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• W1965576756 abstract "An 11-year-old girl presented with a left leg mass. Six months before presentation at the authors’ facility, the patient and her family observed painless swelling in her left calf. It was somewhat tender when struck, but not especially tender to touch. The patient denied constitutional symptoms, including fevers or malaise. Otherwise, her medical history, developmental history, and family history were noncontributory. The patient was able to do routine activities of daily living without difficulty. On presentation there was a soft, minimally tender 8 × 6 × 4 cm deep mass arising from the posterolateral aspect of the left calf. The overlying skin was normal. There was no increased warmth, erythema, or edema. The mass did not move when the ankle or knee was moved. Otherwise the examination was unremarkable. The leg was neurovascularly intact with normal strength and range of motion (ROM) at all joints. Routine laboratory studies, including complete blood count, erythrocyte sedimentation rate, and C-reactive protein were normal. Anteroposterior (AP) and lateral radiographs, computed tomography (CT) scans, and magnetic resonance imaging (MRI) scans of the leg were obtained by the primary physician (Figs 1–4).Fig 1.: Axial CT image of the midcalf obtained after intravenous injection of iodinated contrast.Fig 2.: Axial T1-weighted MRI scan of the midcalf.Fig 3.: Sagittal T2-weighted MRI scan of the midcalf.Fig 4.: Sagittal T1-weighted image obtained after intravenous injection of gadopentetate dimeglumine.Based on the history, physical findings, and imaging studies, what is the differential diagnosis? RADIOGRAPHIC INTERPRETATION Radiographs suggested a posterolateral soft tissue mass in the calf, but otherwise were noncontributory. The CT scan showed a 3 × 5 × 6 cm mass of predominantly decreased attenuation with septations and a thin minimally-enhancing rim in the posterior compartment of the calf (Fig 1). There was no calcification in the mass. The CT scan was done with intravenous contrast and the low attenuation of the mass after injection suggested lack of contrast enhancement, which is seen in a nonvascular or fluid-filled mass such as a cyst, hematoma, or lymphatic malformation. The lack of a thicker enhancing rim or nodule made a necrotic tumor or inflammatory mass a much less likely possibility. The MRI scan showed the minimally heterogeneous mass of predominantly low T1 (Fig 2) and high T2 signal (Fig 3) that enhanced brightly after contrast injection (Fig 4). On CT and MRI scans, some septations in the mass had imaging characteristics that were consistent with fat. There was no evidence of bone involvement or erosion on either study. DIFFERENTIAL DIAGNOSIS Neurofibroma Rhabdomyosarcoma Liposarcoma Malignant fibrous histiocytoma Schwannoma Synovial cell sarcoma Alveolar soft part sarcoma Lipoma Lipoblastoma Incisional biopsy of the mass with intraoperative frozen section was done shortly after presentation. Images of the histologic evaluation are shown in Figure 5.Fig 5A–D.: (A) High magnification view (×400) with hematoxylin and eosin stain. (B) Low magnification view (×100) with hematoxylin and eosin stain. (C) High magnification view (×400) with hematoxylin and eosin stain. (D) High magnification view (×400) with hematoxylin and eosin stain.Based on the history, physical findings, radiographic studies, and histologic evaluation, what is the diagnosis and how should this patient be treated? HISTOLOGY The tumor had several characteristic features. First, the background of the lesion consists of a myxoid matrix that has a gray to faint eosinophilic appearance on hematoxylin and eosin stain. Delicate branching capillaries that form a crow’s feet or chicken-wire vascular pattern often are identified within the myxoid matrix and are of particular diagnostic usefulness (Fig 5A). Microcysts containing finely granular eosinophilic or blue-gray mucinous material, as shown on hematoxylin and eosin stain, are additional background features characteristic of myxoid liposarcoma 8 (Fig 5B). Additionally, the translocation t(12;16)(q13;p11.2), typical of myxoid liposarcoma, was present. Immunohistochemistry was positive for vimentin and S-100 protein. Oil red O stain also was positive. There are several types of tumor cells that make up the myxoid liposarcoma group. The predominant tumor cell type is that of the small, undifferentiated spindle mesenchymal cell. However, it is the additional histologic characteristics that define this lesion as a myxoid liposarcoma. Signet ring cells composed of one large cytoplasmic vacuole and eccentric nucleus also are distributed throughout the lesion with a noticeable accumulation of lipoblastic cells at the peripheral subcapsular region of the tumor (Fig 5C). Mitotic figures are observed infrequently. Tumor cellularity often varies throughout this lesion. Tumors with 10% or greater round cell component are classified as high-grade by some authors and referred to as combined myxoid and round cell liposarcoma. 3,13 Tumors with 75% or more round cell component are referred to as round cell liposarcoma. 3,13 The tumor in the current patient had some round cell differentiation that constituted less than 10% of the examined sectional area (Fig 5D); therefore, the diagnosis of myxoid liposarcoma rather than combined myxoid and round cell liposarcoma was made. DIAGNOSIS Myxoid liposarcoma TREATMENT Other studies (radiographs of the chest, CT scans of the chest and abdomen, and a bone scan) were done preoperatively and the results were negative. An oncology consultation was obtained preoperatively. Once the biopsy confirmed the diagnosis of myxoid liposarcoma, neither chemotherapy nor radiation therapy was recommended as long as wide tissue margins were obtained. A wide resection of the low-grade myxoid liposarcoma was done, including removal of the lateral head of the gastrocnemius. Wide margins were confirmed; neither chemotherapy nor radiation therapy was given. At 36 months followup, the child has no motor, neurologic, or functional deficits, and there is no evidence of recurrence or metastasis. DISCUSSION Although liposarcoma is one of the more common types of soft tissue sarcoma in adults, 10,12,21,25,36 it is rare in children. 5,10 Accordingly, there are several studies about liposarcoma in the pediatric population. 24,33 In a review of 2500 cases of liposarcoma, only 17 occurred in patients younger than 16 years. 33 LaQuaglia et al 24 found only 18 cases of liposarcoma in patients 22 years or younger in a review of 41 years at Memorial Sloan-Kettering Cancer Center. Stout classified liposarcoma into well-differentiated, myxoid, poorly-differentiated myxoid, and round cell types. 37 Enziger and Winslow 11 modified Stout’s classification into well-differentiated, myxoid, round cell, and pleomorphic types. Round cell liposarcoma frequently is included under myxoid type but is considered a more poorly-differentiated form of myxoid liposarcoma. 10,21,34 Fibroblastic, lipoblastic, and dedifferentiated types also are included in certain classifications. There also may be several histologic types seen in any one tumor. Myxoid liposarcoma is the most common of the types 6,10,21,24,30,31,34,36,39 (39%–85% of liposarcomas depending on the study and classification used). The tumor typically has a t(12;16)(q13;p11) translocation 14,19 or more rarely a t(12;22) translocation, 9,38 which results in the fusion of the transcription factor gene CHOP on chromosome 12 with TLS/FUS on chromosome 16 or EWS on chromosome 22. 23 This fusion product may result in transcription errors. It also may be used as a diagnostic molecular marker. 17 It can be used to distinguish liposarcoma from the histologically similar lipoblastoma, which has a breakpoint in the long arm of chromosome 8. 26 Additionally, myxoid liposarcoma expresses vimentin (a nonspecific sarcoma marker) and S-100 protein (a neural stain that is uniformly positive in liposarcoma), and is positive for oil red O, a neutral fat stain. 15 Myxoid liposarcoma usually presents as a painless, soft, enlarging mass which often grows to be large. 10,21,35 Most myxoid liposarcomas measure between 5 and 10 cm in greatest diameter. 10,39 It often causes no symptoms before the mass becomes large. 1,27,36,39 Only 10% to 15% of patients present with “pain, tenderness, or functional disturbances.”10 The tumor usually is deep to the superficial fascia and may be intramuscular but is more commonly intermuscular or perivascular. 10,12,36 Myxoid liposarcoma most commonly occurs in the extremities and next most commonly in the retroperitoneum. 10 The lower extremities are much more commonly affected than the upper extremities, and the thigh is affected most commonly. 6,12,21,26,27,30,31,36,39 When it does occur in the retroperitoneum, the diagnosis often is delayed. When the gastrointestinal tract is involved, nausea, vomiting, and anorexia may be seen. Pressure on the genitourinary structures may result in hydronephrosis, pyelonephritis, and uremia. 10 Typically however, there are no systemic symptoms. Physical examination reveals a nonspecific soft tissue mass but otherwise is noncontributory. After initially screening a mass with plain radiographic imaging, MRI is the imaging study of choice to assess soft tissue tumors. 2,29 Magnetic resonance imaging provides information to evaluate the tumor characteristics, the tumor extent, and invasion of adjacent structures. Most soft tissue neoplasms are low signal on T1-weighted images and high signal on T2-weighted images. Soft tissue sarcomas can be homogenous or heterogenous and may have well- or ill-defined margins. Gadolinium injection is useful and may help differentiate a solid mass from a cystic mass. When there is a predominantly cystic mass, nodular enhancement suggests the presence of an underlying neoplasm. Liposarcoma can have a varied appearance on MRI scans; it may be predominantly fatty tissue with fibrous septa or totally soft tissue signal with minimal or no fat, as in the current case. A fatty tumor only can be considered a benign lipoma (versus lipoblastoma or liposarcoma) if it has purely fatty tissue (no septae) and a well-defined margin. 2,18,20,29 Neurofibroma typically presents with a central dark area on T2-weighted images, which was not seen in the current case. Biopsy often is required for diagnosis. Computed tomography scans of the chest, abdomen, and peritoneum should be done for staging. 31,36 A technetium bone scan should be done to additionally evaluate the bone and the soft tissues. 31,36 Sarcoma should be considered in any patient with a large soft tissue mass deep to the superficial fascia until this diagnosis is excluded by biopsy. 31,38 A biopsy is done using tumor surgery principles when there is any diagnostic doubt. Ideally, the surgeon who will do the definitive operation should do the biopsy. When the biopsy specimen is obtained, frozen section may be necessary to confirm that an adequate sample has been taken. The sample is sent to microbiology for stains and cultures, and should be handled to allow for special studies such as immunohistochemistry, electron microscopy, and cytogenetics. A needle biopsy is an alternative to an open biopsy, but the treating surgeon should identify and record the location of the biopsy tract. Surgical resection with wide margins is the mainstay of treatment for patients with myxoid liposarcoma. 4,22,26,27,36 Lower recurrence rates may be observed in adults when radiation therapy is added, but its role in children is controversial. 10,12,22,35 In a retrospective series, conservation surgery with radiation therapy was used for all patients. 39 Local control was achieved in more than 90% of patients with low-grade tumors when surgical resection and radiation therapy were used. Therefore, radiation therapy is recommended for most patients with liposarcomas. 39 In one study, the recurrence rate was 20% (10 of 49) in patients who received excision with radiation therapy, compared with 73% (eight of 11) in patients who received excision only. 35 However, there were serious limitations in the study: the authors did not distinguish between types of liposarcoma, and statistical analysis was not done. Evaluating myxoid liposarcoma in particular, Evans 12 reported recurrence rates of 78% (14 of 18) for patients who had excision alone, compared with 13% (one of eight) for patients who had excision with radiation therapy. This was statistically significant. Uncontrolled recurrence occurred in 22% (four of 18) of patients with excision alone compared with 0% (0 of eight) for patients with excision with radiation therapy; metastasis occurred in 39% (seven of 18) of patients with excision alone, compared with 25% (two of eight) for patients with excision and radiation therapy. However, neither the rates of uncontrolled recurrence, the rates of metastasis, nor the mortality rates were statistically significant. In contrast, in a series of 10 patients with surgical resection with wide margins and no radiation therapy, no patient had evidence of disease at latest followup (range, 1.3–29.1 years; median, 8.5-year followup). 21 Campbell et al 4 evaluated patients treated with wide resection and patients treated with less than wide resection. No patient with adequate margins had a recurrence. Ninety-one percent of patients with inadequate margins had at least one recurrence. Kilpatrick et al 21 found no statistically significant difference in the rate of metastasis between patients treated with radiation therapy and patients treated without radiation therapy. Radiation therapy also is not a benign treatment. 22,28 Radiation therapy may cause physeal growth arrest, osteonecrosis, slipped capital femoral epiphysis, and muscle and skin injury. When directed near the gonads, it may be associated with sterility and endocrine abnormalities (including delayed sexual maturation and amenorrhea). In the gastrointestinal tract, radiation therapy may be associated with fibrosis, stricture, malabsorption, and liver injury. Radiation therapy may cause nephropathy with secondary hypertension. The risk of secondary malignant neoplasm after radiation therapy may be as high as 10% at 10 years and 14% to 17% at 20 years after treatment. 22,28 Because of this, the role of radiation therapy in children with myxoid liposarcoma is controversial 28; several authors recommend that if wide surgical margins are obtained, no adjuvant treatment (radiation therapy or chemotherapy) is necessary. 4,24,27,28,36 If the tumor encases vital neurovascular structures or organs however, wide resection may not be possible. In that case, or in any case where wide margins are not obtained, repeat (wide) resection when possible, with radiation therapy is recommended. 24,39 Chemotherapy usually is not recommended for patients with tumors resected with wide margins. 10,33,35,36,39 Because lymph node metastasis is rare, lymph node dissection is not recommended, except in cases with obvious lymph node involvement. 10 Outcome is related closely to histologic type. 6,12,30,35,39 In fact, several authors suggest that outcome is so closely related to histologic type that studies that do not so differentiate may be meaningless. 6,10,31,34,36,39 Prognostic factors for survival also include the ability to achieve a wide surgical margin 4,21,39 and tumor size. 30,39 The percentage of round cell tumor within the myxoid liposarcoma 3 seems to correlate with a poor prognosis. Myxoid tumors with greater than 5%34 or 25%21 round cell component are associated with a higher rate of metastasis and death attributable to disease. Age (> 45 years) and spontaneous tumor necrosis also may be associated with a poor prognosis, 16,21 although age has been reported to be a confounding factor in another study. 5 Tumor size 6,21,32,39 greater than 5 cm may correlate inversely with metastasis but not with local recurrence. 39 Azumi et al 3 reported that size of the tumor is not related to survival. Patients with proximal, large, deep lesions in the lower extremity may have a worse prognosis. 32 The 5-year survival rate for patients with myxoid liposarcoma ranges between 70% and 89%. 6,21,30,36 The 10-year survival rate ranges between 66% and 93%. 21,39 In patients who do not respond to initial treatment, survival is better for those who present with local recurrence than for those who present with metastasis. 6,39 For myxoid liposarcoma, the rate of metastasis ranges between 10% to 35% at 10 years. 6,21,31,39 The time between diagnosis and metastasis may be long. 6 Aggressive tumor histologic features, 39 tumor size, 39 the amount of round cell tumor within the myxoid liposarcoma, 3,12,21 and a retroperitoneal location 10 also may correlate with metastatic potential. Myxoid liposarcoma has been recognized to metastasize to unusual locations. Fifty-nine percent to 94% of patients with metastasis, have metastases in extrapulmonary soft tissue sites (retroperitoneum, intestinal mesentery, chest wall, pleura, pericardium, pelvic sidewall, and other soft tissue locations). 7,10,12,31,39 These lesions have been interpreted as multicentric liposarcomas. 10 Metastases uncommonly occur in the lungs, which has important implications for pretreatment staging and posttreatment followup. Some authors 29,36 recommend CT scans of the abdomen and chest (thorax) for accurate staging and for posttreatment followup. These authors also suggest that “what may be a ‘primary’ myxoid liposarcoma of the trunk of retroperitoneum should undergo careful evaluation to rule out an occult primary tumor in an extremity.”31 Low-grade liposarcomas have a 7% to 11% local recurrence rate at 5 years. 6,39 Well-differentiated and myxoid types have a lower 10-year local recurrence rate (7.6%) than pleomorphic liposarcoma. 10,39 Failure to achieve wide surgical margins, 21 aggressive histologic features, 39 previous local recurrence, 39 and retroperitoneal location 10 all are related to local recurrence. The size of the tumor does not seem to be related to local recurrence. 3 Liposarcoma occurs very rarely in children. Myxoid liposarcoma most commonly presents as a painless, growing mass in the thigh. Treatment for myxoid liposarcoma includes surgical resection and includes radiation therapy if surgical margins are inadequate. Despite its low-grade nature, neither metastasis nor local recurrence is rare. Poor prognostic factors for survival include aggressive histologic features, positive surgical margin, spontaneous tumor necrosis, metastasis, and large tumor size; percent round cell involvement and patient age also may correlate with poor outcome. Aggressive histologic features, percent round cell component, retroperitoneal location, and tumor size are risk factors for metastasis; aggressive histologic features, positive surgical margins, retroperitoneal location, and previous local recurrence are risk factors for local recurrence. When metastases occur, they are often located in unusual, extrapulmonary locations; postoperative followup must appreciate this observation." @default.
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• W1965576756 title "Calf Mass in an 11-Year-Old Girl" @default.
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