Disseminated Kaposi Sarcoma

Case Presentation: A 28-year-old male with a recent diagnosis of human immunodeficiency virus presented to the emergency department with odynophagia and dysphagia for a month. Physical exam revealed Kaposi sarcoma partially occluding the airway. Point-of-care ultrasound was used to assist with the diagnosis of reactive lymphadenopathy, and computed tomography revealed systemic disease. Otolaryngology was urgently consulted, and the patient was admitted for prompt tracheostomy the following day. Discussion: Kaposi sarcoma is a violaceous vascular neoplasm that is an acquired immuno-deficiency syndrome (AIDS)-defining illness. Mucocutaneous membranes should be thoroughly evaluated with patients suspected of AIDS. This case demonstrates the vital evaluation of the patient’s airway to assess patency. Highly active antiretroviral therapy should be initiated promptly, as well as chemotherapy in severe systemic cases.

Multifocal Epithelioid Hemangioendothelioma Complicated with Disseminated Intravascular Coagulation

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular neoplasm that requires long-term management. Several reports describe disseminated intravascular coagulation (DIC) associated with angiosarcoma, but no association with EHE has been reported. We encountered a patient with DIC complicated by multifocal EHE. The patient was an 83-year-old woman with spinal lesions, small lung nodules, and a soft tissue mass in the right buttock. The tumor was biopsied and diagnosed as EHE. The patient received pain control therapy without antitumor therapy. One month later, DIC developed with tumor progression. DIC subsided with nafamostat mesylate infusion, and oral apixaban was administered. DIC was managed for 5 months until the patient died of brain metastases. This is the first report of a patient with DIC complicated by EHE. It should be noted that progression of EHE can cause DIC. We were able to manage DIC using anticoagulant agents.

Orthotopic Liver Transplantation in a Case of Novel YAP1-TFE3 Hepatic Epithelioid Hemangioendothelioma [HEHE]

Epithelioid hemangioendothelioma is an extremely rare vascular neoplasm arising in soft-tissues and different visceral organs, with liver being the most commonly involved viscera. Hepatic epithelioid hemangioendothelioma (HEHE) is a malignant tumour with an indolent behaviour and unpredictable clinical course. It has a better prognosis among the malignant tumours of the liver, in spite of being a diffuse multifocal liver disease or metastatic at the time of presentation. HEHE is usually found to be noted in the fifth decade with slight female preponderance. No single treatment strategy has yet been established owing to its variable clinical course, ranging from an indolent tumour with prolonged survival to an aggressive, metastatic disease with a fatal outcome. Here, we present a case of a novel HEHE in a 25-year-old female who was treated successfully with orthotopic living donor liver transplantation and discuss the presentation, histopathology and management of this rare, fatal yet treatable malignant tumour.

Meningothelial Hamartoma of the Scalp: Clinicopathologic Review of an Unusual Tumor Mimicking a Vascular Neoplasm with Review of Literature

Introduction/Objective Benign meningothelial hamartoma of the scalp is a rare entity with only scattered case reports existing in the literature. The hamartomatous process is believed to occur secondary to ectopic displacement during embryogenesis with the migration of meningothelial cell precursors to the incorrect location or secondary to an obliterated meningocele. This tumor may be easily missed and may be misdiagnosed as lipoma or sometimes as cutaneous angiosarcoma due to the several histologic features that mimic the histology of a vascular neoplasm. Methods/Case Report We report an unusual benign skin lesion occurring in a 19-year-old man with no significant past medical history. The lesion had been present since he was an infant and had been slowly enlarging over the past 15 years. The lesion caused pain and discomfort and the patient underwent an excisional biopsy of the lesion. Histologic examination showed a subcutaneous lesion with ill-defined borders and peripheral areas of infiltration between adnexal structures. Immunohistochemistry performed on the tumor cells showed that they were positive for EMA, progesterone receptor, D2-40, and vimentin. A next-generation sequencing study using a hybrid capture-based panel examining 50 commonly mutated genes in human neoplasia was performed and showed no molecular alterations supporting the benign or non-neoplastic nature of the lesion. Along with this case, we review 20 cases of meningothelial hamartomas from 13 papers and summarize them in the table. Results (if a Case Study enter NA) N/A Conclusion To our knowledge, this is the first reported case of one of these lesions with associated molecular genetic testing. The molecular study carried out, in this case, did not disclose any genetic variants commonly associated with human neoplasia supporting the notion that this lesion does not represent the neoplastic process but rather a hamartomatous process. Meningothelial hamartoma is an exceedingly rare, benign lesion that may be confused with a low-grade vascular neoplasm due to its rare nature and unusual histologic features.

Extragingival benign growth-Case report at a rare site

To present a case report of extragingival pyogenic granuloma on lower lip which is an uncommon site for pathological entity and also to emphasize the reason for its occurrence. Pyogenic granuloma (PG) is a benign vascular neoplasm more often appear as lobulated or smooth red nodular lesion commonly in gingiva. Extragingival occurrence of PG is very rare. The present article reports a rare case of 55-year-old female with PG presenting rapid growing mass on the lower lip. This article highlights the possibility on the reason for its occurrence which is important for the diagnosis in this atypical location. The primary etiology of lower lip lesion is trauma and contributory factors being poor oral hygiene and betel quid chewing. Pyogenic granuloma is one of the common lesions in oral cavity. This article highlights the pyogenic granuloma on lip which is an unusual site. Although it can be diagnosed clinically, atypical presentations lead to inappropriate diagnosis. Therefore, further investigation by biopsy confirms final diagnosis upon which treatment is instituted.

Comparison between Kimura’s disease and angiolymphoid hyperplasia with eosinophilia: case reports and literature review

Kimura’s disease (KD) is a rare chronic inflammatory or allergic disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular neoplasm. Their relationship has always been debated. This article reports two rare cases, one of each disease. One patient was a 48-year-old female that presented with a mass on her right mandible. She also had oedema erythema and wheals on her lower limbs. She was diagnosed with Kimura’s disease complicated with chronic urticaria. The second patient was a 23-year-old female that presented with multiple nodules of unequal size on the scalp. She was diagnosed with angiolymphoid hyperplasia with eosinophilia. The first patient recovered after being treated with surgical resection, glucocorticosteroids, cyclophosphamide and radiotherapy. The second patient underwent the first stage of surgical excision and is currently being followed-up. Comparison of the clinical and histopathological features of these two cases supports the theory that KD and ALHE are two separate disease entities.

Primary Angiosarcoma of the Adrenal Gland: Report of 2 Cases and Review of the Literature

Primary adrenal angiosarcoma is a rare, malignant, vascular neoplasm. These neoplasms typically arise in middle age (median age of 60 years) and are more common in males (65%) than in females. Although rare, these neoplasms are aggressive with a propensity for local recurrence and metastasis and a median survival of 18 months. We present 2 cases of primary adrenal angiosarcoma with synchronous, ipsilateral adrenocortical adenomas. We review the cases of adrenal angiosarcoma reported since 1988 and discuss their clinical and histopathologic characteristics.

Gastric Epithelioid Haemangioendothelioma: A Case Report

Objective: To report a case of gastric epithelioid haemangioendothelioma, given its low incidence. Introduction: Epithelioid haemangioendothelioma is a vascular neoplasm composed of epithelioid or histiocytoid cells with endothelial characteristics. They have a very low incidence of approximately one per one million and are usually asymptomatic with a difficult preoperative diagnosis. Case Report: This study reports the case of a 58-year-old woman with a history of chronic anaemia. She underwent an elective upper endoscopy with evidence of an ulcerated submucosal lesion in the gastric antrum. The histological examination performed over the endoscopic biopsies showed the vascular characteristic of the tumor and endosonography confirmed the submucosal origin of the lesion. After resective surgery, the tumor showed the histological and immunohistochemical features of the epithelioid haemangioendothelioma. Conclusion: Surgery in the form of wide excision seems to be the treatment of choice for this rare neoplasm in the absence of histological markers of malignant potential.

Case Report: Multiple Cavernous Pericardial Lymphangioma (Pericardial Lymphangiomatosis) in a Captive Peregrine Falcon (Falco peregrinus brookei)

A 12-year-old female peregrine falcon (Falco peregrinus brookei) from a private raptor breeding facility that presented a good body condition, died suddenly without showing previous symptoms. At necropsy, in the coelomic cavity, multiple cystic structures demarcated by a thin transparent wall and filled with a serous content were observed. They were firmly adhered to the cranial part of the epicardium and adjacent tissues and occupied the entire thoracic area of the coelomic cavity. Microscopically, emerging simultaneously from several areas the epicardium, multiple irregular channels and cystic spaces, lined by a single endothelial cell layer and separated by fibrovascular septa containing smooth muscle tissue, were observed. Immunohistochemical examination revealed that the neoplastic endothelial cells positively immunolabelled for the pan-endothelial marker factor VIII-related antigen but immunostained negative for cytokeratins (PCK26) while strong positivity for sarcomeric α-smooth muscle actin (α-SMA) was detected in the cystic walls. Based on the morphological and immunohistochemical findings, lesions were determined as consistent with a multiple cavernous pericardial lymphangioma, or pericardial lymphangiomatosis, a rare vascular neoplasm. The animal also showed a diffuse chronic perihepatitis, a necrotic area in the liver and foci of cartilaginous metaplasia and calcification in the aorta and vena cava. Literature review, particularly on the epidemiology of lymphangioma, demonstrated the rarity of this tumor in the different animal species and in this location, particularly in birds, being the first report of this type of tumor in a peregrine falcon.