Case Of Liposarcoma Research Articles

The first case of intraosseous liposarcoma of the mandible involving the condyle

The first case of intraosseous liposarcoma of the mandible involving the condyle

Primary Liposarcoma of The Breast: A Report of Five Cases

Liposarcoma of the breast is among the rarest of malignant mammary tumors. It was first described by Neumann in 1862 and accounts for 0.3% of all breast sarcomas. We reviewed in this study 5 cases of primary liposarcoma of the breast treated between 1997 and 2014 in Salah Azaiez institute. The purpose of this study is to highlight one of the rarest and interesting variant of breast sarcoma. The patients were four women and one man. The median age at diagnostic was 50 years for the women. The man was 80 years old. The median tumor size was 6.8 cm. All liposarcomas were unilateral. Four patients underwent total mastectomy and only one had a conservative surgery. Histologically, four tumors were classified as pleomorphic liposarcoma and one as myxoid liposarcoma. None of the patients had axillary lymph node metastases. Four patients had adjuvant treatment involving local radiation therapy, one of them had chemotherapy. None of the patients had regional or distant metastases with median follow up of 56 months (range, 12 to 204 months) after surgery. An immunohistochemical study should be considered in order to avoid misdiagnosis. The mainstay of treatment in breast liposarcoma is surgical excision. Adjuvant chemotherapy and radiation should be considered in high-risk cases.

Histological types of soft-tissue sarcomas at the lagos university teaching hospital.

There is scanty data on histologically diagnosed soft-tissue sarcomas in the Nigerian literature. This is due to paucity of facilities for ancillary testing as well as a dearth of specialist soft tissue pathologists. Knowledge however of the common soft-tissue sarcomas is vital for the establishment of an effective sarcoma service. The aim of this study, therefore, was to determine the histological spectrum of soft-tissue sarcomas in Lagos, Nigeria. Archival haematoxylin and eosin (H and E)-stained slides were retrieved and reviewed by a team of soft-tissue pathologists at the Royal National Orthopaedic Hospital, London, UK. Immunohistochemistry and fluorescent in situ hybridisation studies were performed on cases without definitive diagnosis on routine H and E. Fifty-two cases were studied. The male-to-female ratio was 1:1.3, with a median age of 33 years. Most sarcomas (57.5%) were of intermediate malignant potential according to 2013 World Health Organization classification. Kaposi sarcoma (37.5%), undifferentiated sarcomas (22.5%), dermatofibrosarcoma protuberans (15%) and myxofibrosarcomas (7.5%) were the most common sarcomas seen in adults. There was no case of liposarcoma. Sarcomas in the younger age group (<20 years) accounted for 23.1% of cases with embryonal rhabdomyosarcoma accounting for the majority. Soft-tissue sarcomas in adults in Lagos Nigeria show a different morphologic spectrum than those reported in Western countries.

MDM2・CDK4・p16の発現検索が診断に有用であった頭頸部脂肪肉腫の2例

MDM2・CDK4・p16の発現検索が診断に有用であった頭頸部脂肪肉腫の2例

Lipomes de la main – lesions rares – problèmes de diagnostic et de traitement chirurgical

Introduction The purpose of this paper is to highlight the rarity of lipoma localization in the hand, especially the ones with big dimensions localized in the palm (giant lipoma), the importance of IRM exam in establishing the positive and differential diagnostics, as well as the importance and the necessity of the surgical treatment regarding the functional rehabilitation of the hand. Material and methods Our study is based on 7 patients with lipomatous lesions in the hand localized as - in 2 cases on the dorsal face of the hand, in 4 cases on the thenar eminence and, in only 1 case, in the palm. In the last case, the lesion occupied the entire surface of the palm, with important functional deficiency and with paresthesia on the thumb and the index. In 3 cases out of 5 lesions localized on the volar face of the hand, the diameter of the lesion was over 5 cm, the inferior limit for the giant tumors. The biggest one was 9.5 5 3.5 cm. The IRM exam established the diagnostic of lipoma and in 1 case raised the questionable diagnostic of well-differentiated liposarcoma, which was confirmed by the pathological exam. In those 6 cases of lipoma, the surgical treatment was the complete removal of the lesion followed by the complete remission of the neurological symptoms and by the complete functional reintegration of the hand. In the liposarcoma case, the surgical treatment was followed by the oncological one, the hand amputation not being accepted by the patient. Discussion Lipoma is a slowly growing, asymptomatic tumor, which explains the large size at diagnosis, especially in cases with deep location. The symptoms are given by hand dysfunctions caused by tumor size and its compression on the neighboring tissues such as nerves or intrinsic muscles. The tumors having more than 5 cm in diameter, rapid growth and deep location can be malignant. MRI exploration is preferred in cases of lipoma for diagnostic specificity. Conclusions In establishing the diagnosis of a hand tumor, the surgeon must consider the possibility of a lipoma, despite its rarity. MRI exam is the most useful exploration in these cases, being able to establish the diagnosis with high accuracy. The differential diagnosis must be taken into account, mainly with the liposarcoma but also with other possible benign tumors of the hand.

Perilipin 1 is a highly specific marker for adipocytic differentiation in sarcomas with intermediate sensitivity.

Liposarcomas are the most common soft tissue sarcomas of adults. The identification of lipoblastic cells in soft tissue sarcomas is mandatory for the diagnosis of most subtypes of liposarcomas but may be difficult in conventional histology. The present study focuses on the expression and possible diagnostic impact of two PAT family proteins, perilipin 1/perilipin and perilipin 2/adipophilin in human liposarcomas. Eighty-seven cases of liposarcomas and 30 cases of non-lipomatous sarcomas were investigated immunohistochemically for perilipin 1 and 2 using entire tissue sections. Statistical analyses were performed using appropriate tests. Most liposarcomas and non-lipomatous sarcomas displayed positivity for perilipin 2. In contrast, while more than two-thirds of liposarcomas presented perilipin 1 positivity, all non-lipomatous sarcomas studied were negative for this marker, with statistical significance (p<0.001). Perilipin 1 expression increased with adipocytic differentiation of liposarcoma subtypes showing statistical significance (p<0.001). Non-lipomatous sarcomas demonstrated variable expression levels of perilipin 2. The expression level of perilipin 2 appeared to be correlated with tumor cell degeneration, e.g., through hypoxia. Perilipin 2 is not well suitable for distinction between liposarcomas and non-lipomatous sarcomas. However, perilipin 1 appeared to be a highly specific marker for liposarcoma and adipocytic differentiation in sarcomas with intermediate sensitivity.

Retropharyngeal liposarcoma: A rare cause of dysphagia

IntroductionLiposarcoma is a rare malignant mesenchymal tumour. Very few cases of retropharyngeal liposarcoma have been reported in the literature. SummaryA 30-year-old woman with no notable history reported swallowing disorders for the past year associated with dysphonia. Nasal endoscopic examination demonstrated a tumour arising from the left lateral pharyngeal wall, ascending as far as the junction of the free margin of the epiglottis, aryepiglottic fold, and pharyngoepiglottic fold and obliterating the ipsilateral pyriform sinus with preservation of cord mobility. The rest of the physical examination was normal. The preoperative assessment was completed by gadolinium-enhanced MRI of the neck. Open surgery was performed. Definitive histological examination concluded on dedifferentiated liposarcoma with negative resection margins. Following staging chest computed tomography that did not reveal any metastases, the multidisciplinary consultation meeting decided to deliver adjuvant volumetric modulated arc therapy. No recurrence was observed nine months post-treatment. DiscussionRetropharyngeal liposarcoma is a rare tumour. The authors report the first case of a dedifferentiated histological subtype in this site. A review of the literature completes this case report by providing a description of prognostic factors and the various treatments.

Two cases of canine liposarcomas: an uncommon tumour

Liposarcomas are rare malignant tumors of adipose tissues with overall incidence of 0.2–0.5% among all the canine neoplasms. They may originate either from bone marrow or extra skeletal sites such as adipose tissues. Histological classification include well differentiated, pleomorphic, and myxoid subtypes. Clinical cytological, gross pathological and histopathological features of two extra skeletal canine liposarcoma cases presented to the Veterinary Teaching Hospital (VTH), University of Peradeniya are described here.

Liposarcoma of Hypopharynx and Esophagus: a Unique Entity?

Liposarcoma is the most common soft tissue sarcoma in adults. It represents approximately 20% of all mesenchymal malignancies. It most frequently involves retroperitoneum, trunk, and extremities. Hypopharyngeal and esophageal localization of liposarcoma is extremely rare. We performed a systematic review of literature and reported 26 and 33 cases of hypopharyngeal and esophageal liposarcoma. We analyzed natural history, imaging features, histology, treatment, and prognosis, with a specific focus to similarities and differences between tumors of hypopharynx and esophagus. Hypopharyngeal and esophageal liposarcomas have more similarities than differences. Incidence has a peak at 6th and 7th decades. The diagnostic procedures are barium swallow, endoscopic examination, and CT/MR imaging. Well-differentiated liposarcoma represents the most frequent histological subtype. Surgical excision is the main treatment. Endoscopic resection can be useful for pedunculated tumors of hypopharynx and cervical esophagus. Differences between hypopharyngeal and esophageal liposarcoma are represented by local recurrence rate (greater for hypopharyngeal tumors), number of giant tumors, and time to recurrence (greater for esophageal tumors). Finally, liposarcomas of distal esophagus need more extended approaches. Liposarcomas of hypopharynx and cervical esophagus could be considered a unique pathological entity, with similar features and treatment options. Survival rate is dependent on histological type and location. Local recurrence is common, especially for hypopharyngeal liposarcoma, while the risk of lymph node or distant metastasis is very low. Patients should undergo regular examinations to rule out local recurrence, also for a long time, especially for esophageal tumors.

Primary dumbbell-shaped epidural myxoid liposarcoma of the thoracic spine: A case report and review of the literature.

Myxoid liposarcoma frequently occurs in the deep soft tissue of the extremities, particularly in the thigh. The present study describes an extremely rare case of primary dumbbell-shaped epidural myxoid liposarcoma of the thoracic spine occurring in a 22-year-old woman. Although preoperative magnetic resonance imaging findings were thought to be compatible with schwannoma, the pathological diagnosis of the resected tumor was myxoid liposarcoma. The patient underwent three courses of adjuvant chemotherapy with doxorubicin and ifosfamide, and exhibited no symptoms or signs of local recurrence or metastasis for 18 months post surgery. The present case suggests that, when radiologically diagnosing spinal epidural tumors, clinicians should consider the possibility of myxoid liposarcoma.

Isolated Subcutaneous Metastasis of Myxoid Liposarcoma: A Case Report

Background: Subcutaneous metastatic liposarcomas are rare. Differentiation between primary tumors and metastasis of a single liposarcoma represents the main difficulty in diagnosis. In this article, we describe the first report of Subcutaneous metastasis to the neck in the right level IB from liposarcoma originating in the thigh. Case presentation: The present 19-year-old women presented to a complaint of a rapidly growing mass, of the posterior loge of the right thigh with poor definition of adjacent structures, of 9 months’ duration measuring 188 × 97 mm. Microscopic examination of the mass following excision revealed a myxoid liposarcoma. A wide surgical resection was performed, and margins were negative. At this time, the patient showed no metastatic disease and underwent a complementary treatment including irradiation of the right thigh at a dose of 50 Gy delivered in 25 fractions over 38 days. There weren’t any local recurrence or metastases on her 12 months follow up until May 2015 when she presented with a mass in the neck (right level IB). An excisional biopsy was performed by an in June 2015 revealing a myxoid liposarcoma imposing a large re-excision of the tumor bed with 3cm free tumor margin. Microscopic examination of the surgical specimen found clear margins without an involvement of the skin. Differentiation between primary tumors and metastasis of a single liposarcoma was very difficult. The results of the disease extension workup showed no sigh of other metastases no local recurrence until now. Conclusion: To our knowledge, no case of cutaneous metastatic myxoid liposarcoma has been reported until now.

Liposarcoma of the Uterine Cervix in Nigeria: Case Report

A case of primary liposarcoma of the uterine cervix is described in a 65-year-old woman of the Igbo Ethnic Group in Nigeria on the basis of cytological features following cervical cone biopsy. This appears to be the second Nigerian case. She complements the recent cases from other parts of the world.

Huge Liposarcoma of the Forefoot: A Case Report

Sarcomas of the foot are conventionally treated with amputation. However, limb salvage procedures are possible in cases of specific histological patterns. We report a rare case of a huge myxoid liposarcoma of the dorsal and plantar aspect of the forefoot. A 47 years-old woman was admitted to our Institution complaining of a painless huge mass of the dorsal and plantar aspect of the left forefoot. Following the patient’s medical history remarking a previous myxoid liposarcoma, this finding was interpreted as a local recurrence. The mass was explored through radiographic, magnetic resonance imaging and bone scan assessments. An incisional biopsy was performed, and a myxoid liposarcoma was ultimately diagnosed. A limb salvage procedure was performed in association with coverage plastic surgery. Postoperative stay was uneventfully. Forty days after surgery the patient underwent a cycle of radiotherapy. At 10 year follow-up the patient had a good functionality without signs of local recurrence nor metastatic spread.

Two Forensic Autopsy Cases of Liposarcoma

Primary liposarcoma outside thighs is rare. We present two forensic autopsy cases of clinically undiagnosed primary liposarcoma (Case 1: Mediastinum, Case 2: Mesentery). Case 1 was a thin male in his 60s who had a well-differentiated liposarcoma (850 g, 23 cm × 20 cm × 10 cm) in the superior mediastinum. Case 2 was a thin female in her 50s who had a pleomorphic liposarcoma (10 kg, 40 cm × 25 cm × 11 cm) in the abdominal cavity with liver metastases. The cause of death in Case 1 was airway obstruction due to liposarcoma of the neck; that in Case 2 was complications of liposarcoma with metastasis. Because liposarcoma often appears in deeper tissue without pain, it will grow quite large before it is noticed.

Sclerosingliposarcoma of the Spermatic Cord – Case Report and Literature Review

Liposarcoma are a group of malignant tumours which are derived from mesodermal tissue. The spermatic cord is a rare site for liposarcoma; around 200 cases of Liposarcoma of the spermatic cord have been reported in the English literature to date. They typically present as a non-tender, slow growing masses of variable size in the scrotum or inguinal region. They are frequently misdiagnosed as other common pathologies, such as an inguinal hernia. We present a case of liposarcoma of the spermatic cord, which histopathology confirmed to be of sclerosing subtype.

Chondroitin sulfate synthase 1 expression is associated with malignant potential of soft tissue sarcomas with myxoid substance

The glycosyltransferases chondroitin sulfate synthase 1 (CHSY1) and exostoses-like 3 (EXTL3) specifically function in biosynthesis of the glycans chondroitin sulfate and heparan sulfate, respectively. Although these glycans play important roles in pathogenesis of various tumors, their significance in soft tissue sarcoma remains unknown. Here, we asked whether CHSY1 or EXTL3 expression correlates with malignant potential of soft tissue sarcomas with myxoid substance. To do so, we examined 40 samples representing specific types, including 12 cases of myxoid liposarcoma, 14 of myxofibrosarcoma, 12 of malignant peripheral nerve sheath tumor, and 2 of low-grade fibromyxoid sarcoma. We performed immunohistochemistry with anti-CHSY1 and anti-EXTL3 antibodies and compared enzyme expression levels with tumor histologic grade as assessed by the Fédération Nationale des Centres de Lutte Contre le Cancer classification and with patient 5-year survival rate. CHSY1 and EXTL3 were expressed in 72.5% and 32.5% of all tumors, respectively. Notably, CHSY1 was strongly expressed in myxofibrosarcoma and malignant peripheral nerve sheath tumor compared with other tumors and significantly associated with higher- rather than lower-grade tumors (P < .01). High expression of CHSY1 was also significantly associated with poorer patient outcomes (P = .031) and higher stages assessed by American Joint Committee on Cancer staging system (P = .004). By contrast, EXTL3 expression was not correlated with histologic grade or patient prognosis. We conclude that CHSY1 expression is closely associated with malignant potential of soft tissue sarcomas with myxoid substance.

Retrorectal Tumors in Adults: A 10-Year Retrospective Study

Due to the rarity and large diversity of the primary retrorectal tumors (RTs), the diagnoses are often difficult and they can be misdiagnosed. We present our experience in light of scarce information available on the clinical manifestations of RTs. The retrospective study included 17 patients diagnosed as RTs between January 2004 and January 2014. Demographic characteristics, length of symptoms, clinical findings, diagnostic methods, evaluations on the treatment procedures and postoperative periods, pathology, complications, and length of hospital stay were recorded. A mean of 1.7 of patients were diagnosed with RTs annually in our hospital. Patients comprised 12 females and 5 males. Pain and discomfort were the most common symptoms at presentation. All the lesions were evaluated by using magnetic resonance imaging (MRI) and computed tomography (CT), and all the patients were treated operatively. Based on the preoperative MRI or CT findings, an anterior approach was performed in 7 patients, a posterior approach in 6 patients, and combined approach in 4 patients. Mean size of tumors was 9.2 ± 4.3 cm. Epidermoid cyst (n = 8) was the most common tumor. Except for 1 case of liposarcoma, 16 tumors were confirmed to be of benign nature in histologic examination. Mean length of hospital stay 12.4 ± 6.8 days. Retrorectal tumors are heterogeneous and lead to diagnostic difficulties. A high index of clinical suspicion is needed for diagnosis. Preoperative imaging may be helpful in determining the course of treatment. Total excision of a retrorectal tumor may alleviate pressure symptoms and confirm the diagnosis.

Mediastinal Myxoid Liposarcoma with Intrapericardial Involvement and Large Pericardial Effusion

Liposarcoma is the name given to a group of soft tissue sarcomas (STSs) with adipocytic differentiation. As a group, liposarcomas are the second most common STSs in adults. In 1951 Kozonis et al published that in the English language only four cases of liposarcomas originating in the mediastinum had been described. Primary mediastinal liposarcoma is an uncommon neoplasm of intrathoracic origin. We present the case of a 47-year-old woman diagnosed with a large mediastinal mass with intrapericardial invasion and massive pericardial effusion; biopsies showed a mediastinal liposarcoma.

Liposarcomas of the posterior mediastinum: clinicopathologic study of 18 cases

Sarcomas of the posterior mediastinum are rare and correspond mostly to neurogenic tumors. We studied 18 cases of liposarcoma presenting in the posterior mediastinum; because of their unusual location, some of these tumors posed difficulties for diagnosis. There were 11 men and 7 women aged 29–87 years (mean: 57). The tumors were large lobulated masses ranging from 6 to 30 cm in greatest diameter (median: 15 cm). Symptoms included cough, dysphagia, and chest pain. Four patients were asymptomatic and the tumors were discovered incidentally on chest X-rays. Histologically, 10/18 (55%) cases were atypical lipomatous tumor/well-differentiated liposarcoma, one of which harbored a smooth muscle component (lipoleiomyosarcoma); 3/18 (16%) were de-differentiated liposarcoma, one of which also harbored a smooth muscle component; 3/18 (16%) were myxoid/round cell liposarcoma; and 2/18 (11%) were pleomorphic liposarcoma. The cases of well-differentiated liposarcoma were mostly of the sclerosing type; however, five of them also showed prominent myxoid stroma closely resembling myxoid liposarcoma. Immunohistochemistry was performed in selected cases; 4/8 cases tested showed focal positivity for S-100 protein and 5/8 cases showed nuclear positivity for MDM-2. The three cases of myxoid liposarcoma were all negative for MDM2. Both cases of lipoleiomyosarcoma showed positivity for SMA and desmin in the smooth muscle component. FISH was performed in two cases of well-differentiated liposarcoma and high levels of amplification of MDM2 at 12q13-15 were observed; the CHOP translocation at 12q13.1-q13.2 was absent in both cases. Complete surgical excision was performed in 11 cases; however, negative surgical margins were achieved only in four. Clinical follow-up ranging from 1 to 192 months (median 28 months) was available for 13 patients. Two patients with myxoid/round cell liposarcoma died of tumor after 4 months and 3 years, respectively. Both had widely disseminated metastatic disease at the time of death. Six patients (6/10) with well-differentiated liposarcoma were alive and well with no evidence of disease (at 4, 7, 12, 15, and 25 months) and three (3/10) were alive with disease (at 3, 4, and 6 months). One patient with well-differentiated liposarcoma had multiple recurrences and a liver metastasis after 14 years; however, the patient was alive and well at 16 years. Five patients were lost to follow-up. In general, the biologic behavior of liposarcomas in the posterior mediastinum seems to correlate well with the histologic subtype and mirrors that of their counterpart in the retroperitoneum.

Midline skull tumors

Introduction: Variety of tumors could involve the skull; however, very few may occur over the midline. Some may affect venous blood flow of superior sagittal sinus. Few challenging cases are presented Material: 1-Rare case of osteoblastoma over the torcula, (headache, visual symptoms, papilledema, VI nerve palsy). 2- Rare case of metastatic liposarcoma involving midsagital sinus, partially occluding it (headache and visual blurring). 3- A huge atypical (grade2) meningioma over the vertex Method: Case #1, the tumor over the venous confluences (torcula) was removed easily, without any complications. Complete resolution of symptoms Case #2, complete en-block resection of tumor, with sacrifice of mid-sagittal portion of sinus, without any neurological sequellae. Case #3, subtotal resection, followed by radiotherapy.Discussion &amp;Conclusion: Anterior 3rd of sagital sinus could be sacrificed (if necessary), without major consequences. However, whenever mid or posterior portion of the sinus is involved, interruption of venous flow could pose very serious complications. Occasionally, chronic compression of sinus may force increasing collateral venous return, in which case one may attempt a complete resection of the lesion, with sacrifice of part of the sinus, as in our second case. In the region of torcula, however, one should be very careful not to damage it