a positive predictive value of 68.8%, and a negative predictive value of 99.6%.Whole-body MRI is feasible and effective for detecting bone and soft tissue metastasis in patients with myxoid liposarcoma.     

Atypical and malignant neoplasms showing lipomatous differentiation. A study of 111 cases

One-hundred-eleven cases of histopathologically atypical or malignant lipomatous lesions in the somatic soft tissue and retroperitoneum were studied. These consisted of 48 differentiated fatty neoplasms of the somatic soft tissues (DFT-S), 21 fatty neoplasms of the retroperitoneum (DFT-R), 33 myxoid liposarcomas from various sites and nine pleomorphic liposarcomas. DFT-S were defined as lipomatous lesions composed of mature fat and containing atypical stromal cells or lipoblasts. In the somatic soft tissues, this group included lesions that would be classified using published criteria as "atypical lipoma", "pleomorphic lipoma", "well-differentiated lipoma-like liposarcoma", and "sclerosing liposarcoma". All of the DFT-R met previously published criteria for "well differentiated liposarcoma" or "sclerosing liposarcoma". We found no consistent histologic differences between the DFT-S and DRT-R. No pure "round cell" liposarcomas were encountered although many myxoid liposarcomas had "round cell" areas. Follow-up data were available in 80 cases (72%) with a mean follow-up period of over 7 years. Among the DFT-S there were no uncontrollable recurrences, distant metastases, or tumor-related deaths. The depth of the neoplasm correlated with the tendency for local recurrence; no neoplasms primary in the subcutis recurred; 29% of the tumors recurred when they originated in the deep soft tissues or within the muscle. None of the recurrent tumors demonstrated "dedifferentiation." DFT-R had a recurrence rate of 67% and, although there were no distant metastases, nine patients (43%) died of tumor. Five retroperitoneal tumors dedifferentiated but did not metastasize. In light of this experience, we believe that the term "atypical lipoma" is warranted for the DFT-S and "well differentiated liposarcoma" is an appropriate label for the DFT-R. The overall mean survival for the 52 cases of liposarcoma (excluding DFT-S) was 13.6 years. The mean survival in "well differentiated liposarcoma" (11.25 years) was between that for myxoid liposarcoma (16.25 years) and that for pleomorphic liposarcoma (7 years). Six patients (29%) with myxoid liposarcoma developed local recurrences and 6 patients (29%) developed distant metastases and died. Metastasis was always associated with a round cell (or pleomorphic) component with increased numbers of mitotic figures in either the primary tumor or a local recurrence.     

The utility of fluorescence in situ hybridization (FISH) in the diagnosis of myxoid soft tissue neoplasms

Diagnosing myxoid soft tissue neoplasms can be challenging because of overlapping histologic features. Distinct chromosomal translocations have been identified in several myxoid sarcomas, including t(12;16)(q13;p11) FUS-DDIT3 in myxoid liposarcoma, t(7;16)(q34;p11) FUS-CREB3L2 in low-grade fibromyxoid sarcoma, and t(9;22)(q31;q12) EWSR1-NR4A3 in extraskeletal myxoid chondrosarcoma. These recurrent chromosomal alterations are attractive targets for diagnostic studies. To that end, dual-color, break-apart fluorescence in situ hybridization (FISH) probes spanning the genomic regions of EWSR1 (22q12), DDIT3 (12q13), and FUS (16p11) (Vysis, Downer's Grove, IL) were evaluated in formalin-fixed, paraffin-embedded tissues from myxoid neoplasms, including intramuscular myxoma (n=10), myxoid liposarcoma (n=18), low-grade fibromyxoid sarcoma (n=10), extraskeletal myxoid chondrosarcoma (n=13), and myxofibrosarcoma (n=8). Of the myxoid liposarcomas, 18/18 cases had a rearrangement of the DDIT3 gene, with 17/18 (94.4%) showing both DDIT3 and FUS gene rearrangements. A FUS gene rearrangement was identified in 7/10 (70%) of low-grade fibromyxoid sarcomas, with no changes involving EWSR1 or DDIT3. An EWSR1 translocation was seen in 6/13 (46.2%) of extraskeletal myxoid chondrosarcomas, without changes in DDIT3 or FUS genes. The remaining neoplasms studied showed no rearrangements involving DDIT3, FUS, or EWSR1 genes. In conclusion, interphase FISH using DDIT3 and FUS probes identifies the characteristic translocation in myxoid liposarcoma. FUS and EWSR1 probes are useful in confirming the diagnosis of low-grade fibromyxoid sarcoma and extraskeletal myxoid chondrosarcoma, respectively. The specificity of the probes is documented as none of the non-translocation-associated myxoid tumors showed genomic abnormalities with the probes tested. FISH is capable of providing specific ancillary information useful in this often difficult differential diagnosis.     

Intussusception secondary to metastasis from a low-grade retroperitoneal liposarcoma

Liposarcomas are the most prevalent soft tissue sarcomas in adults. Low-grade liposarcomas are the most frequent and least aggressive and are noted as having a low risk of metastasis. We present a case of low-grade myxoid liposarcoma that metastasized to the small bowel resulting in intussusception. This case involves a 44-year-old woman with a recurrent retroperitoneal liposarcoma. Approximately 30 months after initial diagnosis, the patient was found to have a solitary metastasis to the small bowel that presented as a jejunal intussusception. Interestingly, there was no indication of a round cell component in either the metastasis or recurrent tumor, although the original tumor did contain a small round cell component. This is the first report in the English literature of liposarcoma metastasizing to the small bowel. We suggest that atypical presentations of liposarcoma metastases should always be considered in the management of patients with a history of liposarcoma.     

Liposarcoma with radiologically biphasic patterns

Liposarcoma is a one of the common soft tissue sarcomas, and is estimated to represent 16–18% of sarcomas in adults. According to the World Health Organization, this tumor is mainly classified into five subtypes: well-differentiated, dedifferentiated, myxoid/round, pleomorphic and mixed-type. Previous papers have reported that radiological information for liposarcoma is correlated with the radiological and histopathological findings. In liposarcoma, when biphasic patterns composed of a lipogenic tumor with a sarcomatous appearance are found on radiological examination of computed tomography and/or magnetic resonance imaging, dedifferentiated liposarcoma, defined as a “malignant adipocytic neoplasm showing transition from atypical lipomatous tumor/well-differentiated liposarcoma to non-lipogenic sarcoma of variable histological grade”, is usually considered.     

脂肪肉瘤的病理类型与临床疗效

为探讨脂肪肉瘤的病理类型与临床疗效的关系，作者分析了１９７６年１０月～１９９５年１２月手术治疗的２１例脂肪肉瘤病例。男１５例，女６例，年龄６～７９岁，平均４９．９岁。其中分化型１３例、粘液型４例、去分化型４例。平均随访８．９年。５年、１０年生存率均为９４％，５年、１０年的未复发率分别是７２％和５２％。作者认为，脂肪肉瘤作为软组织恶性肿瘤中常见的一种，生存率与肿瘤的病理类型、部位、大小有关；发生在四肢的分化型、粘液型治疗效果较好，发生在后腹膜的治疗效果差，肿瘤小治疗效果好；广泛切除术是治疗脂肪肉瘤的有效方法。     

Well-Differentiated Liposarcoma of the Hypopharynx Exhibiting Myxoid Liposarcoma-like Morphology with MDM2 and DDIT3 Co-Amplification

Well-differentiated liposarcoma (WDL) is one of the most common soft tissue sarcomas in adults. It has a predilection for middle-aged males and arises in deep-seated locations such as retroperitoneum, mediastinum, and spermatic cord. Its occurrence in young individuals at the hypopharyngeal region is an exceedingly rare event. Myxoid liposarcoma (ML)-like changes can seldom occur in some cases of WDL, which makes the diagnosis of WDL more challenging. Amplification of DDIT3 gene in a subset of cases of WDL has shown to be associated with such unique morphology. Herein, we present a case of a 36-year-old gentleman who presented with difficulty in breathing and swallowing for 3 months duration. CT scan of the neck revealed a lesion along the posterior wall of the hypopharynx measuring 3.5 cm. Histopathologic examination revealed a tumor composed of lobules of oval to spindle cells in a prominent myxoid stroma with delicate chicken-wire vasculature. In the vicinity, there were lobules composed of variably sized adipocytes separated by thick fibrous septa that contains atypical hyperchromatic spindle cells. By immunohistochemistry, the tumor cells in both components were immunoreactive for CDK4, but negative for MDM2. Fluorescence in-situ hybridization (FISH) confirmed the presence of MDM2 gene amplification. There was no evidence of FUS-DDIT3 gene rearrangement, however, DDIT3 gene was also amplified. The diagnosis of well-differentiated liposarcoma with prominent myxoid stroma was rendered. This is the first documentation of WDL with ML-like morphology harboring co-amplification of MDM2 and DDIT3 in the hypopharynx.     

Retroperitoneal well-differentiated liposarcoma presenting as an incarcerated inguinal hernia

A 69-year-old man was admitted with a complaint of left irreducible inguinal mass. On surgical exploration no evidence of hernia was found and the inguinal floor was overwhelmed by a large lobulated mass, arising from the properitoneal fat, that involved the spermatic cord. The mass was partially removed, sparing the elements of cord. The transversalis fascia was repaired by direct suture and a polypropylene mesh was located above. The histopathological diagnosis was well differentiated-type liposarcoma with myxoid features. The liposarcoma is a malignant tumour of the adipose tissue that arises from the primitive mesenchymal cells. These neoplasms have been usually found in the soft tissues of limbs, trunk, mediastinum, retroperitoneum and occasionally in the spermatic cord. The clinical aspect is frequently a complaint of scrotal or inguinal painless mass, mimicking to an inguinal hernia and the diagnosis of tumor is performed mainly during surgery, as in our patient. In the case of a firm not reducible painless inguinal mass without signs and symptoms of bowel obstruction, an abdominal tumor with inguinal or scrotal extension should be suspected and preoperatively excluded. The US and CT scan may be helpful to plane a correct therapeutic strategy before intervention.     

Liposarcomas of the mediastinum and thorax: a clinicopathologic and molecular cytogenetic study of 24 cases, emphasizing unusual and diverse histologic features

Liposarcoma rarely occurs in the mediastinum, and most reports predate the current genetically based classification system. We report the clinicopathologic and molecular genetic features of a series of thoracic liposarcomas identified over a 60-year period. Twenty-four confirmed cases were reclassified using the most recent World Health Organization classification. Fluorescent in situ hybridization for CPM amplification and/or DDIT3 rearrangement was performed on selected cases. The 24 cases occurred in 13 men and 11 women (mean age, 53 y; range, 15 to 73 y) and arose in all mediastinal compartments. All subtypes were encountered with 8 well-differentiated liposarcomas, 6 dedifferentiated liposarcomas (3 of 6 confirmed CPM+), 7 pleomorphic liposarcomas (2 of 7 confirmed CPM-, 1 of 7 confirmed DDIT3-), 2 myxoid liposarcomas, and 1 unclassifiable liposarcoma (CPM- and DDIT3-). Unusual histologic features included myxoid well-differentiated liposarcoma mimicking myxoid liposarcoma (2 cases), lipoleiomyosarcoma (1 case), dedifferentiated liposarcoma with "meningothelial"-like dedifferentiation, differentiated myxoid liposarcoma mimicking well-differentiated liposarcoma (CPM-), and pleomorphic liposarcoma with epithelioid and myxoid change. Follow-up information was available for 19 patients (mean, 55 mo; range, 8 to 252 mo). Outcome was strongly associated with histologic subtype, with death from disease occurring in 1 of 6 well-differentiated, 1 of 4 dedifferentiated, 5 of 7 pleomorphic, and 2 of 2 myxoid liposarcomas. The mediastinum shows a preponderance of uncommon subtypes and unusual morphologic variants. Correct classification has important implications, with most patients with well-differentiated/dedifferentiated liposarcoma having a protracted clinical course, in contrast to the more rapid disease progression seen in patients with myxoid and pleomorphic liposarcoma.     

Liposarcoma of the lower extremity

Twenty patients with primary liposarcoma of the lower extremity were treated over a 25-year period. The adequacy of the initial surgical procedure and histological grade of malignancy both influenced survival rates. In addition local recurrence was noted only in patients who had undergone "inadequate" initial excision. Inguinal lymph nodes were uninvolved by tumor in all cases. In general, patients with liposarcomas of myxoid and/or round cell type survived for long periods of time. However, even patients with myxoid lesions occasionally exhibited evidence of early blood-borne metastases. This study suggests an important relationship between an inadequate primary resection, local recurrence, and eventual retroperitoneal spread. In the majority of cases, this may have resulted from failure to control the primary distal extremity tumor, with subsequent contiguous spread of metastases into the ipsilateral retroperitoneal space. If feasible, radical soft part resection should be performed as the primary surgical therapy of these neoplasms. Tumors contiguous to the knee or ankle joint should be treated by primary amputation. Failure to control local disease may result in blood-borne dissemination or local spread along musculoaponeurotic planes to involve proximal groin or retroperitoneal space.     

Angiomyxolipoma of the right sub-brow: Case report with review of the literature

IntroductionAngiomyxolipoma (AML) is a rare variant of benign lipoma with characteristic histopathological and immuno-histochemical features. It consists of fatty tissue admixed with myxoid stroma and blood vessels. It was first described by Mai et al. in 1996 [1], with a total number of 19 cases reported since.PresentationThis is the first report of an AML in subcutaneous tissue of the face, presenting as a 4-month old cystic lesion in a 78-year old lady. Diagnosis was based on radiological and histopathological with cytochemical findings.DiscussionIt is important to distinguish this lesion as distinct from malignant subcutaneous lesions of fatty tissue, especially with short history as seen in our case.ConclusionPrecise diagnosis of angiomyxolipoma is important to avoid unnecessary investigations, stress and misdiagnosis of myxoid liposarcoma.     

Pleomorphic spermatic cord liposarcoma: A case report and review of management

Liposarcoma of the spermatic cord is very rare, representing about 7% of para testicular sarcomas. It is considered to be one of the highest malignancy grades.We present a case of a liposarcoma of the spermatic cord in a 45-year-old male complaining of a progressive painless swelling in the right inguinoscrotal region. Ultrasonography and computed tomography findings were compatible with liposarcoma of the spermatic cord. We performed a right radical orchiectomy with a wide resection of the mass. Histological examination confirmed the diagnosis and showed a pleomorphic subtype.The mainstay of management of spermatic cord liposarcoma is wide excision with radical orchiectomy. The most important factors for prognosis are the histologic subtype and surgical margin status. Adjuvant radiotherapy should be considered in cases at high risk for local recurrence. Long-term surveillance is mandatory.Liposarcoma of the spermatic cord is an uncommon para testicular tumor which should be part of the differential diagnosis of inguinoscrotal mass. A radical inguinal orchiectomy with wide resection of the soft tissue mass and the spermatic cord are the key to longest local and systemic disease-free survival.     

Treatment of myxoid liposarcoma in pregnancy

Two cases of pregnancy associated with myxoid liposarcoma are presented. Both patients were treated with hyperthermoradiotherapy after the induced delivery of healthy infants and by surgical excision of the tumor 5 to 7 weeks after delivery. No local recurrence or distant metastasis occurred; the children have progressed normally for 4 and 5 years, respectively, after surgery. Because coexistence of pregnancy and sarcoma is rare, it is difficult for the clinician to develop an appropriate approach to the management of bone and soft tissue sarcoma during pregnancy. The literature was reviewed regarding the effect that pregnancy may have on the clinical behavior of sarcoma and the effect treatment for sarcoma may have on the fetus.     

Liposarcoma associated with multiple intramuscular lipomas. A case report

A 71-year-old slender, previously healthy man was admitted to the authors' institution because of a huge painless mass in his left scapular area. Physical examination revealed a second soft tissue mass in his right scapular region and a third soft tissue mass in the anterior side of his right shoulder. Surgical treatment including marginal resection of the second and third small masses followed by wide local resection of the huge tumor was performed. Histologic examination showed that the first mass was a well differentiated lipomalike liposarcoma, whereas the second and third lesions were identified as intramuscular lipomas. A review of the literature showed two cases of retroperitoneal liposarcoma associated with multiple subcutaneous lipomas and two cases of liposarcoma involving an extremity associated with multiple subcutaneous lipomas. There is no previous report in which intramuscular liposarcoma was associated with multiple intramuscular lipomas.     

A rare case of myxoid pleomorphic liposarcoma in an infant: A report

Introduction and importanceMPL (myxoid pleomorphic liposarcoma) is an uncommon type of liposarcoma that affects mostly children and infants. Its aggressive behavior and tendency to recur warrant complete excision despite the challenges of troublesome locations.Case presentationA 12-month-old infant presented with an insidious onset of noisy breathing and respiratory distress not relieved by supplemental oxygen via face mask. Examination revealed dullness and decreased air entry on the left chest. Computed Tomographic (CT) scan showed a large solid mass occupying the left hemithorax and displacing the mediastinum to the right. Intraoperatively, a large solid mass arising from the left chest wall and attached to the fifth rib was seen. Histopathology of the resected mass showed myxoid pleomorphic liposarcoma which is non-reactive for MDM2 immunostain.Clinical discussionUnlike other liposarcomas, myxoid pleomorphic liposarcoma occurs in children, commonly in the chest. CT scan is the preferred imaging modality. Treatment is by complete excision where possible. Molecular studies like Fluorescent in-situ Hybridization (FISH) and Immunohistochemistry (IHC) is used for confirmation. It has a high propensity to metastasize and recurrence is expected. Chemotherapy and irradiation following complete resection decrease the disease recurrence.ConclusionSoft tissue malignancy must be considered in the differential diagnosis of a large intrathoracic tumor in an infant. FISH and IHC are essential for confirmation.