Myxoid Sarcoma Research Articles

Testicular Germ Cell Tumors With Sarcomatous Components

The development of sarcomatous component (SC) in testicular germ cell tumor (GCT) is an uncommon phenomenon. We searched our surgical pathology files from 1985 to 2007 and identified 33 cases of testicular GCTs with SC. The average age of patients was 31 years. All patients underwent radical orchiectomy, which demonstrated a GCT in all patients except for 3 patients who had received neoadjuvant chemotherapy. All testicular GCTs contained a teratomatous component. The GCTs were pure teratomas in 3 cases, and were mixed GCTs in the other cases. The SC was observed in primary testicular tumor (n=19), in metastasis (n=11), or in both primary testicular tumor and metastasis (n=3). The average percentage of the SC in the primary testicular GCT was 32% (range: 5% to 99%). The most common histologic type of SC was rhabdomyosarcoma (n=24), followed by high-grade unclassified sarcoma (n=5), rhabdomyosarcoma admixed with high-grade unclassified sarcoma (n=2), angiosarcoma (n=1), and low-grade myxoid sarcoma (n=1). Clinical follow-up information was available for 27 patients. Of the 13 patients whose SC was limited to the testicular GCT, 2 patients died of GCT not otherwise specified at 37 and 68 months, respectively; and 11 patients were free of disease at a mean of 46 months. Of the 14 patients with a SC in the metastasis, 7 patients died of GCT not otherwise specified at a mean of 95 months, and 7 patients were free of disease at a mean of 104 months. These results suggest that patients with a SC confined to the primary testicular GCT may not have a higher risk of mortality than those at a comparable stage without a SC. However, patients with a SC in the metastasis have an increased risk of mortality.

Treatment Results of Sinonasal Sarcomas: 20 Cases in Asan Medical Center

Background and Objectives Sinonasal sarcomas are relatively rare tumors, accounting for only about 1 percent of all sinonasal malignancies. The purpose of this study is to analyze the clinical features, treatment modalities and outcomes, survival and prognostic factors of sinonasal sarcomas. Subjects and Method We retrospectively reviewed the medical records of 20 patients who were diagnosed and treated for sinonasal sarcomas between July 1993 and June 2006. Potential prognostic factors including age, tumor size, histology and adjuvant treatment were evaluated. Results According to histologic subtype, twenty cases consisted of 7 rhabdomyosarcomas, each 2 cases of spindle cell sarcoma, Ewing’s sarcoma, angiosarcoma, and each 1 case of fibrosarcoma, leiomyosarcoma, hemangiopericytoma, peripheral neuroectodermal tumor, myxoid sarcoma, osteosarcoma, chondrosarcoma. The mean follow-up period was 61 months. Fourteen patients (65%) had undergone surgical approach with adjuvant chemotherapy or radiotherapy. Four patients (20%) had undergone surgical treatment only. Two patients (15%) had undergone chemotherapy or radiotherapy. At 5 years, the overall survival rates of sinonasal sarcomas were 65%. The survival rates according to the treatment modality were 100% (surgery only), 66% (surgery with adjuvant chemotherapy or radiotherapy), and 33% (chemotherapy or radiotherapy), respectively. The overall recurrence rates of the sinonasal sarcomas were 25%. The univariate analysis showed that the treatment modality and surgical margin are important prognostic factors for survival. Conclusion The 5-year survival rate of sinonasal sarcomas was 65%. Early diagnosis and wide excision with safe resection margin are important for the treatment of sinonasal sarcomas.

Extraskeletal myxoid chondrosarcoma: Diagnosis of a rare soft tissue tumor based on fine needle aspiration cytology

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma with limited literature available on its cytological features. We report here one such case where a diagnosis of EMC was made based on fine needle aspiration cytology (FNAC). A 51 year-old male presented to our FNAC clinic with a slowly growing mass in the left thigh, which was subjected to fine needle aspiration biopsy. Radiological images showed no involvement of the underlying bone. Magnetic resonance imaging was suggestive of a malignant neoplasm. The FNA smears showed cell fragments and cords of monotonous cells embedded in abundant myxoid stroma. A diagnosis of a myxoid sarcoma favoring an EMC was made in this patient. Subsequent excision of the mass for histopathological examination confirmed this diagnosis. EMC has distinctive cytological features that are helpful in confidently making a diagnosis in the appropriate clinical setting.

Non-germ cell tumours arising in germ cell tumours (teratoma with malignant transformation) in men: CT and MR findings

Purpose To describe the imaging findings of germ cell tumours (GCT) containing non-germ cell malignant components (also designated teratoma with malignant transformation or TMT). Patients and methods The records of 14 male patients with GCT and a non-germ cell histological component TMT were retrospectively reviewed. All patients had computed tomography (CT) and/or magnetic resonance (MR) studies before and after initial surgery and chemotherapy, as well as during follow-up. Imaging findings were correlated with the response to treatment and with overall survival. Pathological evaluation, immunohistochemistry, serum alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG) were also taken into consideration. Sarcoma was identified in 10 out of 14 patients, with rhabdomyosarcoma ranking first ( n = 4), followed by osteosarcoma ( n = 2), fusiform cell sarcoma ( n = 1), undifferentiated sarcoma ( n = 1), neurosarcoma ( n = 1) and myxoid sarcoma ( n = 1). Other histological types of malignant transformation included adenocarcinoma ( n = 3) and bronchoalveolar carcinoma ( n = 1). Overall, 9 patients relapsed at a median time of 84 months (range 60–168). Results Non-GCT malignant transformation was identified in the retroperitoneum (5), testis (3), mediastinum (3), peritoneum (2) and lungs (1). The CT and MR imaging findings before treatment and after relapse were evaluated with emphasis on imaging features that could possibly imply the presence of malignant transformation (heterogeneously enhancing soft-tissue masses, ossified masses with calcified lymph nodes, diffuse epiploic thickening associated with ascites and peritoneal nodules, pulmonary alveolar infiltration with septal thickening). All but 1 patient with TMT presented with nodal and distant metastases. The prognosis was poor: within a median follow-up of 59 months (range 3–180), 4 out of 14 patients were alive. Conclusion TMT is rare and associated with poorer survival compared to GCT. Imaging can be useful as CT and MR findings may suggest this entity and lead to an early biopsy and appropriate treatment.

Fluorescence in situ hybridization is a useful ancillary diagnostic tool for extraskeletal myxoid chondrosarcoma

Extraskeletal myxoid chondrosarcoma is a rare soft tissue tumor characterized by a nodular growth pattern with eosinophilic cells usually in a reticular pattern and abundant myxoid stroma. In contrast to other myxoid sarcomas, the majority of extraskeletal myxoid chondrosarcomas harbor a balanced translocation, t(9;22)(q22;q12), that fuses EWSR1 with NR4A3 (also known as CHN). Other less common translocations involving NR4A3 have also been described. We examined the diagnostic utility of fluorescence in situ hybridization for extraskeletal myxoid chondrosarcoma using the LSI EWSR1 break-apart probe (Abbott Molecular/Vysis, Des Plaines, IL, USA). Sixteen cases of extraskeletal myxoid chondrosarcoma with formalin-fixed paraffin-embedded tissue available were retrieved (1991–2007). The mean age at time of presentation was 57 years (range, 30–78). The male to female ratio was 7:1. All cases where either consistent with or highly suggestive of the diagnosis, with most of the primary tumors occurring in the thigh, inguinal or gluteal region. Fifteen of 16 cases were analyzable, of which 14 (93%) were positive for the rearrangement of the EWSR1 locus. In this study, the vast majority of extraskeletal myxoid chondrosarcomas are associated with a rearrangement at the EWSR1 locus (22q12). Fluorescence in situ hybridization is useful to support the diagnosis of extraskeletal myxoid chondrosarcomas and may help to differentiate it from mimics such as other myxoid sarcomas, particularly in limited biopsies.

Effectiveness of Radiotherapy in Myxoid Sarcomas Is Associated With a Dense Vascular Pattern

Surgery and adjuvant radiotherapy (RT) have long been the standard treatment for most deep-seated sarcomas; however, since the randomized trial from the National Cancer Institute of Canada, which described similar local control for pre- vs. postoperative RT, both modalities are now widely accepted. As a group, sarcomas are classified as radiation resistant. The subgroup of myxoid liposarcoma (MLS), a sarcoma with a typical vascular crow's feet pattern, is highly radiosensitive, but a mechanism for this phenomenon is unknown. Here we describe our results with preoperative RT and propose a mechanism explaining the high sensitivity based on the distinctive vascularization pattern of MLS. Between 2002 and 2006, 31 sarcoma patients, including 10 with MLS, underwent preoperative RT at our institute. Resected specimens were histologically evaluated, focusing on classification, grade, and vascularization patterns. Twenty sarcomas showed more than 80% pathologic response after preoperative RT. A pathologic complete response was found in all "pure" MLS specimens after preoperative RT (n = 8). There were no pathologic complete responses in the remaining sarcoma patients (n = 23), although 12 showed 80% to 90% pathologic response. In contrast to the remaining RT-resistant sarcomas, the highly responding specimens contained branching vasculature, partial thrombus formation and inflammation of medium sized arterioles, similar to the vascular changes in MLS. Both MLS and sarcomas with MLS-like vasculature are highly radiosensitive. Radiation sensitivity may be explained by changes in medium-sized arterioles, obstructing the specific crow's feet vascularization and inducing hypoxia with secondary tumor cell death.

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.

Mixoma de ovario

Ovarian myxoma is a rare ovarian tumor. We identified only 16 cases in the literature reviewed. This neoplasm presents as a unilateral mass in women of reproductive age and is usually asymptomatic. We report the case of a 24-year-old woman, with an unremarkable personal and familial history, in whom a right adnexal tumor was detected on routine ultrasound examination. A right ovarian complex mass was diagnosed and exploratory laparotomy was performed, showing a 10 × 10 irregular mucinous pediculated tumor of the right ovary. The mass was excised and an intraoperative biopsy specimen showed no signs of malignancy. Microscopic examination revealed edematous, myxomatous stroma positive for PAS-Alcian blue and colloidal iron staining. Fusiform and stellate cells without nuclear atypia or mitosis were observed, with no findings of conserved ovarian parenchyma. Abundant thin-walled vessels were identified. Immunohistochemical analysis was positive for actin, weakly positive for desmin and negative for cytokeratin. The patient is currently asymptomatic, with no evidence of recurrence after 9 months of follow-up. Ovarian myxoma is uncommon and should be distinguished from other entities such as massive ovarian edema, myxoid sarcoma and embryonic rhabdomyosarcoma.

Myxoid Dermatofibrosarcoma Protuberans: Clinicopathologic, Immunohistochemical, and Molecular Analysis of Eight Cases

Dermatofibrosarcoma protuberans (DFSP) represents a locally aggressive mesenchymal neoplasm of skin and subcutis with characteristic clinicopathologic, immunohistochemical, and molecular findings. In addition to typical cases, morphologic variants such as pigmented, fibrosarcomatous, myofibroblastic, and granular cell DFSP have been described. Purely or predominantly myxoid DFSP is extremely rare, and may cause considerable diagnostic problems. Eight cases of predominantly myxoid DFSP were studied. Paraffin-embedded blocks and slides were retrieved from the files of the authors. Clinical data were obtained from the referring pathologists and dermatologists. Immunohistochemistry was performed using the ABC method, and three cases were studied by polymerase chain reaction technique. There were six male and two female patients (age range: 29 to 74 years). Locations included the inguinal area (three cases), thigh, upper arm, shoulder, abdominal wall, and back (one each). The patients were treated by wide excision as well as reexcision. Tumor size ranged from 1.5 to 12 cm. Histologically, a nodular growth with peripheral diffuse infiltration, as well as a diffusely infiltrating growth of relatively uniform spindled and stellated tumor cells containing slightly enlarged nuclei, was noted. Three cases were entirely myxoid, and in five cases more than 80% of the tumor area showed myxoid stromal changes. In two cases each, focal fibrosarcomatous and focal giant cell fibroblastoma-like changes were present. At least focally, hypocellular areas were evident in one case. Scattered enlarged tumor cells were seen in two cases. The mitotic rate ranged from 1 to 10 mitoses in 10 high-power fields. Numerous blood vessels with slightly fibrosed vessel walls were seen in seven cases. Immunohistochemically, tumor cells in all cases stained positively for CD34, and in one case each a focal expression of alpha-smooth muscle actin and epithelial membrane antigen (EMA) was noted. The remaining antibodies (CD99, CD31, S-100, Factor XIIIa) were all negative. Polymerase chain reaction technique showed in one case the characteristic COL1A1-PDGFB fusion gene. Follow-up information in seven cases (range: 2 months to 10 years; mean: 62 months; median: 48 months) revealed a local recurrence at 5 years. In conclusion, myxoid DFSP represents a very rare morphologic variant with characteristic changes that has to be distinguished from benign and malignant myxoid mesenchymal neoplasms as superficial angiomyxoma, superficial acral fibromyxoma, myxoid solitary fibrous tumor, myxoid perineurioma, low-grade myxofibrosarcoma, low-grade fibromyxoid sarcoma, myxoid liposarcoma, and myxoid synovial sarcoma.

Fine needle aspiration cytology of myxoid lesions of soft tissues: a study of 24 cases

To summarize the diagnostic features of fine needle aspiration cytology (FNAC) of myxoid lesions in soft tissue, and to define the cytological criteria for differentiating benign lesions from sarcomas and between various myxoid lesions. FNAC data of 24 soft tissue myxoid lesions (14 benign lesions, 10 malignant lesions) from 1993 to 2006 from Kiang Wu Hospital, Macau were reviewed in correlation with the clinical course or the results of biopsy. Ganglion, myxoma, and myxoid nodular fasciitis were the most common benign myxoid lesions of the soft tissues, all of which had low cellularity and lack of marked cellular atypia in smears in common. Ganglion was characterized by the disappearance of or the gradual minimization of the nodule after aspiration and the lack of blood vessel in smears. Myxoid nodular fasciitis was characterized by a pleomorphic pattern of proliferative cells and the presence of ganglion cell-like cells. Myxoid liposarcoma, extraskeletal myxoid chondrosarcoma and myxofibrosarcoma were the most common myxoid sarcomas. Cytologically, they had high cellularity and various degrees of cellular atypia. Myxoid liposarcoma exhibited branching chicken-wire like capillary vessels and/or lipoblasts; myxofibrosarcoma were prominent in both pleomorphic and atypia of the cells. The data revealed that the diagnostic accuracy of FNAC was nearly 100% in differentiating benign and malignant myxoid lesions of the soft tissues. The coordinate rate to the histopathology diagnosis of the common myxoid lesions of the soft tissues was above 75% (benign 71.4% and malignancy 80% respectively). FNAC is an effective method in diagnosing myxoid lesions of soft tissue, in correlation with the clinical data and the accessory examinations. FNAC can provide an objective basis for the treatment of myxoid lesions and prevention of unnecessary surgical operations.

Cytopathology of extraskeletal myxoid chondrosarcoma

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft-tissue sarcoma rarely subjected to cytologic analysis. With the exception of a few small series, the cytology literature of EMC is largely limited to single-case reports. The purpose was to evaluate the cytomorphology of 8 EMC cases as obtained by imprint/scrape cytology and fine-needle aspiration (FNA) biopsy, to review the literature, and to demonstrate the utility of cytogenetic analysis in the diagnosis of EMC. The cytology files were reviewed for all soft-tissue lesions signed out as chondrosarcoma, myxoid sarcoma, and EMC, and the tissue files for any cases of EMC that had corresponding cytopathology. FNA was performed using a standard technique. Scrape preparations were performed from tissue sent fresh to the laboratory for either frozen section or for special studies such as electron microscopy or tissue banking. Eight cases of EMC were retrieved from 4 men and 3 women (median age = 62 years). One patient had 2 separate cytologic specimens 4.5 years apart. All patients had subsequent tissue confirmation of the diagnosis of EMC. Five individuals presented as new patients, and 2 had a prior diagnosis of EMC. Sites included 4 masses from the foot/ankle, 2 from the calf, 1 wrist mass, and 1 buttock mass. Five patients were diagnosed from FNA biopsy, whereas 3 were diagnosed using scrape slides. Five cases were correctly and categorically diagnosed by the cytologic method as EMC, 1 as chondrosarcoma favor EMC, 1 as sarcoma favor EMC, and 1 as myxoid spindle/epithelial neoplasm. Cytologic features ranged from hypocellular to highly cellular smears composed primarily of rounded cells set in an abundant myxoid stroma that varied from opaque to semitransparent and lacked vascularity or necrosis. Smears showed cells in short, sometimes anastomosing cords, but also as single cells and nondescript cell clusters. Cells displayed a monotonous uniformity in nuclear diameter and cell size. Bland nuclei with evenly dispersed chromatin displayed variably sized nucleoli, and a moderate amount of infrequently vacuolated cytoplasm. Tissue fragments of variable size were found in 5 of 5 FNA cell blocks. Fluorescence in situ hybridization (FISH) analysis using the EWSR1 probe showed a positive 22q12 translocation in 2 of 3 FNA cases that were tested. One case with negative FISH results on the cytologic preparation showed a positive translocation using the same technique in the subsequent resection specimen. A confident cytologic diagnosis of EMC depends on the presence of a uniform, round to oval cell population often arranged in cords and set in an abundant myxoid/chondromyxoid background and arising in the appropriate clinical context. If positive, FISH testing (of paraffin cell blocks or cytospin preparations) is confirmatory when coupled with this cytomorphology.

Phase I/II study of docetaxel (D), gemcitabine (G), and bevacizumab (B) in patients (pts) with advanced or recurrent soft tissue sarcoma (STS)

10056 Background: The combination of G and D has shown efficacy against refractory STS (Hensley, JCO 2002). STS have a high content of VEGF, which is associated with poor prognosis. This phase I/II study assesses the safety, tolerability, efficacy, and pharmacodynamics (PD) of B in combination with G+D, given on a two-weekly schedule to minimize adverse events (AEs). We are reporting the phase I results. Methods: Untreated pts with advanced or recurrent STS and ECOG PS =2 were eligible. Some pts were treated in a neoadjuvant setting, when surgically appropriate. Planned doses were G 1,000, 1,250, and 1,500 mg/m2, D 50 mg/m2, and B 5 mg/kg iv, every 2 wks. G doses were escalated in serial pt cohorts to determine the maximum tolerated dose (MTD) of G with fixed doses of D and B. MTD was assessed on the first 2 cycles (1 cycle = 2 wks). Treatment was continued until progression or unacceptable toxicity. For neoadjuvant therapy, B was given only for 4 cycles, followed by 4 cycles without B in anticipation of surgery and pts came off study at that point. PD and antitumor efficacy were also assessed. Results: Nine pts have been treated on the phase I escalation arm, including 3 in the neoadjuvant setting. There were no dose limiting toxicities. After 4 cycles with G at 1,500 mg/m2, there was 1 asymptomatic grade 4 bowel perforation at the site of the tumor in a pt with initially inoperable leiomyosarcoma (LMS). After emergency surgery, the pt is free of disease. Observed grade 1 and 2 AEs include alopecia, diarrhea, fatigue (5 each), rigors (4), nausea, dyspnea, headaches (3 each), chest pain, epistaxis, stomatitis, anemia (2 each), rash, hypertension, neuropathy, leukopenia (1 each). There were 1 CR (angiosarcoma), 2 PR, (myxoid sarcoma, undifferentiated sarcoma), 4 NC (2 LMS, liposarcoma, PNST), and 2 PD (myxoid sarcoma, PNST). Necrosis was observed, including in NC disease. Three pts are free of disease after surgery. Conclusions: The combination of G, D, and B given every 2 weeks is safe and has demonstrated some activity in pts with advanced or recurrent STS. The phase II arm is ongoing at G 1,500 mg/m2 with 4 pts already enrolled. Mature data including PD will be reported at the meeting. No significant financial relationships to disclose.

Primary malignant myoepithelioma of the distal femur

A 55-year-old male presented with a 1-month history of localized pain caused by an osteolytic and destructive lesion in the right distal femur. Histologically, the tumour consisted of spindle cells intermingled with epithelioid eosinophilic cells arranged in small cords embedded in a hyalinized-to-chondromyxoid stroma. Electron microscopy and immunohistochemistry showed features of myoepithelial differentiation. RT-PCR failed to demonstrate chimeric transcripts of extraskeletal myxoid chondrosarcoma. The final diagnosis was primary malignant myoepithelioma of bone. The patient is alive with lung metastases 13 months after surgery. Primary malignant myoepithelioma of bone is an exceptionally rare neoplasm that should be considered in the differential diagnosis with the more aggressive myxoid spindle cell sarcomas.

The Diagnostic Utility of Fine-Needle Aspiration Biopsy of Soft-Tissue Sarcomas in the Core Needle Biopsy Era

Abstract: Fine-needle aspiration biopsy (FNAB) is a widely accepted and established diagnostic technique in diagnosing the presence of primary malignancies, metastatic disease, and benign nonneoplastic lesions. However, its role in the evaluation of soft-tissue sarcomas has remained controversial, especially as the primary modality for establishing an initial diagnosis. Arguments in favor of using core needle biopsies (CNB) relate to the fact that such samples provide ample “tissue” for evaluating architectural patterns, as well as a source for performing ancillary studies. However, in the hands of experienced cytopathologists, FNAB in conjunction with ancillary studies (performed on tissue obtained in the form of cell blocks) has been shown to have a diagnostic yield nearly identical to CNB, with an accuracy rate approaching 95% for the diagnosis of malignancy. Additionally, as therapy and prognosis are heavily dependent on the grade and stage of the tumor, it has been shown that approximately 90% of soft-tissue sarcomas can be successfully subtyped and even graded by FNAB. There are, however, certain limitations of FNAB, especially in the workup of low-grade myxoid and spindle cell sarcomas and in their separation from borderline and benign lesions. In these select subgroups, CNB may be more advantageous. Also, in the absence of an on-site cytopathologist for immediate evaluation of the sample and triage of materials for ancillary studies, CNB is preferred, although FNAB can still identify benign soft-tissue lesions and malignancies of nonmesenchymal origin not requiring ancillary studies. This review will focus on the more commonly encountered soft-tissue sarcomas and highlight the advantages and limitations of FNAB in establishing their diagnosis.

Diagnostic pitfalls associated with fine-needle aspiration biopsy in a patient with the myxoid variant of monophasic fibrous synovial sarcoma

Synovial sarcoma (SS) is one of the most common soft tissue tumors that typically presents in the extremities of young adults, but may occur at any site and affect children during the first decade. Herein we discuss a 12-yr-old male who complained of left foot pain and plantar mass. A fine-needle aspiration biopsy of an 8 cm subcutaneous mass was performed revealing a myxoid spindle cell neoplasm. The cytologic differential diagnosis included a myxoid neurofibroma, neurothekeoma, and a myxoid sarcoma. Subsequent excision of the mass revealed a monophasic fibrous SS with myxoid features. Examination of the tissue by fluorescence in situ hybridization confirmed the presence of characteristic SS SYT gene rearrangement at chromosome 18q11.2. This case underscores that the cytologic distinction of mxyoid spindle cell tumors may be challenging. We report the cytologic features of a myxoid monophasic fibrous SS, and discuss its distinction from other benign and malignant myxoid soft tissue neoplasms.

Matrix “blues”: clue to a cranial thoracic mass in a dog

A 5-year-old, intact male Italian Spinone dog was presented for progressive, severe dyspnea and coughing. Thoracic radiographs revealed a large mass in the right cranial thorax. Fine needle aspiration of the mass yielded a highly cellular sample containing dense clumps of oval to spindle-shaped mesenchymal cells with distinct intracytoplasmic vacuolation, consistent with lipoblasts and lipocytes. Cell clusters were associated with abundant eosinophilic matrix, which was identified as mucin, based on Alcian blue staining. At exploratory thoracotomy, the mass was found to be nonresectable, and the dog was euthanized. Histologic sections of the multilobular mass had discrete regions of variable cellular differentiation, including highly cellular areas of pleomorphic cells, areas of spindle cells and lipoblasts in a myxoid background, and areas of well-differentiated lipogenic cells. The histologic diagnosis was myxoid liposarcoma. The thoracic cavity is a rare site for liposarcoma in the dog. The cytologic features of lipoblasts together with a mucopolysaccharide matrix were useful for distinguishing the myxoid variant of liposarcoma from other forms of liposarcoma and myxoid sarcomas.

Conservative management of a myxoid endometrial stromal sarcoma in a 16-year-old nulliparous woman

Background. Sarcomas comprise less than 1% of all gynecological cancers and 2% to 5% of all uterine malignancies, of which endometrial stromal sarcoma accounts for approximately 15%. Surgery is fundamental in sarcoma management and a total hysterectomy is performed in the majority of cases. Case. Presented is a case of low-grade myxoid endometrial stromal sarcoma in a nulliparous adolescent female managed by local resection and uterine reconstruction. Conclusion. In selected cases, the initial management of a localized and well defined low-grade endometrial stromal sarcoma could potentially be conservative (uterine-sparing) surgical resection.

Myxoid Clear Cell Sarcoma

Clear cell sarcoma is a rare soft-tissue tumor presenting typically in the extremities of young adults. It has been also known as malignant melanoma of the soft parts because of the presence of melanin and cytoplasmic melanosomes. However, clear cell sarcoma is, at present, usually considered as a unique lesion because the t(12;22)(q13;q12) translocation is present only in clear cell sarcoma. Myxoid malignant melanoma is now a well-recognized morphologic variant of malignant melanoma. However, a myxoid variant of clear cell sarcoma has not been well described yet. We report a case of myxoid clear cell sarcoma occurring on the heel in a 22-year-old man. The tumor was composed of nests and fascicles of oval to fusiform cells with clear to pale eosinophilic cytoplasm, often separated by fibrous septa. The tumor cells were reactive for S-100 protein, HMB-45, and MART-1. Variably sized cysts lined by one or several layers of tumor cells were observed. Alcian blue and mucicarmine stains demonstrated prominent mucin deposition in the tumor stroma and especially in the lumen of the cysts. Fluorescence in situ hybridization for the Ewing sarcoma gene showed rearrangement in nearly all of the neoplastic cells.

Superficial Acral Fibromyxoma: Clinical and Pathological Features

Superficial acral fibromyxoma (SAFM) is a recently recognized myxoid tumor that usually occurs on the fingers and toes of middle-aged adults. We report on the typical case of a 50-year-old woman with a SAFM in the right big toenail that had been slowly growing for more than 10 years. To our knowledge, this case is the first reported case for which clinical pictures are available. Histologically, the lesion was non-encapsulated and was composed of stellate and spindle cells, arranged in a myxoid matrix. No atypia or mitotic figures were found. Neoplastic cells showed positive staining for CD34 and negative staining for epithelial membrane antigen (EMA), actin, desmin, keratins, S100 protein, CD99, and HMB45. Differential diagnosis encompasses benign and malignant myxoid and spindle cells tumors such as myxoid neurofibroma, sclerosing perineurioma, superficial angiomyxoma, and several low-grade myxoid sarcomas.

Anesthetic Management for the Excision of Left Atrial Myxoid Sarcoma Preoperatively Diagnosed as a Left Atrial Myxoma: A case report

We report a case of myxoid sarcoma in the left atrium, which was diagnosed as benign myxoma on preoperative echocardiography. At operation, semisolid mobile masses were found that attached with broad stalk to the posterior left atrial wall and that invaded into the left pulmonary vein and the anterior mitral leaflet. The patient underwent excision of left atrial tumor masses, mitral valve replacement, and left pneumonectomy. The histological diagnosis was undifferentiated cardiac myxoid sarcoma. Although echocardiography lead to the choice of diagnostic test of intra-atrial tumor, magnetic resonance imaging, computed tomography, and intraoperative transesophageal echocardiography may be needed for differential diagnosis, with indeterminate malignant findings on preoperative transthoracic echocardiography. Anesthesiologists are often confronted with cases that are unusual, therefore preoperative understanding of the cases are important for safe anesthetic management.