Blood-filled Cystic Cavities Research Articles

MRI of Peliosis Hepatis: A Case Series Presentation With a 2022 Systematic Literature Update.

Peliosis hepatis (PH) is a rare benign condition, characterized by hepatic sinusoidal dilatation and blood-filled cystic cavities, often found incidentally, with still challenging diagnosis by imaging due to polymorphic appearance. Based on a retrospective analysis of our series (12 patients) and systematic literature review (1990-2022), to organize data about PH and identify features to improve characterization. Retrospective case series and systematic review. Twelve patients (mean age 48 years, 55% female) with pathology-proven PH and 49 patients (mean age 52 years, 67% female) identified in 33 studies from the literature (1990-2022). 1,5-T; T1-weighted (T1W), T2-weighted (T2W), diffusion-weighted (DW), contrast-enhanced (CE) T1W imaging. We compared our series and literature data in terms of demographic (gender/age/ethnicity), clinical characteristics (symptoms/physical examination/liver test), associated conditions (malignancies/infectious/hematologic/genetic or chronic disorders/drugs or toxic exposure) percentage. On magnetic resonance imaging lesion numbers/shape/mean maximum diameter/location/mass effect/signal intensity were compared. PH pathological type/proposed imaging diagnosis/patient follow-up were also considered. Joanna Briggs Institute (JBI) Critical Appraisal Checklist for Case Reports/Series quality assessment. Intraclass correlation and Cohen's kappa coefficients for levels of inter/intrareader agreement in our experience. Patients were mainly asymptomatic (92% vs. 70% in our study and literature) with associated conditions (83% vs. 80%). Lesions showed homogeneous T1W-hypointensity (58% vs. 65%) and T2W-hyperintensity (58% vs. 66%). Heterogeneous nonspecific (25% vs. 51%), centrifugal (34% vs. 8%), or rim-like centripetal (25% vs. 23%) patterns of enhancement were most frequent, with hypointensity on the hepatobiliary phase (HBP), without restricted diffusivity. Good inter- and intrareader agreement was observed in our experience. Concerning JBI Checklist, 19 out of 31 case reports met at least 7 out of 8 criteria, whereas 2 case series fulfilled 5 and 6 out of 10 items respectively. A homogeneous, not well-demarcated T1W-hypointense and T2W-hyperintense mass, with heterogeneous nonspecific or rim-like centripetal or centrifugal pattern of enhancement, and hypointensity on HBP, may be helpful for PH diagnosis. Among associated conditions, malignancies and drug exposures were the most frequent. 4 TECHNICAL EFFICACY: Stage 2.

2388 Peliosis Hepatis Progressing to Acute Liver Failure

INTRODUCTION: Peliosis hepatis is characterized as a vascular condition in which blood-filled cystic cavities are distributed diffusely throughout the liver parenchyma. The condition is rare, with the pathogenesis poorly described in the literature. It is believed that this disease is secondary to an underlying malignancy, immune disorder, infection, or a drug/toxin mediated effect. We present a case of a patient diagnosed with peliosis hepatis complicated by the development of acute liver failure. CASE DESCRIPTION/METHODS: A 71-year-old man with a past medical history of coronary artery disease, hypertension, hyperlipidemia, and prostate cancer treated with radiosurgery was admitted to our hospital with symptoms of fatigue, dysphagia and anorexia. He was in his usual state of health until four weeks prior to admission, when he began experiencing early satiety with poor oral intake, nausea, diarrhea, right upper quadrant abdominal pain, and weight loss. He was evaluated by his primary care provider who prescribed him a five day course of levofloxacin with no resolution of symptoms. Upon admission, labs were notable for leukocytosis and a mild transaminitis. Imaging revealed hepatomegaly with hepatic hemangiomatosis and splenic hemangiomas. A liver MRI was performed which returned consistent with diffuse hepatic peliosis with splenic peliosis. He underwent an intensive hematologic/oncologic, rheumatologic, and infectious workup that was significant for a positive quantiferon TB gold and he was subsequently started on empiric TB therapy. Synthetic liver function continued to worsen consistent with acute liver injury, which progressed to acute liver failure. The patient continued to decline and ultimately passed from multi-organ system failure. DISCUSSION: The majority of patients with peliosis hepatis will present asymptomatically and are found incidentally, while a subset of patients will present with abdominal pain or stigmata of liver disease. A liver biopsy is useful for diagnosis and is used in the context of the clinical picture, laboratory data, and imaging findings. Once the underlying offending etiology is recognized and appropriately treated, symptoms can improve and regression of the hepatic lesions can be seen. Our patient is unique in that his presentation was atypical and rapid, and a true causal etiology was never concretely identified, leading to progression to acute hepatic failure.

Analysis of lymphoid tissue markers in angiomatoid fibrous histiocytoma

Abstract Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of uncertain differentiation. AFH is characterized by nodular distribution of ovoid and spindle cells with blood-filled cystic cavities. It is circumscribed by thick fibrous pseudo-capsule and a surrounding dense lympho-plasmacytic infiltration encompassing germinal centers. In this study, we analyzed the structure of AFH with immunohistochemistry using markers for lymphoid organogenesis. The tumor was a circumscribed pyriform mass, composed of histiocytoid and spindle cells. The top part of the mass encompassed cribriform vasculature, which was positive for CD31, a marker for blood endothelial cells (BECs). This BEC-dominant area contained numerous lymphoid, histiocytoid, or fibroblastoid cells, which expressed CD4 (but not CD3), CD31, CXCL13, SDF-1 (CXCL12), and receptor for LTβ (LTβR). The peripheral lymph node address in (PNAd), a marker for high endothelial venules (HEVs), was positive in some vessels, where T and B cells were clustered. The bottom part of the mass was occupied with polygonal cells expressing podolanin, a marker for lymphatic epithelial cells (LECs). The whole mass was encapsulated with CD10+ fibroblast-like cells and surrounded by organized lymphoid infiltration containing T cell areas and B cell follicles with germinal centers. Our analysis illustrates a case of AFH composed of BEC- and LEC-dominant compartments. The well-organized peritumoral lymphoid infiltration indicated that AFH may produce an environment suitable for inducing inducible skin associated lymphoid tissue (iSALT).

Spinal aneurysmal bone cyst presenting as acute paraparesis during pregnancy

Aneurysmal bone cysts (ABC) are benign but locally aggressive osseous lesions characterized by blood-filled cystic cavities that account for 1–2% of all bone tumors. While pain remains the most common presenting symptom of spinal ABC, extensive anterior column involvement can result in vertebral fractures, spinal instability, and neurological deficits from compression of neural elements. An exceedingly rare case of thoracic ABC presenting as acute paraparesis in a 21-year-old pregnant woman, that was successfully treated with urgent surgical decompression and stabilization, is reported, with discussion of management strategies and review of the pertinent literature.

Angiomatoid fibrous histiocytoma: the current status of pathology and genetics.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate biologic potential and uncertain differentiation, most often arising in the superficial extremities of children and young adults. While it has characteristic histologic features of nodular distributions of ovoid and spindle cells with blood-filled cystic cavities and a surrounding dense lymphoplasmacytic infiltrate, there is a significant morphologic spectrum, which coupled with its rarity and lack of specific immunoprofile can make diagnosis challenging. Angiomatoid fibrous histiocytoma is associated with 3 characteristic gene fusions, EWSR1-CREB1 and EWSR1-ATF1, which are also described in other neoplasms, and rarely FUS-ATF1. Angiomatoid fibrous histiocytoma is now recognized at an increasing number of sites and is known to display a variety of unusual histologic features. To review the current status of AFH, discussing putative etiology, histopathology with variant morphology and differential diagnosis, and current genetics, including overlap with other tumors harboring EWSR1-CREB1 and EWSR1-ATF1 fusions. Review of published literature, including case series, case reports, and review articles, in online medical databases. The occurrence of AFH at several unusual anatomic sites and its spectrum of morphologic patterns can result in significant diagnostic difficulty, and correct diagnosis is particularly important because of its small risk of metastasis and death. This highlights the importance of diagnostic recognition, ancillary molecular genetic confirmation, and close clinical follow-up of patients with AFH. Further insight into the genetic and epigenetic changes arising secondary to the characteristic gene fusions of AFH will be integral to understanding its tumorigenic mechanisms.

Angiomatoid fibrous histiocytoma: comparison of fluorescence in situ hybridization and reverse transcription polymerase chain reaction as adjunct diagnostic modalities.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate biologic potential and uncertain differentiation, most often arising in the extremities of children and young adults. Although it has characteristic histologic features of a lymphoid cuff surrounding nodules of ovoid cells with blood-filled cystic cavities, diagnosis is often difficult due to its morphologic heterogeneity and lack of specific immunoprofile. Angiomatoid fibrous histiocytoma is associated with recurrent chromosomal translocations, leading to characteristic EWSR1-CREB1, EWSR1-ATF1, and, rarely, FUS-ATF1 gene fusions; fluorescence in situ hybridization (FISH), detecting EWSR1 or FUS rearrangements, and reverse transcription-polymerase chain reaction (RT-PCR) for EWSR1-CREB1 and EWSR1-ATF1 fusion transcripts have become routine ancillary tools. We present a large comparative series of FISH and RT-PCR for AFH. Seventeen neoplasms (from 16 patients) histologically diagnosed as AFH were assessed for EWSR1 rearrangements or EWSR1-CREB1 and EWSR1-ATF1 fusion transcripts. All 17 were positive for either FISH or RT-PCR or both. Of 16, 14 (87.5%) had detectable EWSR1-CREB1 or EWSR1-ATF1 fusion transcripts by RT-PCR, whereas 13 (76.5%) of 17 had positive EWSR1 rearrangement with FISH. All 13 of 13 non-AFH control neoplasms failed to show EWSR1-CREB1 or EWSR1-ATF1 fusion transcripts, whereas EWSR1 rearrangement was present in 2 of these 13 cases (which were histopathologically myoepithelial neoplasms). This study shows that EWSR1-CREB1 or EWSR1-ATF1 fusions predominate in AFH (supporting previous reports that FUS rearrangement is rare in AFH) and that RT-PCR has a comparable detection rate to FISH for AFH. Importantly, cases of AFH can be missed if RT-PCR is not performed in conjunction with FISH, and RT-PCR has the added advantage of specificity, which is crucial, as EWSR1 rearrangements are present in a variety of neoplasms in the histologic differential diagnosis of AFH, that differ in behavior and treatment.

From aneurysmal bone cyst to telangiectatic osteosarcoma with metastasis in inguinal lymph nodes: Case report

Aneurysmal bone cyst is a benign bone lesion composed of blood filled cystic cavities lined by fibrous septa. Its malignant transformation of is a rare event. We report a case of a lesion in the second metatarsal bone in a 29-year-old male, presented as a slight swelling of the right foot. After the curettage had been done, the diagnosis of aneurysmal bone cyst was made but the recurrence occurred 4 years later. The biopsy of the recurrent tumor showed compact neoplastic tissue consistent with diagnosis of giant cell tumor with malignancy. The malignant component was recognized as a high grade sarcoma with osteoid production. A tumor mass with the whole II metatarsal bone was extirpated and a resected part of fibula was transplanted. A year later, another recurrence occurred, an amputation was performed and a teleangiectatic osteosarcoma with ingvinal lymph nodes metastases was diagnosed. No other tumor mass was confirmed, either clinically or by imaging techniques at the time of his third operation. He died 4 months later with multiple pulmonary metastases. We emphasize the importance of team work in order to achieve the accurate diagnosis, highlighting careful radiological examinations, good sampling and awareness of unusual cases in bone tumor pathology.

Secondary Aneurysmal Bone Cystic Change of the Chondroblastoma, Mistaken for a Primary Aneurysmal Bone Cyst in the Patella

A 29-year-old woman complained of a 3-month history of left knee pain without trauma history. X-ray showed a well-defined osteolytic lesion with a sclerotic margin in the patella and magnetic resonance imaging showed T1-low and T2-high signal intensity with different fluid level. Our impression was an aneurysmal bone cyst. At surgery, the lesion was a blood-filled cystic cavity, surrounded by a gray or brownish tissue. Hemorrhagic soft tissues with recognizable bone fragments were observed. Curettage and autogenous bone graft was done. Microscopically, sheets of tumor cells were intermingled with some areas of eosinophilic chondroid matrix. The tumor cells showed oval-shaped nuclei with moderate eosinophilic cytoplasm. Several multinucleated giant cells and blood filled cystic cavities were observed. The final diagnosis was a chondroblastoma with a secondary aneurysmal bone cyst. At the post-operative 1.5-year follow-up, grafted bones were well incorporated radiographically and there were no recurrent evidence or any other abnormal symptoms.

Angiomatoid Fibrous Histiocytoma: A Review With Recent Genetic Findings

Angiomatoid fibrous histiocytoma is a neoplasm of intermediate biologic potential most often arising in the extremities of children and young adults. Its rarity may lead to misdiagnosis as either a reactive lesion or a benign or higher-grade tumor. Originally described as a type of malignant fibrous histiocytoma, its differentiation remains enigmatic, with precise histogenesis still only hypothesized. Its morphology is distinct, as a circumscribed lesion with sheets of bland spindle to ovoid cells, peripheral lymphoplasmacytic infiltrate, and blood-filled cystic cavities, and half of the cases show strong desmin expression. Cytogenetically, 2 specific translocations, t(12:16)(q13:p11) and more recently t(12:22)(q13:q12), have been characterized. The literature on angiomatoid fibrous histiocytoma is reviewed, particularly with regard to recent molecular genetic developments and differentiation, and its morphology, immunohistochemistry, and differential diagnosis are summarized.

Aneurysmal Bone Cyst of the Second Cervical Vertebrae Causing Multilevel Upper Cervical Instability

Aneurysmal bone cyst (ABC) is a rare expansile osteolytic lesion of bone comprising proliferating vascular tissue lining blood-filled cystic cavities. ABCs occur most frequently in patients under age 20 and are uncommon after 30 years of age. Three to 20% of cases occur in the spine, and upper cervical involvement is rare. Lesions may grow rapidly and attain considerable size. When involving the spine, ABCs may result in instability and neurologic compromise, making prompt diagnosis and treatment imperative. We present a report of a 6-year-old child with an ABC of the second cervical vertebrae causing atlantoaxial and C2-C3 instability, treated successfully with curettage, decompression, and anterior and posterior arthrodesis with posterior instrumentation. The patient underwent a staged procedure consisting of posterior instrumentation from occiput to C4 and curettage of the lesion followed by anterior cervical discectomy and fusion of C2-C4. The diagnosis, surgical treatment, and outcome of the case are described and relevant literature reviewed. The patient sustained no lasting neurologic deficits and was disease-free at 3 years of follow-up. ABC is a rare but potentially devastating cause of upper cervical spine instability. Prompt detection and treatment with curettage, decompression, and fusion can produce a satisfactory result and prevent spinal cord injury.

Aneurysmal bone cyst of the spine

The authors present the case of an 11-year-old boy with a painful, rapidly expanding lesion in the posterior triangle of the neck. There was no history of cervical trauma. Computerized tomography of the neck revealed a unicameral (single-chambered) aneurysmal bone cyst involving the C3 vertebra. Treatment was by open resection and curettage; no recurrence was seen at six months. We discuss the natural history, differential diagnosis, radiographic appearance and treatment modalities for this unusual, benign, expanding, osteolytic lesion containing blood-filled cystic cavities.

Diagnosing of peliosis hepatis by magnetic resonance imaging

Peliosis hepatis, an uncommon benign disorder, is characterized by widespread blood-filled cystic cavities appearing in variable size and shape in the liver. It may be associated with malignancy, acquired immune deficiency syndrome, and anabolic steroids usage [1].

Surgical management of aneurysmal bone cysts of the spine

Aneurysmal bone cysts of the spine are benign, highly vascular osseous lesions of unknown origin that may present difficult diagnostic and therapeutic challenges. They are expansile lesions containing thin-walled, blood-filled cystic cavities that cause bone destruction and sometimes spinal deformity and neurological compromise. The treatment of aneurysmal bone cysts of the spine remains controversial according to the literature. In this review, the authors discuss the clinical manifestations, pathophysiological features, neuroimaging characteristics, and treatment strategies for these lesions. Treatment options include simple curettage with bone grafting, complete excision, embolization, and radiation therapy. Reconstruction and stabilization of the spine may be warranted if deformity and instability are present. Special factors need to be considered in the management of these lesions. Complete excision of aneurysmal bone cysts offers the best chance of cure and spinal decompression if neurological deficits are present.

Malignant tumors of the osteogenic matrix

This article focuses on major clinical and imaging features that are of practical interest in the diagnosis and management of osteosarcoma, a malignant tumor arising from the osteogenic matrix. The current histologic classification of this tumor is also reported. Different types of osteosarcoma are described, each of them with a definite clinical and radiographic pattern. Conventional radiography is the keystone to diagnosis because it allows analysis of the patterns relevant to the different lesions (location, site, bone destruction, periostal reaction, soft tissue masses). The most common type of osteosarcoma is defined classic or conventional high grade (75%) and it typically involves the medullary cavity. Radiographically, it may be predominantly osteosclerotic or osteolytic, but more frequently it has a mixed (osteoslerotic/osteolytic) pattern. The teleangiectatic osteosarcoma is an aggressive form (5%) characterized by marked vascularization with large blood-filled cystic cavities; its typical radiographic pattern is purely osteolytic. Juxtacortical osteosarcoma (8–10%) indicates a group of osteosarcomas apparently arising on bone surface. The most common type is parosteal osteosarcoma which affects older subjects and has a better prognosis than the classic type. Radiography shows a heavily ossified mass with a broad base attached to the underlying cortex. CT and MRI are useful in the differential diagnosis of osteosarcoma and myositis ossificans or osteocondroma. Rare types of osteosarcoma include the periosteal and high-grade surface variants, as well as secondary and multifocal osteosarcoma (osteosarcomatosis). CT and MRI are the imaging procedures of choice in locoregional staging (intraosseous and extraosseous spread, skip metastases, growth plate and articular involvement). CT of the chest is a useful tool for detecting lung metastases. Also MRI has a role in monitoring the response to chemotherapy and in detecting recurrence. It permits a more accurate study of the tumor volume than other imaging techniques and clinical examination. MRI becomes even more useful when paramagnetic contrast agents are administered because dynamic MRI with contrast enhancement help differentiate postchemotherapy changes from viable tumor—the latter enhancing rapidly and the former slowly. Thus, dynamic MRI allows a precise mapping of any residual tumor activity.

Peliosis hepatis. Report of nine cases.

Nine cases of peliosis hepatis are reported: five of these were associated with the administration of androgen or anabolic androgenic steroids and a sixth with large doses of medroxy-progesterone acetate. In five instances, neoplasm was present as an underlying disease. Antemortem evidence of liver disease was detected in six of seven cases but was not severe and had not contributed to death. The pathogenesis of blood-filled cystic cavities is discussed and the literature reviewed. Angiopathy of hepatic sinusoids in patients with wasting diseases or in those receiving androgens in coexistence with passive congestion of the liver appear to be factors in the pathogenesis of pleiosis hepatis.