Surgical Challenges in a Case of a Recurrent Giant Pleomorphic Liposarcoma of Neck And Mediastinum in a Child: A Rare Occurrence.
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ABSTRACT: Supplementary information
The online version contains supplementary material available at 10.1007/s12070-021-02837-w.
SUBMITTER: Mishra AK
PROVIDER: S-EPMC8396137 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Introduction and importanceRetroperitoneal liposarcomas (RPLS) are usually grow large with frequent recurrences. Complete surgical excision remains the gold standard treatment for primary and even recurrent tumours. Their prognosis depends on their histological type and grade. We report a recurrent giant de-differentiated RPLS weighing 18.55 kg which was completely excised. To the best of our knowledge, this is one of the largest liposarcoma reported in the literature.Case presentationA 40 year old female presented with a gradually progressing large abdominal lump for 1year. She had had a similar large lump twice in the past and undergone excision of the tumour elsewhere. Firm non-tender mass felt all over abdomen with edema noted over abdominal wall and bilateral lower limbs. PET CT showed large heterogeneously enhancing mass occupying almost the entire abdominopelvic cavity. 50 × 40 × 40cm tumour was completely excised and biopsy showed grade 2 dedifferentiated liposarcoma (DDLS). She is under close follow up with no recurrence at 12months.Clinical discussionDDLS have lower risk of distant metastases but have a high risk of local recurrence. The most important favourable prognostic factor in these tumours is complete resection with negative margins. Because of the ineffectiveness of current chemotherapy and the requirement of intolerably high radiation doses, surgical excision remains the most effective treatment even for the localized recurrences of RPLS.ConclusionThe dedifferentiated subtype should be suspected in locally aggressive RPLS. Close follow up with early detection of recurrences and prompt excision with negative margins lowers the risk of recurrences and improves survival.
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Project description:Liposarcoma is a carcinomatous mesenchymal tumor with various histologic features and is the most common soft tissue sarcoma originating in adipose tissue. Liposarcoma commonly occurs in the lower extremities and retroperitoneum but rarely in the mediastinum, specially extending into the thoracic cavity. We report a giant primary liposarcoma of the posterior mediastinum in a 63-year-old female who complained of cough, sputum, and pain in the right chest wall. A computed tomography scan of the chest showed a giant mass of 24 × 15 × 24 cm in the posterior mediastinum of the right thoracic cavity. After a thorough examination, no suspected lipomatous lesions were found elsewhere in the patient's body. The patient underwent a thoracotomy to remove the mediastinal mass through a right thoracic approach. Subsequently, hematoxylin-eosin staining revealed dedifferentiated liposarcoma (DDL), immunohistochemistry showed positive expression of cyclin-dependent kinase 4, and murine double minute 2 (MDM2), in addition, fluorescence in situ hybridization for the MDM2 gene was also positive, which suggested DDL. The patient was discharged without any complications, and no metastasis or recurrence was observed after 19 months of follow-up. To provide a reference for clinical diagnosis and treatment, we reviewed and discussed the literature on primary liposarcoma of the mediastinum.
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Project description:Angiosarcoma of the Posterior Mediastinum is a rare entity. We herein report a case of a giant posterior mediastinal Angiosarcoma. A 54-year female presented with a one-year history of epigastric pain. Her upper gastrointestinal tract endoscopy revealed no abnormality. Chest x-Ray showed a shadow in the hilar area. A computed tomographic scan of the Thorax (CT) and MRI Scan showed a mass in the Posterior Mediastinum. CT-guided biopsy revealed the tumor of vascular origin. The tumor was completely resected. Post-operative recovery was uneventful. After 14 months follow up patient is disease-free, and a CT scan of the chest showed no recurrence.
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Project description:Lipomas are rare in the pediatric age group. A 10-month-old male child presented with an asymptomatic neck mass which was evaluated and excised completely. Histopathology was consistent with pleomorphic lipoma, not previously reported in children.
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Project description:BackgroundPleomorphic liposarcoma is the least common but most aggressive subtype of liposarcoma. Very few studies have presented data on pleomorphic liposarcoma specifically, often including a limited number of cases and short-term follow-up. As a result, the survivorship and prognostic characteristics of this tumor remain incompletely identified.Study design and settingCross-sectional analysis of the Surveillance Epidemiology and End Results database (1996-2015).ResultsOverall survival for the entire series was 54% (95% confidence interval [CI], 49-58%) and 40% (95% CI, 35-45%) at 5 and 10 years, respectively. Disease-specific survival for the entire series was 60% (95% CI, 56-65%) and 53% (95% CI, 48-58%) at 5 and 10 years, respectively. Patients who survived 10 years or more were more likely to die of events unrelated to pleomorphic liposarcoma. Univariate and multivariate analysis demonstrated that not receiving cancer-directed surgery was an independent poor prognostic factor. Older age (≥ 65 years old) was associated with worse overall survival but not disease-specific survival. Tumor stage and radiotherapy showed different impact on survival depending on tumor size. In comparison to localized staged tumors, regional stage only predicts poor survival in patients with tumor size less than 5 cm, while distant stage is an independent worse prognosis factor. Radiotherapy only benefits patients with tumor size larger than 10 cm. These results were confirmed in competing risk analysis.ConclusionSurvival rates of patients with pleomorphic liposarcoma has not changed over the past 20 years. Patients with distant stage have poor prognosis; regional stage indicates worse survival in patients with tumor size less than 5 cm. Receiving surgery could prolong the survival, while radiotherapy only benefits patients with large tumor size (> 10 cm). Older age is associated with poor overall survival but not disease-specific survival. Routine patient surveillance following initial diagnosis should at least be 10 years for pleomorphic liposarcoma.
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Project description:Background and Objective The posterior mediastinum is a potential space along the paravertebral sulci or between the posterior aspect of the pericardium and the vertebrae. This compartment is classically the most frequent location site of neurogenic tumors. Whereas neurofibroma and schwannoma are neurogenic tumors that commonly arise from peripheral nerves, sympathetic nerves are the origin of ganglioneuroma, neuroblastoma, ganglioneuroblastoma, and neuroectodermal cells closely associated with autonomic nerves are the origin of paragangliomas and pheochromocytomas. Additionally, tumors from the esophagus, tumors of mesenchymal origin, lymphoma, ectopic goiter, and diseases with lymph node hyperplasia may also settle on this compartment. The objectives are to identify term “giant posterior mediastinal tumor” and the etiology, clinical features, diagnostic methods, pathological types, surgical methods applied, and technical details of these methods for the treatment of these tumors. Methods In this review, the term “giant tumor” was used to define tumors larger than 10 cm settled in the posterior mediastinum. PubMed database was searched with keywords “posterior mediastinum, giant mass” or “posterior mediastinum, tumor, giant” limited to English language and full-text available studies published between years 1984–2021. Key Content and Findings As a result of the literature review with the relevant terms, 23 case reports were found in accordance with the inclusion criteria. We detected the most common giant posterior mediastinum tumors were neurogenic origin (schwannoma, ganglioneuroma, ganglioneuroblastoma, triton tumor) in that review. The most common surgical approach was posterolateral thoracotomy. Treatment response to surgical total excision was good in most of cases. Conclusions The definitive diagnosis and treatment of giant posterior mediastinal tumors is made by surgical excision. Diagnostic procedures and subsequent surgical planning may vary depending on the origin and localization. Adjuvant treatment and follow-up should be conducted on the histopathological features.
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Project description:Liposarcoma of the spermatic cord (LSC) is a very rare disease. In literature, are reported less than 350 cases. Genitourinary sarcomas account for <5% of all soft-tissue sarcomas and <2% of malignant urologic tumours. An inguinal mass is the clinical presentation, which can mimic a hernia or hydrocele. Since it is such a rare disease, there are insufficient data on chemotherapy and radiotherapy, and in any case, the data come from low-level scientific evidence. Here, we report the case of a patient who came to the observation for a giant inguinal mass, in which a definitive diagnosis was obtained with the histological examination.
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Project description:The mediastinum is a unique anatomic area containing various structures and pluripotent cells, which allow for the development of a range of tumors. We report two pediatric cases of a lipomatous tumor of the posterior mediastinum. Complete surgical excision of the mass in each was achieved through a lateral thoracotomy. Histopathologic findings showed a lipoma in one case and a lipoblastoma in the other. A lipomatous tumor in the posterior mediastinum is extremely rare and preoperative diagnosis is difficult. When an operative plan is being considered, the presence of a potentially malignant tumor should be kept in mind.
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