Sporadic Intradural Extramedullary Hemangioblastoma of Cauda Equina with Large Peritumoral Cyst—A Rare Presentation

Cauda equina intradural tumors commonly reported include ependymoma, schwannoma, neurofibroma, meningioma, and drop metastasis. Hemangioblastoma of the neural axis is a rare benign vascular tumor comprising only 1.6 to 6.4% of spinal tumors, and are usually associated with Von-Hippel Lindau disease. Sporadic intradural extramedullary hemangioblastoma involving cauda equina is very rare with only countable reports, and the presence of peritumoral cyst has been reported only once. We report one such case of hemangioblastoma with a large peritumoral cyst, which was diagnosed radiologically and confirmed by histopathology following surgical excision. Pertinent radiological characteristics, diagnostic clues, treatment, and surgical outcomes are discussed.

Case Report: Primary Pulmonary Angiosarcoma With Brain Metastasis

Primary pulmonary angiosarcoma (PPA) is a rare malignant vascular tumor, of which early diagnosis is challenging due to lack of specific clinical manifestations and a low level of suspicion. Here, we report a case of PPA presented with advanced brain metastasis. A 21-year-old patient with 1 week history of headache and mild cough was hospitalized for a head injury. Head MRI showed multiple intracranial lesions with brain edema. Chest CT displayed bilateral pulmonary infiltrates with mediastinal lymph node enlargement. After 2 months of anti-tuberculosis treatment, the patient was readmitted for persistent headache and cough with occasional hemosputum along with worsening pulmonary and intracranial lesions. Despite seizure prophylaxis and control of intracranial pressure and brain edema, his symptoms progressively aggravated, accompanied by cough with bloody sputum, frequent epileptic seizures, and hypotension. He eventually developed coma and died within 3 months of onset of symptoms. An autopsy confirmed PPA with brain metastasis.

A Case of Lobular Capillary Hemangioma at the False Vocal Cord With Intermittent Stridor

Lobular capillary hemangioma (LCH) is a type of benign vascular tumor. It arises from vascular endothelial cells and contains capillaries arranged in a lobular pattern. In the head and neck, the most common presenting location of LCH is the lips, and presentation in the larynx is very rare. LCH might not be distinct from granuloma in macroscopic views. We report a 71-year-old female with LCH of the larynx that was totally resected via laryngeal microsurgery with a CO2 laser and briefly review the literature.

Epithelioid Hemangioendothelioma of the Foot

We describe the case of an 18-year-old male Army reservist presenting with left lower extremity pain for which he was initially diagnosed with a stress injury. After failing conservative treatment, a radiograph was obtained showing a "lacelike" appearance of the medullary bone in the foot and ankle. Magnetic resonance imaging subsequently demonstrated widespread polyostotic marrow replacement with coarsened trabeculations. A biopsy was obtained which diagnosed the patient with polyostotic epithelioid hemangioendothelioma which is the most common malignant vascular tumor of bone. The patient ultimately underwent a below the knee amputation once computed tomography of the chest, abdomen, and pelvis excluded distant metastatic disease. It is important for radiologists to be aware of this diagnosis because osseous epithelioid hemangioendothelioma can present like a stress injury and be mistaken for a less serious diagnosis while potentially having visceral involvement.

Giant cell angioblastoma

Giant cell angioblastoma is an extremely rare tumor of vascular origin, described at the end of the 20th century. It belongs to tumors with intermediate malignant potential and is characterized by locally infiltrative growth. The tumor doesn’t have any clear distinctive clinical characteristics. The diagnosis is established on the basis of histological examination. Two main treatment options for this pathology are discussed in the literature: radical removal of the tumor and therapy with low doses of interferon alpha. As a rule, this is a combination treatment. This article describes our own clinical case. The patient’s parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications. Interest is in the rarity of the disease and the features of the clinical characteristics of this case, specifically the extremely unfavorable localization in the oropharynx region and, accordingly, the impossibility of carrying out not only a radical removal of the tumor, but also its resection. The high probability of developing irreversible neurological complications in this age group associated with interferon alpha therapy questioned the possibility of its use. For the first time in this histological variant of a vascular tumor, chemotherapy was applied, including metronomic therapy with cyclophosphamide and vinblastine in combination with a liposomal form of doxorubicin. After 8 courses of chemotherapy, a complete clinical response was obtained with the restoration of the patency of the respiratory and digestive tracts. The observation period at the time writing of this article was 36 months.

Massive Calcified Epithelioid Hemangioendothelioma With Multifocal Involvement: An Imaging Diagnosis Dilemma and a Rare Case Report

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor that develops from vascular endothelial or pre-endothelial cells. More than 60% patients have single-organ involvement, and involvement of multiple organs including the liver, lungs, and bones is extremely rare. The typical radiographic features of EHE include multiple small nodules in both lungs, which are usually located near small- and medium-sized blood vessels and the bronchi, and solitary, multiple, or diffuse lesions located at the hepatic periphery, spreading within the branches of the portal and hepatic veins. Radiologic calcification has been rarely reported in the literature. Here, we firstly described a case of a 53-year-old woman with EHE who presented with lungs, liver, bone, and right hilar lymph node involvement, manifesting as massive calcification on computed tomography. This case reminds physicians that EHE may present with unusual imaging manifestations, like massive calcification, and should be considered during the diagnostic process.

A case report of an extremely rare type of cardiac tumor: Primary Cardiac Angiofibroma

Cardiac angiofibroma is a very rare diagnosis when a patient develops an intracardiac mass. It is a primary benign cardiac tumor with a scarcity of information in the literature. This case report illustrates a 26-year-old man with a complaint of chronic chest tightness who was firstly diagnosed with right ventricle tumor by echocardiography then underwent cardiac MRI which confirmed the presence of a highly-vascular tumor with radiologically benign behavior. Then his tumor was excised, his postoperative course was uncomplicated and he was well within almost 2 months after discharge. Ultimately the histopathologic findings demonstrated vascular and stromal tissue in favor of angiofibroma and excluded the other diagnoses with IHC and trichrome staining. Angiofibroma is a benign, highly vascular tumor, mostly discovered in the nasopharynx. When it is found in the heart, CMR and pathology are pivotal to rule in its diagnosis. It is isointense in T1 weighted and hyperintense in T2 weighted sequences with intense enhancement following contrast injection. Its pathology contains an admixture of vasculatures with CD31 positive immunoreactivity for endothelial cells and fibrotic tissue with bluish coloration in trichrome staining. Eventually, its treatment includes merely surgical excision given its benign nature.

Potential Role of CTNNA3 and FRMPD4 in Vascular Thrombosis of Colon Adenocarcinoma

Background: Vascular tumor thrombus is an important pathological feature of malignant tumors, closely related to lymph node metastasis, and considered to be tumor micrometastasis. Two down-regulated genes, Catenin Alpha 3 (CTNNA3) and FERM And PDZ Domain Containing 4 (FRMPD4) were selected via analyzing the differential expression of vascular tumor thrombus in colon adenocarcinoma and paracancerous tissues. Furthermore, we observed the potential role of them in vascular thrombosis of colon adenocarcinoma.Method: Candidate genes for vascular thrombosis of colon adenocarcinoma were screened by GSE127069, and then pan-cancer verification and immune infiltration analysis were performed. The relationship between gene and vascular thrombosis was analyzed from the level of gene mutation by cBioPortal. Finally, the clinical samples collected were used for expression verification.Results: CTNNA3 and FRMPD4 were low-expressed in vascular tumor thrombus of colon adenocarcinoma, which were positively correlated with microsatellite instability (MSI). They were also closely related to immune microenvironment and immune cell subtype infiltration. According to the analysis of gene mutation, their gene deletion was related to the vascular invasion indicator. Finally, the protein and mRNA expression of CTNNA3 and FRMPD4 were down-regulated in the samples of vascular thrombosis of colon adenocarcinoma, compared with the normal glands which from paracancerous tissues.Conclusion: Our study demonstrates that CTNNA3 and FRMPD4 may be potential biomarkers of vascular thrombosis in colon adenocarcinoma. These results may provide a new strategy to identify the micrometastasis in colon adenocarcinoma.

The Clinicopathological Characteristics and Prognoses of dMMR Gastric Adenocarcinoma Patients

Background. Few studies on the clinicopathological features and prognosis of DNA mismatch repair deficiency (dMMR) gastric cancer (GC) have been reported, and no clear conclusions have been drawn about the factors affecting the prognosis of dMMR GC. The aim of this study was to explore the clinicopathological characteristics and prognoses of dMMR GC patients. Methods. From May 2011 to November 2020, GC patients who underwent surgery with dMMR confirmed by immunohistochemistry (IHC) at the Affiliated Cancer Hospital of Shanxi Medical University were selected. The patients’ clinical and pathological data were collected. The recurrence-free survival (RFS) and overall survival (OS) rates of the patients were determined through follow-up. SPSS 26.0 was used to analyze the patients’ clinicopathological features and prognoses. Results. A total of 162 dMMR GC patients met the inclusion criteria, and the median age was 63.5 years (32–89 years). dMMR GC was more common in males (65% vs. 35%), and most of the cases were stage II (the prevalence of stage I was 22%, that of stage II was 43%, that of stage III was 30%, and that of stage IV was 5%). Most of the lesions were located in the antrum (49%), followed by the cardia (25%). PMS2 and MLH1 (57%) deficiency was most common. Kaplan–Meier analysis showed that factors related to OS were family history ( P = 0.048 ), number of lymph node (LN) metastases ( P < 0.001 ), vascular tumor thrombus ( P < 0.001 ), HER2 expression status ( P = 0.025 ), and clinical stage ( P < 0.001 ). The factors related to RFS included vascular tumor thrombus ( P < 0.001 ), number of LN metastases ( P < 0.001 ), and clinical stage ( P < 0.001 ). Conclusion. In this study, dMMR GC was more common in men, and the median age was 63.5 years. Most of the lesions were in the antrum and showed the combined deletion of MLH1 and PMS2. dMMR GC patients tended to be early stage, and the prognosis of those with early-stage GC was better. dMMR GC patients with vascular tumor thrombus or >6 LN metastases had a high recurrence rate and poor survival outcome.