O lipossarcoma pleomórfico (PLS), o subtipo mais raro de lipossarcoma (LS), é um tumor agressivo e de rápido crescimento localizado geralmente nos tecidos moles profundos das extremidades inferiores e superiores. É caracterizado por um número variável de lipoblastos pleomórficos e, ao contrário do lipossarcoma desdiferenciado, não possui qualquer associação com o lipossarcoma bem diferenciado.
Introdução
O que você precisa saber de cara
O lipossarcoma pleomórfico é um tipo muito raro e de crescimento rápido de câncer dos tecidos profundos, que atinge principalmente adultos. Ele costuma aparecer como um caroço ou massa nos braços ou nas pernas. A confirmação é feita por exame do tecido, pois não há exames genéticos disponíveis para identificar a condição. No momento, esta doença não possui um PCDT oficial nem medicamentos específicos com entrega cadastrada pelo SUS, sendo acompanhada nos centros médicos especializados.
O lipossarcoma pleomórfico (PLS), o subtipo mais raro de lipossarcoma (LS), é um tumor agressivo e de rápido crescimento localizado geralmente nos tecidos moles profundos das extremidades inferiores e superiores. É caracterizado por um número variável de lipoblastos pleomórficos e, ao contrário do lipossarcoma desdiferenciado, não possui qualquer associação com o lipossarcoma bem diferenciado.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 2 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Lipossarcoma pleomórfico
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Surgical Strategies for Closure of Complex Posterior Trunk Defects in Myxoid Pleomorphic Liposarcoma: A Case Report and Literature Review.
Myxoid pleomorphic liposarcoma (MPL) is an exceptionally rare subtype of liposarcoma characterized by an aggressive clinical course. Since its first description in 2009, fewer than a dozen cases have been reported, predominantly in pediatric populations and mediastinal locations. Reports in elderly patients and with posterior trunk involvement are exceedingly uncommon. We report the case of an 83-year-old male with a two-year history of progressive cervical-thoracic swelling, ultimately diagnosed as MPL on biopsy. Imaging revealed an 8.6 × 6.4 × 7.6 cm mass originating from the right cervico-thoracic paravertebral muscles, infiltrating trapezius, rhomboid, subscapularis, and latissimus dorsi. The patient underwent en bloc tumor excision, including paraspinal muscles and partial vertebral elements, followed by immediate reconstruction using regional rotational musculocutaneous flaps (latissimus dorsi and trapezius) performed by a combined oncologic and reconstructive surgical team. Postoperative recovery was uneventful, and the patient was discharged with preserved functional mobility and no wound complications. MPL is recognized for its high recurrence rate and poor survival. Complete surgical resection remains the cornerstone of treatment, particularly when neoadjuvant therapy is not feasible or ineffective. Reconstruction of large posterior trunk defects is technically demanding due to limited tissue elasticity and the scarcity of recipient vessels for free flaps. Regional pedicled options, including trapezius, latissimus dorsi, and paraspinous flaps, remain reliable strategies. This case emphasizes the feasibility of achieving both oncologic clearance and functional coverage in elderly patients with extensive defects. This case highlights the rarity of MPL in advanced age and posterior trunk location, underscoring the critical role of individualized reconstructive planning. Regional pedicled flaps provide safe and effective coverage, ensuring functional preservation and reduced morbidity following radical resection.
Pleomorphic liposarcoma of the breast.
Primary sarcomas of the breast are exceptionally rare, accounting for fewer than 1% of malignant breast lesions. Mammary liposarcomas comprise less than 5% of all breast sarcomas, and among these, primary pleomorphic liposarcoma is particularly uncommon, with a reported frequency of less than 0.01% of malignant breast lesions. It is a highly aggressive tumour, characterised by a high recurrence rate. The characteristic microscopic findings of pleomorphic liposarcoma include atypical lipomatous cells with marked nuclear pleomorphism, as well as abundant lipoblasts, frequent mitoses, and extensive areas of necrosis. In this article, we present a case of primary pleomorphic liposarcoma of the breast.
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Multiple primary malignancies (MPM), defined as two or more distinct primary tumors in 1 patient, are uncommon, with synchronous presentations (diagnosed within 6 months) being rarer. The coexistence of retroperitoneal liposarcoma and a synchronous gastrointestinal malignancy, particularly colorectal adenocarcinoma, is an exceptionally rare clinical entity, posing significant diagnostic and therapeutic challenges. A 63-year-old male presented with fatigue, anorexia, and weight loss. The initial workup revealed microcytic anemia and positive fecal occult blood. While initial endoscopic biopsies were inconclusive, a contrast-enhanced CT scan identified two distinct masses: a large (20 cm) heterogeneous retroperitoneal mass suggestive of liposarcoma and a thickened right colic flexure. The patient underwent a combined en bloc surgical resection, including an extended right hemicolectomy and resection of the retroperitoneal mass with the right kidney. Histopathology confirmed two high-grade primary malignancies: a retroperitoneal pleomorphic liposarcoma (stage IIIb) and a colonic mucinous adenocarcinoma (stage pT3 pN2b). Adjuvant chemotherapy (CAPOX regimen) was initiated for high-risk colon cancer. This case highlights the necessity of a high index of suspicion for synchronous malignancies in patients with non-specific constitutional symptoms. Comprehensive cross-sectional imaging is crucial for accurate staging. Management requires a multidisciplinary approach to plan complex surgical interventions and tailor adjuvant therapy, often prioritizing the more aggressive or higher stage malignancy. This report underscores the complexity of MPM and the need for individualized patient care.
Myxoid Pleomorphic Liposarcoma: A Review and Update.
Myxoid pleomorphic liposarcoma (MPLPS) is an exceedingly rare and recently recognized adipocytic neoplasm that primarily occurs in children and young adults and shows a strong predilection for the mediastinum. Clinically, MPLPS demonstrates aggressive behavior and exhibits a high propensity for systemic spread and a worse overall survival. Some cases have been associated with Li-Fraumeni syndrome. Histologically, MPLPS is composed of a variable mixture of myxoid and pleomorphic liposarcoma-like components. Immunohistochemically, the tumor cells show diffuse expression of CD34 and p16 and loss of nuclear RB expression. MPLPS lacks DNA damage inducible transcript 3 (DDIT3) rearrangements and MDM2 proto-oncogene (MDM2) amplifications but shows tumor protein p53 (TP53) mutations and RB transcriptional co-repressor 1 (RB1) deletions. Moreover, recent studies have demonstrated that the most consistent molecular feature of MPLPS is genome-wide loss of heterozygosity. Surgical excision with negative margins is the mainstay of treatment for localized MPLPS. The treatment of advanced/metastatic MPLPS still poses a huge therapeutic challenge. This review provides information about the clinicoradiological features, pathogenesis, histopathology, and management currently available for MPLPS. In addition, we discuss the differential diagnosis of this novel entity.
Publicações recentes
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Pleomorphic liposarcoma of the breast.
Synchronous Retroperitoneal Pleomorphic Liposarcoma and Colonic Mucinous Adenocarcinoma.
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📚 EuropePMC199 artigos no totalmostrando 198
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Virchows Archiv : an international journal of pathologySurvivorship and prognostic factors for pleomorphic liposarcoma: a population-based study.
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Faculty reviewsPrediction of Histologic Subtype and FNCLCC Grade by SUVmax Measured on 18F-FDG PET/CT in Patients with Retroperitoneal Liposarcoma.
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The American journal of surgical pathologyMediastinal sarcomas: experience using fine needle aspiration cytopathology.
Mediastinum (Hong Kong, China)A Reappraisal of Superficial Pleomorphic Liposarcoma.
American journal of clinical pathologyTumor-associated macrophages and macrophage-related immune checkpoint expression in sarcomas.
Oncoimmunology[Systemic Therapy Options for Locally Advanced and Metastatic Myxoid, Dedifferentiated and Pleomorphic Liposarcoma].
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Ocular oncology and pathologyPrimary anatomical site as a prognostic factor for pleomorphic liposarcoma.
Journal of cancer research and clinical oncologyToxic epidermal necrolysis after radiotherapy for pleomorphic liposarcoma.
Dermatology online journalFrom head and neck lipoma to liposarcoma: a wide spectrum of differential diagnoses and their therapeutic implications.
Current opinion in otolaryngology & head and neck surgeryPleomorphic Liposarcoma of Finger-a Rare Entity.
Indian journal of surgical oncologyRecurrent Pleomorphic Myxoid Liposarcoma in a Patient With Li-Fraumeni Syndrome.
International journal of surgical pathologyWhat's new in adipocytic neoplasia?
Virchows Archiv : an international journal of pathologyDedifferentiated Liposarcoma of the Left Thigh: a Rare Case.
Medical archives (Sarajevo, Bosnia and Herzegovina)Cerebral pleomorphic liposarcoma: Clinicopathologic findings.
Clinical neuropathologyAbnormal vascularization of soft-tissue sarcomas on conventional MRI: Diagnostic and prognostic values.
European journal of radiologyLipomatous Tumors in Pediatric Patients: A Retrospective Analysis of 50 cases.
Turk patoloji dergisiAn atypical pleomorphic lipomatous tumor arising on the cheek.
Journal of cutaneous pathologySonographic Appearances of Liposarcoma: Correlations with Pathologic Subtypes.
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Clinical sarcoma researchCBX3 predicts an unfavorable prognosis and promotes tumorigenesis in osteosarcoma.
Molecular medicine reportsReal-World Experiences with Pazopanib in Patients with Advanced Soft Tissue and Bone Sarcoma in Northern California.
Medical sciences (Basel, Switzerland)A contemporary review of myxoid adipocytic tumors.
Seminars in diagnostic pathologyPleomorphic liposarcoma: Updates and current differential diagnosis.
Seminars in diagnostic pathologyPleomorphic liposarcoma of chest wall: a rare entity with challenging management.
Asian cardiovascular & thoracic annalsHistology driven systemic therapy of liposarcoma-ready for prime time?
Translational gastroenterology and hepatologyPRDM10-rearranged Soft Tissue Tumor: A Clinicopathologic Study of 9 Cases.
The American journal of surgical pathologyCase Report: presentation of pleomorphic liposarcoma arising in a borderline phyllodes tumor.
International journal of surgery case reportsSubclassification of pleomorphic sarcomas: How and why should we care?
Annals of diagnostic pathologyAtypical spindle cell/pleomorphic lipomatous tumor: A clinicopathologic, immunohistochemical, and molecular study of 20 cases.
Pathology internationalFree latissimus dorsi musculocutaneous flap for external hemipelvectomy reconstruction.
MicrosurgeryTrabectedin arrests a doxorubicin-resistant PDGFRA-activated liposarcoma patient-derived orthotopic xenograft (PDOX) nude mouse model.
BMC cancerDoxorubicin-resistant pleomorphic liposarcoma with PDGFRA gene amplification is targeted and regressed by pazopanib in a patient-derived orthotopic xenograft mouse model.
Tissue & cellTumor-targeting Salmonella typhimurium A1-R arrests a doxorubicin-resistant PDGFRA-amplified patient-derived orthotopic xenograft mouse model of pleomorphic liposarcoma.
Journal of cellular biochemistryMetaplastic Breast Cancer Masquerading as Liposarcoma of the Breast: A Case Report following Oncoplastic Treatment.
Pathobiology : journal of immunopathology, molecular and cellular biology[Clinical experience in diagnosis and treatment of primary retroperitoneal liposarcoma].
Zhonghua yi xue za zhiSimultaneous pulmonary and inferior vena cava tumor thromboembolism secondary to retroperitoneal pleomorphic liposarcoma.
Hellenic journal of nuclear medicinePleomorphic liposarcoma: An analysis of 6 case reports and literature review.
MedicinePleomorphic liposarcoma of bone: a rare primary malignant bone tumour.
Clinical sarcoma researchPrimary Pleomorphic Liposarcoma of Fallopian Tube with Recurrence: A Case Report and Review of the Literature.
Open medicine (Warsaw, Poland)Amplification of FRS2 in atypical lipomatous tumour/well-differentiated liposarcoma and de-differentiated liposarcoma: a clinicopathological and genetic study of 146 cases.
HistopathologyClinical and Molecular Spectrum of Liposarcoma.
Journal of clinical oncology : official journal of the American Society of Clinical Oncology[One case report of pleomorphic liposarcoma of larynx].
Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgeryProgrammed Death Ligand 1 (PD-L1) Expression in Malignant Mesenchymal Tumors.
Turk patoloji dergisiEffective apatinib treatment of pleomorphic liposarcoma: A case report.
MedicineA huge primary pleomorphic liposarcoma of the left atrium.
Kardiochirurgia i torakochirurgia polska = Polish journal of cardio-thoracic surgeryPD-1 and PD-L1 expression in bone and soft tissue sarcomas.
Pathology[Relapsed Pleomorphic Liposarcoma with Mediastinal Metastasis: A Case Report and Review of the Literature].
Zhongguo fei ai za zhi = Chinese journal of lung cancerAssociações
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Lipossarcoma pleomórfico e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
É o subtipo mais raro de lipossarcoma, caracterizado por ser um tumor agressivo e de crescimento rápido. Costuma se instalar nos tecidos moles profundos das pernas ou dos braços em adultos.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
5 publicações do grafo RarasNet (PubMed) · 5 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Citar este verbete
Raras. (s.d.). Lipossarcoma pleomórfico. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/lipossarcoma-pleomorfico
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Lipossarcoma pleomórfico
📋 Origem dos dados
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- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- NIH/GARD
- fonte: GARD (NIH)
- Dado público estruturado
- fonte: Wikidata
- Ensaios clínicos
- fonte: ClinicalTrials.gov