Pulmonary artery sarcoma affecting the pulmonary valve mistaken as pulmonary vasculitis: a case report and comparative literature review.

Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.

A rare case of epithelioid angiosarcoma.

Epithelioid angiosarcoma is a rare high-grade vascular neoplasm with a poor prognosis. We present an anticoagulated 77-year-old man, with a history of popliteal/soleal vein thrombosis in the previous month, complaining of ipsilateral persistent lower limb pain and claudication. Absent popliteal/distal pulses prompted an arterial doppler ultrasound (DUS), revealing thrombosis of the distal superficial femoral artery and a popliteal mass. As the arterial wall's integrity could not be appropriately evaluated by DUS, adventitial cystic disease of the popliteal artery was suspected. Computed tomography angiography and magnetic resonance imaging findings were also suggestive. Due to refractory pain, he was submitted to a popliteal mass excision along with a femoral-posterior tibial bypass. Pathology revealed an epithelioid angiosarcoma. He was referred to a Sarcoma Center, requiring hospitalization for agitation and fever. A positron emission tomography (PET) scan revealed extensive lower limb disease persistence and distant metastases. He died on the 56th day after surgery. To our knowledge, there are only 15 cases of angiosarcoma of the popliteal artery described in the literature. Ours stands out as the first one unrelated to a popliteal aneurysm. Being a highly-aggressive tumor, an early diagnosis is challenging but essential to a successful treatment, warranting the need for suspicion of this neoplasm. An early core biopsy or surgical sample may expedite the diagnosis.

Tislelizumab combined with GT chemotherapy for intimal sarcoma of inferior vena cava: A case report.

RATIONALE: Intimal sarcoma of inferior vena cava (IVC) is a rare soft tissue sarcoma with no typical symptoms and specific imaging features in the early stage, and there is a lack of standardized treatment and methods. PATIENT CONCERNS: A 54-year-old female patient presented to Fenghua District People's Hospital with a post-active cough and hemoptysis and was subsequently referred to our hospital. DIAGNOSES: The patient was pathologically diagnosed as intimal sarcoma of IVC complicating multiple intrapulmonary metastases. Chest CT revealed left lung malignant tumor with multiple intrapulmonary metastases; while enhanced upper abdominal CT showed cancer embolus of IVC with extension to right atrium and bilateral renal veins. Besides, hematoxylin and eosin staining suggested intimal sarcoma of veins. Immunohistochemical staining showed positivity for PD-L1, Ki-67, CD31, Desmin and ERG. INTERVENTIONS: The patient initially received GT chemotherapy (gemcitabine injection + docetaxel). Then, immunotherapy (tislelizumab) was added based on the results of genetic testing (TP53 gene mutation). OUTCOMES: The disease was stabilized after receiving the treatment. LESSONS: Given the lack of characteristic clinical manifestations in patients with intimal sarcoma of IVC, imaging examination combined with immunohistochemical index were helpful for diagnosis of intimal sarcoma of IVC. Furthermore, the combination of tislelizumab and GT chemotherapy was feasible in such patients with positive PD-L1 expression and TP53 mutation.

Infantile Hemangiomas and Vascular Anomalies.

Vascular anomalies represent a diverse group of disorders of abnormal vascular development or proliferation. Vascular anomalies are classified as vascular tumors and vascular malformations. Significant advances have been made in the understanding of the pathogenesis, natural history, and genetics of vascular anomalies, allowing for improvements in management including targeted molecular therapies. Infantile hemangiomas are the most common vascular tumor of childhood and follow a distinct natural history of proliferation and involution. Although benign, infantile hemangiomas can be associated with important complications. The use of beta-blockers has revolutionized the management of infantile hemangiomas. Other vascular tumors include pyogenic granulomas, congenital hemangiomas, and kaposiform hemangioendotheliomas, among others. Vascular malformations are categorized based on the type of involved vessel, including capillary malformations, venous malformations, lymphatic malformations, arteriovenous malformations, and mixed vascular malformations. Expert multidisciplinary management of vascular anomalies is critical to optimize outcomes in these patients. [Pediatr Ann. 2024;53(4):e129-e137.].

Thrombus or tumor? A case report of a rare sarcoma entity: intimal sarcoma of the pulmonary arteries.

BACKGROUND: Tumor embolism is a very rare primary manifestation of cancers and the diagnosis is challenging, especially if located in the pulmonary arteries, where it can mimic nonmalignant pulmonary embolism. Intimal sarcoma is one of the least commonly reported primary tumors of vessels with only a few cases reported worldwide. A typical location of this malignancy is the pulmonary artery. Herein, we present a case report of an intimal sarcoma with primary manifestation in the pulmonary arteries. A 53-year-old male initially presented with dyspnea. On imaging, a pulmonary artery embolism was detected and was followed by thrombectomy of the right ventricular outflow tract, main pulmonary artery trunk, and right pulmonary artery after ineffective lysis therapy. Complementary imaging of the chest and abdomen including a PET-CT scan demonstrated no evidence of a primary tumor. Subsequent pathology assessment suggested an intimal sarcoma further confirmed by DNA methylation based molecular analysis. We initiated adjuvant chemotherapy with doxorubicin. Four months after the completion of adjuvant therapy a follow-up scan revealed a local recurrence without distant metastases. DISCUSSION: Primary pulmonary artery intimal sarcoma (PAS) is an exceedingly rare entity and pathological diagnosis remains challenging. Therefore, the detection of entity-specific molecular alterations is a supporting argument in the diagnostic spectrum. Complete surgical resection is the prognostically most important treatment for intimal cardiac sarcomas. Despite adjuvant chemotherapy, the prognosis of cardiac sarcomas remains very poor. This case of a PAS highlights the difficulty in establishing a diagnosis and the aggressive natural course of the disease. CONCLUSION: In case of atypical presentation of a pulmonary embolism, a tumor originating from the great vessels should be considered. Molecular pathology techniques support in establishing a reliable diagnosis.

Optimizing random skin biopsies: a review of techniques and indications for intravascular large B-cell lymphoma.

Intravascular large B-cell lymphoma (IVLBCL), a rare subtype of malignant lymphoma, is diagnosed by observation of intravascular proliferation of tumor cells in samples taken from affected organs. However, diagnosis of IVLBCL is usually difficult due to the lack of mass formation. IVLBCL may be fatal when the diagnosis is delayed, so an accurate early diagnosis is the key to successful treatment. Random skin biopsy (RSB), in which specimens are sampled from normal-appearing skin, has been reported as useful. However, the specific method of RSB remains controversial, with individual institutions using either the punch method or the incisional method. Research has shown that the incisional method has higher sensitivity than the punch method. We discuss whether this difference might owe to the collection of punch specimens from an insufficient depth and whether the punch method might result in false negatives. For RSB, we recommend taking specimens not only from normal-appearing skin, but also from any lesional skin, because lesions may reflect micro IVLBCL lesions. To ensure accurate diagnosis, both dermatologists and hematologists should know the proper method of RSB. This review summarizes the appropriate biopsy method and sites for RSB.

Intravascular large B-cell lymphoma of the eye: Literature review and new findings.

PURPOSE: Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare, aggressive, multi-system disease that can affect the eye. We describe the ophthalmic presentation, multimodal imaging and treatment response of uveal IVLBCL. METHODS: Review and case report. RESULTS: Twenty-five published cases of IVLBCL involving the eye including our own were identified. Of these, 15 patients (60%) had clinically-detectable intraocular involvement, 6 (24%) had extraocular ophthalmic involvement only and 4 (16%) had subclinical, undiagnosed intraocular involvement that was retrospectively detected on post-mortem ocular histopathology. The male to female ratio was 1.08:1 with a mean presenting age of 65.1 ± 11.7 years (range 38-82 years). The majority of cases had bilateral involvement (21/25 patients, 84%). Extraocular manifestations included diplopia, ptosis and ophthalmoplegia. Intraocular manifestations included serous retinal detachment (13/28, 46%), retinal hemorrhages (9/28, 32%), vascular changes (9/28, 32%), retinal pigment epithelial changes (7/28, 25%), thickened choroid (6/28, 21%), vitritis (5/28, 17%), cotton-wool spots (3/28, 10%), and a subretinal lesion (1/28, 3%). Histopathological diagnosis was most commonly confirmed on post-mortem enucleation (8/25 patients, 32%), skin (6/25 patients, 24%) or brain biopsy (6/25 patients, 24%). CONCLUSION: The presence of intra-retinal hemorrhages, cotton wool spots and/or Roth spots help differentiate IVLBCL from other similarly presenting diseases such as central serous chorioretinopathy and Vogt-Koyanagi-Harada disease. New signs not previously described in IVLBCL include macular bacillary layer detachment and hypo-cyanescent spots on ultra-wide field indocyanine green angiography. The diagnosis is elusive and requires tissue biopsy, but systemic chemotherapy and rituximab can lead to rapid improvement of the eye.

A Rare Case of Pulmonary Artery Sarcoma.

Pulmonary artery sarcoma (PAS) is a rare and aggressive mesenchymal tumor with an overall poor prognosis1-5. Due to similar clinical and radiologic findings, PAS is often misdiagnosed as a pulmonary embolism (PE) frequently leading to prolonged anticoagulation therapy, which delays the correct diagnosis 1-3. By presenting this clinical case our objective is to emphasize characteristic CT findings that favour a neoplastic origin of a pulmonary intravascular filling defect. PET-CT and MRI have also an important potential role in its diagnosis and therapeutical management.

Hemangiossarcoma ocular em um equino: relato de caso / Equine ocular hemangiosarcoma: case report

O hemangiossarcoma ocular na espécie equina é um tumor maligno, raro e agressivo, de origem vascular endotelial. No presente trabalho, descreve-se um caso de hemangiossarcoma ocular em uma égua de 10 anos que apresentava secreção serossanguinolenta advinda de uma massa, acometendo a conjuntiva bulbar e a terceira pálpebra do olho direito. O diagnóstico foi realizado com base na avaliação histopatológica e na imuno-histoquímica. Foi realizada a enucleação, assim como a completa excisão cirúrgica do tecido acometido, não sendo observada, após seis meses da terapia, a recidiva ou a metástase da lesão.

Equine ocular hemangiosarcoma is a rare and aggressive malignant tumor of vascular endothelial origin. We describe a case of ocular hemangiosarcoma in a 10-year-old mare with serosanguineous secretion arising from a mass involving the bulbar conjunctiva and third eyelid of the right eye. The diagnosis was based on histopathological evaluation and immunohistochemistry. Enucleation was performed as complete surgical excision of the affected tissue, with no recurrence or metastasis of the lesion being observed after six months of.

Fibrosarcoma de arteria pulmonar simulando un síndrome de Hughes Stovin / Pulmonary artery sarcoma mimicking a Hughes Stovin syndrome. Report of one case

Pulmonary artery sarcoma is an uncommon entity with high mortality. Its clinical presentation is usually indistinguishable from pulmonary embolism, which leads to a significant delay in diagnosis. Hughes-Stovin syndrome is characterized by venous thrombosis and aneurysms of the pulmonary or bronchial artery. We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy. The patient died five days after surgery and the pathological examination of the surgical piece revealed a pulmonary artery sarcoma.

Hemangiopericitoma: reporte de 3 casos / Hemangiopericytoma: a report of 3 cases and a literature review

Introduction: The hemangiopericytoma (HPC) is a rare vascular tumor that can be potentially malignant. It can be found in any part of the body but usually in the lower extremities or the retroperitoneum. Because its potential malign nature, it's necessary to perform oncological resections when are operating on. Methods: Three cases are presented. Results: One of them was located at the sacrococcigeal space, being the second reported case in the international literature. The other cases were in cervical and adrenal localization.

Introducción: El hemangiopericitoma (HPC) es un tumor vascular raro y potencialmente maligno que puede localizarse en cualquier parte del cuerpo, con mayor frecuencia en extremidades inferiores y retrope-ritoneo. Su potencial malignidad da la necesidad de realizar resecciones oncológicas al operarlos. Material y Método: Se presentan tres casos tratados por nuestro grupo. Resultados: Uno de ellos tiene localización sacra siendo, hasta donde sabemos, el segundo en ser publicado. Otro es de localización suprarrenal y el tercero es cervical.

Anomalías vasculares / Vascular anomalies

Las anomalías vasculares incluyen un amplio número de enfermedades de la red vascular que tiene similitudes en su manifestación clínica, esto ha llevado por años a una confusión en la nomenclatura, diagnóstico y tratamiento. A continuación se presenta una revisión del tema que pretende describir la nomenclatura y clasificación vigente así como revisar la sintomatología, complicaciones y manejo actualizado.

Sonographic assessment of vessel invasion by cervical masses in dogs / Avaliação ultrassonográfica da invasão vascular por massas cervicais em cães

Tumor invasion of the vessels displays both therapeutic and prognostic implications and represents a challenge for head and neck surgeons. Although previous research has shown that ultrasound can detect such invasions, accurate sonographic parameters to do so have not yet been established. We sought to determine sonographic criteria which are able to characterize these invasions. A high-resolution transducer was used to perform ultrasound examinations of 15 patients selected from a group with inconclusive radiography and computed tomography diagnosis. We found that encasement of the vessel, tumor immobility or fixation in the vessel wall, and narrowing and/or deformity of the lumen were the best criteria. Indeed, when loss of hyperechoic interface of the vessel wall was used as a single criterion it generated false positive results. This study shows that a combination of parameters can be used to provide the best sensitivity and specificity values to produce conclusive diagnosis of vessel invasion by tumors in the cervical region.

Determinaram-se critérios ultrassonográficos capazes de caracterizar a invasão vascular por tumores em cães. Utilizaram-se transdutores de alta resolução para os exames ultrassonográficos realizados em 15 pacientes, selecionados de um grupo submetido previamente à radiografia e tomografia computadorizada, com resultados inconclusivos. Os melhores critérios encontrados foram: encarceramento do vaso, imobilidade do tumor ou aderência na parede vascular e estreitamento ou deformidade luminal. A perda de definição da interface hiperecoica da parede vascular quando foi usada como critério isolado produziu resultados falso positivos. O estudo demonstrou que uma combinação de parâmetros pode ser usada para aumentar a sensibilidade e especificidade diagnóstica, produzindo diagnósticos mais conclusivos e precisos pra definir a invasão vascular por tumores na região cervical ventral.

Hemangioma sinovial: presentación de un caso y revisión bibliográfica / Synovial hemangioma: case report and bibliographic revision

El hemangioma sinovial es un tumor benigno vascular de partes blandas mayormente visto en niños y adolescentes. La patogenia de esta lesión es incierta y se encuentra en debate. Presentamos el caso de un paciente de sexo masculino de 41 años de edad, futbolista y tenista amateur, que concurre a nuestro centro con antecedente de dolor de rodilla izquierda de 3 años de evolución. Al examen físico la rodilla era estable, las maniobras de Pívot y Lachman fueron negativas, tampoco presentaba semiología meniscal ni efusión. Se constató un déficit de extensión de 15º, con una flexión completa. Las radiografías de la rodilla afectada no presentaban lesiones óseas y en la resonancia magnética no se visualizaban alteraciones. Luego de realizar tratamiento kinesiológico sin resultados satisfactorios se realizo cirugía artroscópica. Constatándose en la región anterior a la inserción tibial del ligamento cruzado anterior la presencia de una masa de aspecto quístico, similar a la observada en los casos de Ciclops Síndrome. El informe de anatomía patológica informo hemangioma arteriovenoso. En aquellos casos donde se trata de hemangiomas focalizados o pediculados y de tamaño apropiado, la resección artroscópica es la alternativa de tratamiento, y en los hemangiomas puramente sinoviales la embolización selectiva del vaso principal es una alternativa a la cirugía.

Leiomiomatosis intravenosa de origen pelviano con extensión intracardiaca: Reporte de dos casos / Pelvic intravenous leiomyomatosis with intracardiac extension: Report of two cases

Background: symptoms predominate. Diagnosis is based on clinical findings and appropriate imaging. We report two females, aged 35 and 51 years. One of them presented with a pelvic mass and dyspnea, the other patient had severe cardiac failure on admission. Computed axial tomography scan allowed an accurate preoperative diagnosis on both patients. Successful one stage resection of the tumor was performed under cardiopulmonary bypass. Both patients are asymptomatic on follow up at 6 months and 25 years.