“…Additionally, although the common variant of the banal fibrous histiocytoma is non-invasive and considered benign, the aneurysmal fibrous histiocytoma shows signs of aggressive locoregional growth as well as distant metastasis in exceptionally rare cases [ 2 , 6 , 7 , 8 ]. Malignant fibrous histiocytomas have been documented to show a wide variety of sites of dissemination such as cardiac, vascular and cerebral metastases [ 9 , 10 , 11 ].…”
An aneurysmal fibrous histiocytoma is a rare cutaneous soft-tissue tumour which accounts for approximately 0.06% of all dermatopathologies. Metastasis is exceedingly uncommon, to the point that there have only been eight reported cases in the scientific literature. We present the case of a 25-year-old male with a primary aneurysmal fibrous histiocytoma located in the nuchal region which exhibited rapid growth and abrupt ulceration over a short time span and showed signs of locoregional aggressive infiltration. A subsequent histopathological analysis confirmed the presence of diffuse solid and cystic pulmonary metastases. Further genetic sequencing verified LAMTOR1-PRKCD fusion. This case report seeks to review the existing literature on aneurysmal fibrous histiocytoma, discuss the challenges of differential diagnosis and propose standardised diagnostic criteria.
“…Additionally, although the common variant of the banal fibrous histiocytoma is non-invasive and considered benign, the aneurysmal fibrous histiocytoma shows signs of aggressive locoregional growth as well as distant metastasis in exceptionally rare cases [ 2 , 6 , 7 , 8 ]. Malignant fibrous histiocytomas have been documented to show a wide variety of sites of dissemination such as cardiac, vascular and cerebral metastases [ 9 , 10 , 11 ].…”
An aneurysmal fibrous histiocytoma is a rare cutaneous soft-tissue tumour which accounts for approximately 0.06% of all dermatopathologies. Metastasis is exceedingly uncommon, to the point that there have only been eight reported cases in the scientific literature. We present the case of a 25-year-old male with a primary aneurysmal fibrous histiocytoma located in the nuchal region which exhibited rapid growth and abrupt ulceration over a short time span and showed signs of locoregional aggressive infiltration. A subsequent histopathological analysis confirmed the presence of diffuse solid and cystic pulmonary metastases. Further genetic sequencing verified LAMTOR1-PRKCD fusion. This case report seeks to review the existing literature on aneurysmal fibrous histiocytoma, discuss the challenges of differential diagnosis and propose standardised diagnostic criteria.
“…It has been reported that the potential use of adjuvant chemotherapy and/or radiation therapy remains controversial (55,56). For example, four patients treated with chemotherapy and/ or radiation without surgery died within a year after diagnosis (7,13,24,36). Thus, we did not add therapeutic regimens other than the surgery, which was based on informed consent.…”
A rare case of cardiac malignant fibrous histiocytoma (MFH)is reported. A 55-year-old womancomplained of palpitation due to atrial fibrillation. Echocardiography, magnetic resonance imaging, and angiography demonstrated a tumor arising from the posterior wall of the left atrium. At surgery, the tumor was almost entirely resected and histologically defined as MFH.Neither chemotherapy nor irradiation was administered. Echocardiography revealed a local recurrence two months after the surgery and the patient died of advanced cachexy and heart failure 2 years later. The details of this case are presented with a review of the literature.
“…MFH has been reported to arise within the heart and produce multiple cerebral metastases [4]. It has been recognized that MFH may arise within the aortic wall and heart [4]. This may have been the case for our patient, though it cannot be confirmed without direct inspection of the aortic tissue.…”
Section: Discussionmentioning
confidence: 63%
“…MFH can rarely arise primarily within the CNS and take cystic forms [3]. MFH has been reported to arise within the heart and produce multiple cerebral metastases [4]. It has been recognized that MFH may arise within the aortic wall and heart [4].…”
Malignant fibrous histiocytoma (MFH) is one of the most common subtypes of the pleomorphic sarcomas. It has rarely been reported to metastasize to the brain and even less often to manifest as cystic brain lesions. We report an unusual case of MFH occurring in the aorta and presenting as multiple cystic brain lesions and peripheral blood eosinophilia. The combination of eosinophilia and multiple cystic brain lesions in a patient from an area of the world endemic for Taenia solium suggested the more common diagnosis of neurocysticercosis. However the patient's clinical course and subsequent investigations revealed the very rare combination of features (aortic origin, eosinophilia induction, and multiple cystic brain metastases) of this unusual tumor.
Case ReportA 56-year-old male from South America presented with right sided weakness that had developed slowly over the course of 2 days. Blood work revealed a normal white count (8.8 9 10 9 cells/dl) with eosinophilia (8.0%). Head CT revealed multiple cystic lesions throughout the brain parenchyma, some with surrounding edema (see Fig. 1a). MRI showed multiple cystic ring-enhancing lesions, some with surrounding edema. No scolix were identified, however one cyst had necrotic material within it. Due to the systemic eosinophilia and the patient residing in an endemic region, neurocysticercosis was suspected. Serum antibody for Taenia antigens was negative. CT of the chest abdomen and pelvis was initially normal, but transesophageal echocardiography revealed a large, mobile, irregularly shaped mass with echo free spaces in the aorta which appeared to extend into the left subclavian artery (see Fig. 1b). This was confirmed by aortic MRA (see Fig. 1c). During the course of these investigations (15 days), the patient developed new onset left sided weakness, and was found to have new cerebral lesions. Brain biopsy performed 23 days after initial presentation revealed pleomorphic cells with variable size and shapes, some cells with lymphocytic and histiocytic features in an angiocentric distribution (see Supplementary Figure). These cells invariably stained positive for vimentin and KP-1 (a CD68 histiocytic marker), and variably for CD30 and Alk-1. A TEE done 2 months after the initial one showed that the mass had increased in size and filled the distal aortic arch. There was also a new large mass in the posterior atrioventricular groove near the left atrium which invaded the left atrial free wall and entered the left atrial cavity. At this time, the patient had developed a new mass in his trapezius muscle which was biopsied and showed large, Electronic supplementary material The online version of this article (
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