RESUMEN
AIMS: Bone cancer produces severe pain that is treated with opioids, but serious side effects limit opioid utilization. There is therefore a need to develop effective and safe non-opioid alternatives. The lipid mediator, Resolvin D1 (RvD1), could be a prospective candidate for cancer pain treatment. To assess RvD1 and other potential candidates, appropriate animal models that recapitulate clinical features must be used. Although several preclinical models of cancer pain have been developed, the influence of sex on the development of cancer pain and the effectiveness of RvD1 have not been studied. RESULTS: Using a mouse model of fibrosarcoma growth in and around the calcaneus bone, we demonstrated that the mechanical hyperalgesia in the tumor-bearing hind paw develops independently of sex, except that it developed a little sooner in female mice. A single intravenous injection of RvD1 (0.001-10 µg/kg) decreased hyperalgesia in both sexes with similar potency (ED50 = 0.0015 µg/kg) and efficacy. Repeated daily administration of 10 µg/kg RvD1 prolonged the analgesic effect and completely abolished hyperalgesia. This was also independent of sex. CONCLUSION: In this preclinical mouse model of bone cancer pain, the development of pain and the analgesic effectiveness of RvD1 are not influenced by sex.
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Neoplasias Óseas , Dolor en Cáncer , Modelos Animales de Enfermedad , Ácidos Docosahexaenoicos , Hiperalgesia , Animales , Femenino , Neoplasias Óseas/tratamiento farmacológico , Neoplasias Óseas/complicaciones , Neoplasias Óseas/secundario , Masculino , Dolor en Cáncer/tratamiento farmacológico , Dolor en Cáncer/etiología , Ácidos Docosahexaenoicos/administración & dosificación , Ácidos Docosahexaenoicos/farmacología , Ratones , Hiperalgesia/tratamiento farmacológico , Hiperalgesia/etiología , Analgésicos/farmacología , Analgésicos/administración & dosificación , Fibrosarcoma/tratamiento farmacológico , Fibrosarcoma/patología , Fibrosarcoma/complicaciones , Factores Sexuales , Dimensión del DolorRESUMEN
For many cancer patients tumor burden negatively impacts quality of life due to associated pain onset. Neuropathic pain is commonly associated with late cancer stages, and is resultant of tumor metastasis to bone, herein referred to as cancer-induced bone pain. Given the severe impact on quality of life and clinical treatment strategies focusing on symptom management, novel therapeutics are needed to alleviate cancer-induced bone pain and/or reduce cancer burden. In the current study we characterized a commercially available murine fibrosarcoma cell line, NCTC-2472 in vitro, which can be used to assess the capacity of novel compounds to impact cellular viability. We found that dimethyl sulfoxide, a known cytotoxic agent and common drug preparation compound, significantly decreased cell viability in a dose-related manner. We then characterized the in vivo tumor development and associated pain behavior characteristics following implantation of NCTC-2472 fibrosarcoma into male and female C3H/HeJ mice. The C3H/HeJ strain was utilized as these mice are syngeneic with NCTC-2472 fibrosarcoma and their use reduces potential implantation failure. We found that tumor development in mice resulted in the development of mechanical allodynia but not thermal hyperalgesia. Gabapentin, a clinically relevant analgesic, produced dose-related mechanical allodynia reversal. These studies provide further characterization of a cancer-induced bone pain model that can be used to examine novel compounds as anti-cancer and analgesic therapeutics.
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Neoplasias Óseas , Dolor en Cáncer , Fibrosarcoma , Ratones Endogámicos C3H , Animales , Fibrosarcoma/patología , Fibrosarcoma/tratamiento farmacológico , Fibrosarcoma/complicaciones , Neoplasias Óseas/secundario , Neoplasias Óseas/complicaciones , Neoplasias Óseas/tratamiento farmacológico , Neoplasias Óseas/patología , Línea Celular Tumoral , Ratones , Femenino , Dolor en Cáncer/tratamiento farmacológico , Dolor en Cáncer/etiología , Masculino , Supervivencia Celular/efectos de los fármacos , Gabapentina/farmacología , Dimetilsulfóxido/farmacología , Modelos Animales de Enfermedad , Ácido gamma-Aminobutírico/farmacología , Aminas/química , Aminas/farmacología , Analgésicos/farmacologíaRESUMEN
BACKGROUND: Sclerosing epithelioid fibrosarcoma is an aggressive sarcoma subtype with poor prognosis and limited response to conventional chemotherapy regimens. Diagnosis can be difficult owing to its variable presentation, and cases of sclerosing epithelioid fibrosarcoma are rare. Sclerosing epithelioid fibrosarcoma typically affects middle-aged individuals, with studies inconsistently citing gender predominance. Sclerosing epithelioid fibrosarcoma typically arises from the bones and soft tissues and often has local recurrence after resection and late metastases. Immunohistochemical staining typically is positive for mucin-4. Werner syndrome is due to an autosomal recessive mutation in the WRN gene and predisposes patients to malignancy. CASE PRESENTATION: A 37-year-old Caucasian female presented to the emergency department with 4 months of dyspnea and back pain. She had been treated for pneumonia but had persistent symptoms. A chest, abdomen, and pelvis computed tomography showed near-complete right upper lobe collapse and consolidation, mediastinal lymphadenopathy, lytic spinal lesions, and a single 15-mm hypodense liver nodule. The patient underwent a transthoracic right upper lobe biopsy, bronchoscopy, endobronchial ultrasound with transbronchial lymph node sampling, and bronchoalveolar lavage of the right upper lobe. The bronchoalveolar lavage cytology was positive for malignant cells compatible with poorly differentiated non-small cell carcinoma; however, the cell block materials were insufficient to run immunostains for further investigation of the bronchoalveolar lavage results. Consequently, the patient also underwent a liver biopsy of the liver nodule, which later confirmed a diagnosis of sclerosing epithelioid fibrosarcoma. Next-generation sequencing revealed a variant of unknown significance in the WRN gene. She was subsequently started on doxorubicin. CONCLUSION: Sclerosing epithelioid fibrosarcoma is a very rare entity, only cited approximately 100 times in literature to date. Physicians should be aware of this disease entity and consider it in their differential diagnosis. Though pulmonary involvement has been described in the context of sclerosing epithelioid fibrosarcoma, this malignancy may affect many organ systems, warranting extensive investigation. Through our diagnostic workup, we suggest a possible link between sclerosing epithelioid fibrosarcoma and the WRN gene. Further study is needed to advance our understanding of sclerosing epithelioid fibrosarcoma and its clinical associations as it is an exceedingly rare diagnosis.
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Fibrosarcoma , Fracturas Espontáneas , Traumatismos del Cuello , Sarcoma , Fracturas de la Columna Vertebral , Síndrome de Werner , Persona de Mediana Edad , Humanos , Femenino , Adulto , Fibrosarcoma/complicaciones , Fibrosarcoma/diagnóstico , Fibrosarcoma/genética , Tomografía Computarizada por Rayos X , Disnea , Helicasa del Síndrome de WernerRESUMEN
Neuropathic pain associated with cancers is caused by tumor growth compressing and damaging nerves, which would also be enhanced by inflammatory factors through sensitizing nociceptor neurons. A troublesome hallmark symptom of neuropathic pain is hypersensitivity to innocuous stimuli, a condition known as "tactile allodynia", which is often refractory to NSAIDs and opioids. The involvement of chemokine CCL2 (monocyte chemoattractant protein-1) in cancer-evoked neuropathic pain is well established, but opinions remain divided as to whether CCL2 is involved in the production of tactile allodynia with tumor growth. In this study, we constructed Ccl2 knockout NCTC 2472 (Ccl2-KO NCTC) fibrosarcoma cells and conducted pain behavioral test using Ccl2-KO NCTC-implanted mice. Implantation of naïve NCTC cells around the sciatic nerves of mice produced tactile allodynia in the inoculated paw. Although the growth of Ccl2 KO NCTC-formed tumors was comparable to that of naïve NCTC-formed tumors, Ccl2-KO NCTC-bearing mice failed to show tactile pain hypersensitivity, suggesting the involvement of CCL2 in cancer-induced allodynia. Subcutaneous administration of controlled-release nanoparticles containing the CCL2 expression inhibitor NS-3-008 (1-benzyl-3-hexylguanidine) significantly attenuated tactile allodynia in naïve NCTC-bearing mice accompanied by a reduction of CCL2 content in tumor masses. Our present findings suggest that inhibition of CCL2 expression in cancer cells is a useful strategy to attenuate tactile allodynia induced by tumor growth. Development of a controlled-release system of CCL2 expression inhibitor may be a preventative option for the treatment of cancer-evoked neuropathic pain. SIGNIFICANCE STATEMENT: The blockade of chemokine/receptor signaling, particularly for C-C motif chemokine ligand 2 (CCL2) and its high-affinity receptor C-C chemokine receptor type 2 (CCR2), has been implicated to attenuate cancer-induced inflammatory and nociceptive pain. This study demonstrated that continuous inhibition of CCL2 production from cancer cells also prevents the development of tactile allodynia associated with tumor growth. Development of a controlled-release system of CCL2 expression inhibitor may be a preventative option for management of cancer-evoked tactile allodynia.
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Fibrosarcoma , Neuralgia , Animales , Ratones , Quimiocina CCL2/metabolismo , Quimiocina CCL2/uso terapéutico , Preparaciones de Acción Retardada , Fibrosarcoma/complicaciones , Fibrosarcoma/tratamiento farmacológico , Hiperalgesia/tratamiento farmacológico , Hiperalgesia/etiología , Hiperalgesia/metabolismo , Ligandos , Neuralgia/tratamiento farmacológicoRESUMEN
BACKGROUND: Cancer-associated cachexia (CAC) is a wasting syndrome drastically reducing efficacy of chemotherapy and life expectancy of patients. CAC affects up to 80% of cancer patients, yet the mechanisms underlying the disease are not well understood and no approved disease-specific medication exists. As a multiorgan disorder, CAC can only be studied on an organismal level. To cover the diverse aetiologies of CAC, researchers rely on the availability of a multifaceted pool of cancer models with varying degrees of cachexia symptoms. So far, no tumour model syngeneic to C57BL/6 mice exists that allows direct comparison between cachexigenic- and non-cachexigenic tumours. METHODS: MCA207 and CHX207 fibrosarcoma cells were intramuscularly implanted into male or female, 10-11-week-old C57BL/6J mice. Tumour tissues were subjected to magnetic resonance imaging, immunohistochemical-, and transcriptomic analysis. Mice were analysed for tumour growth, body weight and -composition, food- and water intake, locomotor activity, O2 consumption, CO2 production, circulating blood cells, metabolites, and tumourkines. Mice were sacrificed with same tumour weights in all groups. Adipose tissues were examined using high-resolution respirometry, lipolysis measurements in vitro and ex vivo, and radioactive tracer studies in vivo. Gene expression was determined in adipose- and muscle tissues by quantitative PCR and Western blotting analyses. Muscles and cultured myotubes were analysed histologically and by immunofluorescence microscopy for myofibre cross sectional area and myofibre diameter, respectively. Interleukin-6 (Il-6) was deleted from cancer cells using CRISPR/Cas9 mediated gene editing. RESULTS: CHX207, but not MCA207-tumour-bearing mice exhibited major clinical features of CAC, including systemic inflammation, increased plasma IL-6 concentrations (190 pg/mL, P ≤ 0.0001), increased energy expenditure (+28%, P ≤ 0.01), adipose tissue loss (-47%, P ≤ 0.0001), skeletal muscle wasting (-18%, P ≤ 0.001), and body weight reduction (-13%, P ≤ 0.01) 13 days after cancer cell inoculation. Adipose tissue loss resulted from reduced lipid uptake and -synthesis combined with increased lipolysis but was not associated with elevated beta-adrenergic signalling or adipose tissue browning. Muscle atrophy was evident by reduced myofibre cross sectional area (-21.8%, P ≤ 0.001), increased catabolic- and reduced anabolic signalling. Deletion of IL-6 from CHX207 cancer cells completely protected CHX207IL6KO -tumour-bearing mice from CAC. CONCLUSIONS: In this study, we present CHX207 fibrosarcoma cells as a novel tool to investigate the mediators and metabolic consequences of CAC in C57BL/6 mice in comparison to non-cachectic MCA207-tumour-bearing mice. IL-6 represents an essential trigger for CAC development in CHX207-tumour-bearing mice.
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Caquexia , Interleucina-6 , Neoplasias , Animales , Femenino , Masculino , Ratones , Tejido Adiposo/patología , Caquexia/patología , Fibrosarcoma/complicaciones , Interleucina-6/metabolismo , Ratones Endogámicos C57BL , Fibras Musculares Esqueléticas/metabolismo , Atrofia Muscular/patología , Neoplasias/complicacionesRESUMEN
Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a rare soft tissue sarcoma in childhood. We present the case of a newborn male who experienced a severe hemorrhage in utero from the tumor on the scalp. He died at the age of 24 hours owing to hemorrhagic shock. The tumor was posthumously diagnosed as PMMTI. A literature search indicated that cases of severe hemorrhage from soft tissue sarcomas in utero or at birth are limited to infantile fibrosarcoma. This is the first case of PMMTI with massive hemorrhage. Clinicians must be aware of hemorrhagic complications of PMMTI.
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Fibrosarcoma , Sarcoma , Neoplasias de los Tejidos Blandos , Recién Nacido , Humanos , Lactante , Masculino , Fibrosarcoma/complicaciones , Fibrosarcoma/patología , Sarcoma/patología , Neoplasias de los Tejidos Blandos/complicaciones , Neoplasias de los Tejidos Blandos/patología , Hemorragia/etiologíaRESUMEN
BACKGROUND: Solitary fibrous tumors of the pleura are rare diseases of the thoracic cavity. They frequently grow unnoticed until they exert compressive effects on adjacent organs. Treatment of solitary fibrous tumors of the pleura is surgical resection. Post-operative surveillance is recommended to identify early recurrent disease. CASE PRESENTATION: We present a rare case of a 76-year-old female patient with no previous pulmonary history who presented with progressive dyspnea, fatigue, and involuntary weight loss. On chest X-ray and computed chest tomography scan, she was found to have a 16.7 cm × 12.8 cm × 10.1 cm bulky mass occupying the left hemithorax with associated compressive atelectasis of the lung. She underwent a computed tomography guided biopsy that revealed the mass to be a solitary fibrous tumor. The patient underwent left muscle sparing lateral thoracotomy with complete resection of the tumor. Post procedure, the left lung fully expanded. 18 months post-resection, she developed a 3.3 cm × 1.7 cm tumor along the left internal thoracic artery lymph node chain which was histologically identical to the resected tumor. The patient is currently being treated with bevacizumab and temozolomide. CONCLUSION: Solitary fibrous tumors are very rare pleural tumors. Surgical resection is the treatment of choice followed by close post-operative surveillance.
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Fibrosarcoma , Neoplasias Pleurales , Tumor Fibroso Solitario Pleural , Cavidad Torácica , Anciano , Femenino , Fibrosarcoma/complicaciones , Humanos , Pleura/patología , Pleura/cirugía , Neoplasias Pleurales/diagnóstico , Neoplasias Pleurales/cirugía , Tumor Fibroso Solitario Pleural/diagnóstico por imagen , Tumor Fibroso Solitario Pleural/cirugía , Cavidad Torácica/patologíaRESUMEN
We report the first case of a patient with myxofibrosarcoma (MFS) who presented acutely with a rib fracture and developed a rapidly expanding loculated hemothorax after chest trauma. The patient was taken to the operating room for evacuation of hemothorax, and samples and biopsy specimens were taken for cytologic and pathologic examination. Final report with immunohistochemical staining showed a high-grade MFS. After the procedure, there was clinical and radiological improvement, and the patient was followed up as an outpatient. Myxofibrosarcoma is a very rare and aggressive connective tissue neoplasm with variable presentations. Surgical resection is the preferred treatment. Prompt diagnosis and adequate management of these tumors are important to reduce their high local recurrence and distant metastasis rates. Therefore, it is important to be aware of its common and uncommon presentations.
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Accidentes por Caídas , Fibrosarcoma/diagnóstico por imagen , Hemotórax/diagnóstico por imagen , Mixosarcoma/diagnóstico por imagen , Fracturas de las Costillas/diagnóstico por imagen , Pared Torácica/diagnóstico por imagen , Anciano , Fibrosarcoma/complicaciones , Fibrosarcoma/patología , Fibrosarcoma/cirugía , Fracturas Espontáneas/diagnóstico por imagen , Fracturas Espontáneas/etiología , Fracturas Espontáneas/terapia , Hemotórax/etiología , Hemotórax/terapia , Humanos , Masculino , Mixosarcoma/complicaciones , Mixosarcoma/patología , Mixosarcoma/cirugía , Clasificación del Tumor , Fracturas de las Costillas/etiología , Fracturas de las Costillas/terapia , Traumatismos Torácicos , Pared Torácica/patología , Pared Torácica/cirugíaRESUMEN
BACKGROUND/AIM: Dermat of ibrosarcoma protuberans (DFSP) is a soft-tissue sarcoma with a high risk of local recurrence, though typically never metastasizes. DFSP can transform into high-grade fibrosarcoma (DFSP-FS), which has a risk of metastasis. Currently, treatment for DFSP includes Moh's micrographic surgery (MMS); however, this is not recommended for DFSP-FS. Often, the transformation to DFSP-FS is not recognized until the final histological diagnosis. At that point, wide local excision (WLE) of a previous MMS site can be morbid. As such, we analyzed patient risk factors to allow identification of DFSP-FS transformation at presentation. PATIENTS AND METHODS: We reviewed 368 (174 female, 194 male) patients with a mean age of 42 years from two sarcoma centers. A total of 319 (87%) patients had a history of DFSP and 49 (13%) had DFSP-FS. RESULTS: When comparing patients with a DFSP to those with a DFSP-FS, patients with a DFSP-FS were more likely (p<0.05) to be older, female and with larger tumors. A painful mass and rapidly enlarging mass were associated with DFSP-FS. CONCLUSION: Patients who presented with DFSP-FS were found to typically have a larger, painful, and growing mass. Patients with these features should be referred for WLE over MMS at presentation.
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Dermatofibrosarcoma/etiología , Fibrosarcoma/complicaciones , Adulto , Dermatofibrosarcoma/patología , Femenino , Humanos , Masculino , Periodo Preoperatorio , Factores de RiesgoRESUMEN
Infantile fibrosarcoma (IFS) is a rare pediatric tumor which often presents the ETV6-NTRK3 gene fusion. NTRK3 encodes the neurotrophin-3 growth factor receptor tyrosine kinase, a druggable therapeutic target. Selective tropomyosin receptor kinase (TRK) inhibitors, such as larotrectinib, have shown efficacy and safety in the treatment of IFS. We report a case of an abdominal IFS diagnosed in a newborn associated with an aortic aneurysm that was successfully treated with larotrectinib without relevant adverse effects.
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Neoplasias Abdominales/tratamiento farmacológico , Aneurisma de la Aorta Abdominal/complicaciones , Fibrosarcoma/tratamiento farmacológico , Inhibidores de Proteínas Quinasas/uso terapéutico , Pirazoles/uso terapéutico , Pirimidinas/uso terapéutico , Neoplasias Abdominales/complicaciones , Neoplasias Abdominales/diagnóstico , Femenino , Fibrosarcoma/complicaciones , Fibrosarcoma/diagnóstico , Humanos , Lactante , Recién NacidoRESUMEN
BACKGROUND: Cancer cachexia is a complex metabolic disease with unmet medical need. Although many rodent models are available, none are identical to the human disease. Therefore, the development of new preclinical models that simulate some of the physiological, biochemical, and clinical characteristics of the human disease is valuable. The HT-1080 human fibrosarcoma tumour cell line was reported to induce cachexia in mice. Therefore, the purpose of this work was to determine how well the HT-1080 tumour model could recapitulate human cachexia and to examine its technical performance. Furthermore, the efficacy of ghrelin receptor activation via anamorelin treatment was evaluated, because it is one of few clinically validated mechanisms. METHODS: Female severe combined immunodeficient mice were implanted subcutaneously or heterotopically (renal capsule) with HT-1080 tumour cells. The cachectic phenotype was evaluated during tumour development, including body weight, body composition, food intake, muscle function (force and fatigue), grip strength, and physical activity measurements. Heterotopic and subcutaneous tumour histology was also compared. Energy balance was evaluated at standard and thermoneutral housing temperatures in the subcutaneous model. The effect of anamorelin (ghrelin analogue) treatment was also examined. RESULTS: The HT-1080 tumour model had excellent technical performance and was reproducible across multiple experimental conditions. Heterotopic and subcutaneous tumour cell implantation resulted in similar cachexia phenotypes independent of housing temperature. Tumour weight and histology was comparable between both routes of administration with minimal inflammation. Subcutaneous HT-1080 tumour-bearing mice presented with weight loss (decreased fat mass and skeletal muscle mass/fibre cross-sectional area), reduced food intake, impaired muscle function (reduced force and grip strength), and decreased spontaneous activity and voluntary wheel running. Key circulating inflammatory biomarkers were produced by the tumour, including growth differentiation factor 15, Activin A, interleukin 6, and TNF alpha. Anamorelin prevented but did not reverse anorexia and weight loss in the subcutaneous model. CONCLUSIONS: The subcutaneous HT-1080 tumour model displays many of the perturbations of energy balance and physical performance described in human cachexia, consistent with the production of key inflammatory factors. Anamorelin was most effective when administered early in disease progression. The HT-1080 tumour model is valuable for studying potential therapeutic targets for the treatment of cachexia.
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Caquexia , Fibrosarcoma , Animales , Anorexia , Caquexia/etiología , Modelos Animales de Enfermedad , Femenino , Fibrosarcoma/complicaciones , Humanos , Ratones , Actividad MotoraAsunto(s)
Fibrosarcoma/patología , Neoplasias del Mediastino/patología , Neoplasias del Timo/patología , Terapia Combinada , Fibrosarcoma/complicaciones , Fibrosarcoma/terapia , Humanos , Lactante , Masculino , Neoplasias del Mediastino/complicaciones , Neoplasias del Mediastino/terapia , Terapia Neoadyuvante , Pronóstico , Neoplasias del Timo/complicaciones , Neoplasias del Timo/terapiaRESUMEN
BACKGROUND: Although congenital infantile fibrosarcoma (cIFS) is a rare soft tissue sarcoma among children, it constitutes one of the most common soft tissue sarcomas during the first year of life. Congenital mesoblastic nephroma (CMN) is the most common benign renal tumor usually developing during the first 3 months of life. cIFS and cellular type CMN (cCMN) share not only similar histopathologic features but identical molecular genetic abnormality including the ETV6/NTRK3 fusion gene. Here, we report an unusual case of cIFS occurring with cCMN. CASE PRESENTATION: An 18-month-old girl presented with a 1-month history of abdominal distension and a few days' history of a palpable abdominal mass. A large heterogenous mass sized 9.0×11.2×11.6 cm on the right side of the abdomen and an isolated heterogenous lesion sized 4×4.5 cm within the right kidney were noted from the imaging study. Pathologic findings were consistent with cIFS and cCMN of the right kidney. In addition, both pathologic specimens contained the ETV6/NTRK3 fusion gene. CONCLUSION: Although cIFS and cCMN share similar histopathologic features and molecular genetic abnormality, simultaneous occurrence of these 2 types of tumor is exceedingly rare. To our knowledge, this is the first unusual case report of concurrent cIFS and cCMN.
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Fibrosarcoma/patología , Nefroma Mesoblástico/patología , Neoplasias Retroperitoneales/patología , Femenino , Fibrosarcoma/complicaciones , Fibrosarcoma/congénito , Humanos , Lactante , Nefroma Mesoblástico/complicaciones , Nefroma Mesoblástico/congénito , Pronóstico , Neoplasias Retroperitoneales/complicaciones , Neoplasias Retroperitoneales/congénitoRESUMEN
An 18-year-old female presented with rapidly progressive proptosis of the left eye for one month and grade II relative afferent pupillary defect. Orbital imaging showed a well-defined homogenous extraconal mass in close relation to the lateral rectus muscle and extending up to the superior orbital fissure, associated with bony erosion. An incisional biopsy was performed, with the histopathology demonstrating stellate to spindle-shaped tumor cells (fibroblasts) embedded in a richly myxoid matrix. A diagnosis of low-grade fibromyxoid sarcoma (LGFS) was made. The patient was treated by stereotactic external beam radiotherapy. Here, we report a case of LGFS which, to the best of our knowledge, is the first at an orbital location.
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Fibrosarcoma/diagnóstico , Neoplasias Orbitales/diagnóstico , Adolescente , Biopsia , Diagnóstico Diferencial , Exoftalmia/diagnóstico , Exoftalmia/etiología , Femenino , Fibrosarcoma/complicaciones , Fibrosarcoma/radioterapia , Humanos , Órbita/diagnóstico por imagen , Neoplasias Orbitales/complicaciones , Neoplasias Orbitales/radioterapia , Tomografía Computarizada por Rayos XRESUMEN
The purpose of this study was to analyse the characteristics of 6 patients managed in a university hospital between 1996 and 2016 for non-islet cell tumor hypoglycemia (NICTH), a form of hypoglycaemia due to the paraneoplastic secretion of IGF-2 or its related substances. RESULTS: Three of these 6 patients (50%), aged over 69 years, including 2 with acromegaloid phenotype, presented with a pleural solitary fibrous tumor (SFT), with median diameter 20 cm (interquartile range, 12.5-20.5) with a low median SUV (3.3 g/mL (QR, 2-7.5)) on 18F-FDG PET. The other 3 patients presented respectively neuroendocrine carcinoma (NEC) of the palate (70-year-old woman), retroperitoneal myxofibrosarcoma (66-year-old man) and meningeal hemangiopericytoma (36-year-old woman). All 3 were inoperable and did not respond to any therapy other than glucose solution. Corticosteroid therapy was effective in the 3 SFTs and the NEC. One of the SFTs recurred 10 years later with asymptomatic hypoglycemia, which resolved after reintervention. Median (IQR) blood glucose levels of the 6 patients was 0.4g/L (QR, 0.31-0.41), with hypoinsulinemia at 0.7mIU/L (QR 0.7-2.0), undetectable GH, low IGF-1, normal IGF-2 level in 5/6 cases, a high IGF-2:IGF-1 ratio at 26.9 (QR, 20.8-37.8), hypokalemia and hypomagnesemia. CONCLUSION: NICTH is a rare syndrome, which should be considered in the presence of hypoinsulinemic hypoglycemia with low GH and IGF-1, and a IGF-2:IGF-1 ratio>10. Corticosteroid therapy was effective in elderly subjects, particularly with solitary fibrous tumor, which was generally operable. Hemangiopericytoma and myxofibrosarcoma had poor prognosis in younger patients.
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Hipoglucemia/etiología , Tumores Neuroendocrinos/complicaciones , Tumor Fibroso Solitario Pleural/complicaciones , Adulto , Anciano , Glucemia/análisis , Femenino , Fibroma , Fibrosarcoma/sangre , Fibrosarcoma/complicaciones , Hemangiopericitoma/sangre , Hemangiopericitoma/complicaciones , Hospitales Universitarios , Hormona de Crecimiento Humana/sangre , Humanos , Hipoglucemia/sangre , Hipoglucemia/tratamiento farmacológico , Factor I del Crecimiento Similar a la Insulina/análisis , Factor II del Crecimiento Similar a la Insulina/análisis , Magnesio/sangre , Masculino , Neoplasias Meníngeas/sangre , Neoplasias Meníngeas/complicaciones , Tumores Neuroendocrinos/sangre , Potasio/sangre , Pronóstico , Neoplasias Retroperitoneales/sangre , Neoplasias Retroperitoneales/complicaciones , Tumor Fibroso Solitario Pleural/sangreRESUMEN
BACKGROUND: In both humans and animals, cardiac fibrosarcoma is rare among primary cardiac malignant neoplasia. The overall prevalence of cardiac neoplasia in dogs is low, reported to be between 0.17% and 0.19% of hospital admissions. The aim of this report is to describe the clinical and pathological findings of a dog presenting signs of right sided congestive heart failure due to an intracardiac and venous obstructing mass, diagnosed by histopathology as cardiac fibrosarcoma with myxoid features. CASE PRESENTATION: A 7 years old male mix breed Husky weighing 23 kg was presented to our Veterinary Teaching Hospital the owner reporting weight loss, inappetence and exercise intolerance and on presentation exhibited breathlessness and an enlarged abdomen. A 5 minutes six leads electrocardiogram and cardiac ultrasonography were performed using standard, established techniques. Complete blood count, serum liver enzyme activities and renal parameters were assessed. Shortly after the cardiologic examination, the dog died and necropsy examination of the cardiovascular system revealed an elongated and branched mass attached dorsally to the endocardial insertion of the septal tricuspid valve leaflet. This mass extended retrogradely into the lumen of the cervical veins, obstructing the venous flow. Histological diagnosis of the mass was cardiac fibrosarcoma with myxoid features. Multiple metastases were found inside the lungs only. CONCLUSION: This is the first report describing a right cardiac fibrosarcoma with myxoid features and venous obstruction in a dog. Cardiac fibrosarcoma is a rare finding, however should be considered when an intracardiac mass is diagnosed.
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Fibrosarcoma/veterinaria , Insuficiencia Cardíaca/veterinaria , Neoplasias Cardíacas/veterinaria , Animales , Perros , Resultado Fatal , Fibrosarcoma/complicaciones , Fibrosarcoma/diagnóstico , Fibrosarcoma/patología , Insuficiencia Cardíaca/etiología , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/patología , MasculinoAsunto(s)
Fibrosarcoma/diagnóstico , Neoplasias Pulmonares/diagnóstico , Neoplasias Primarias Múltiples/diagnóstico , Adolescente , Tos/etiología , Diagnóstico Diferencial , Disnea/etiología , Femenino , Fibrosarcoma/complicaciones , Fibrosarcoma/diagnóstico por imagen , Fibrosarcoma/cirugía , Humanos , Neoplasias Pulmonares/complicaciones , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/cirugía , Neoplasias Primarias Múltiples/complicaciones , Neoplasias Primarias Múltiples/diagnóstico por imagen , Neoplasias Primarias Múltiples/cirugía , Neumonectomía , Tomografía Computarizada por Rayos XRESUMEN
A two-year-old boy presented with a large, non-healing ulceration on his left buttock, which was originally noted as a brown patch present at birth. Punch skin biopsy was performed and histopathology revealed an atypical, pleomorphic, spindled proliferation in whorled fascicles replacing the dermis and trapping fat in the subcutis, consistent with a diagnosis of congenital/infantile fibrosarcoma. No evidence of metastatic spread was seen on imaging. The tumor was initially deemed unresectable owing to extent of local invasion. Neo-adjuvant chemotherapy caused significant tumor shrinkage and the patient underwent complete resection.
Asunto(s)
Fibrosarcoma/congénito , Neoplasias Cutáneas/congénito , Úlcera Cutánea/etiología , Nalgas , Preescolar , Fibrosarcoma/complicaciones , Fibrosarcoma/patología , Humanos , Masculino , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/patología , Úlcera Cutánea/patologíaRESUMEN
We report a case of low-grade fibromyxoid sarcoma arising within the median nerve. A 31-year-old woman presented with symptoms of carpal tunnel syndrome and an enlarging mass in her right palm over 1 year. MRI demonstrated a mass associated with the right median nerve with solid and cystic components. At surgery, the mass was located within the epineurium, could be bluntly dissected from the nerve fascicles, and was suspected to be a schwannoma. A 3.4 cm, tan-pink, glistening, smooth, homogenous mass was submitted to pathology. Microscopically, the tumor was a solid and cystic circumscribed nodule with a dense fibrous pseudocapsule. The tumor cells were uniformly bland and spindle-shaped, with small, hyperchromatic oval nuclei and were embedded in an alternating fibrous and myxoid stroma with a prominent curvilinear vasculature and perivascular sclerosis. The differential diagnosis for this lesion included myxoid neurofibroma, schwannoma, soft tissue perineurioma, low-grade malignant peripheral nerve sheath tumor and low-grade fibromyxoid sarcoma. The tumor cells expressed MUC4, GLUT-1, and vimentin and were negative for S-100 protein, epithelial membrane antigen, smooth muscle actin, desmin, claudin-1, neurofilament and SOX10. Fluorescence in situ hybridization, with a break-apart probe strategy, demonstrated FUS rearrangement, consistent in this morphological context with the low-grade fibromyxoid sarcoma-associated FUS-CREB3L2 or FUS-CREB3L1 fusions. Low-grade fibromyxoid sarcoma is exceptionally rare in the peripheral nerve, with only a single previously reported case. Nonetheless, as our case illustrates, this entity must be included in the differential diagnosis of unusual intraneural mesenchymal tumors. As in all other locations, intraneural low-grade fibromyxoid sarcomas should be excised with negative margins. Patients with this disease require long-term clinical follow-up, given this tumor's propensity for very late distant metastases to the lungs and other sites.