暂无摘要。

OBJECTIVE: To explore the genetic basis and pathogenesis for a child with type I Hereditary hemorrhagic telangiectasia (HHTⅠ) and Splenic sinus shore cell hemangioma (LCA).
METHODS: A child with HHT complicated with LCA diagnosed at the First Affiliated Hospital of Dali University in April 2022 was selected as the study subject. Clinical data of the child and her relatives were collected, and pathogenic variants were screened by whole exome sequencing. Candidate variant was verified by Sanger sequencing and bioinformatic analysis.
RESULTS: The patient, a 16-year-old female, had recurrent epitaxis since childhood, which sometimes necessitated hemostasis treatment. She also had splenectomy due to splenic rupture and was diagnosed with LCA. Her father and grandmother also had a history of recurrent epitaxis. Her father had deceased due to cerebral vascular rupture. The child was found to harbor a c.360+1G>A variant in the ENG gene. The same variant was not found in her asymptomatic mother and brother.
CONCLUSIONS: The c.360+1G>A variant of the ENG gene probably underlay the pathogenesis in this child.

Burkitt lymphoma is a non-Hodgkin B-cell lymphoma with a high prevalence in the pediatric population. Abdominal manifestations are well known in sporadic Burkitt lymphoma and vary from nonspecific symptoms to intestinal obstruction due to intussusception; however, mass-like splenic involvement has been scarcely described.
OBJECTIVE: To present a case of a patient with a splenic mass whose histopathological analysis revealed Burkitt lymphoma.
METHODS: A 13-year-old female patient presented with abdominal pain, progressive weight loss, and fever. Imaging studies showed a splenic mass, intestinal thickening, and ileal intussusception. Histopathological analysis of spleen biopsy revealed Burkitt lymphoma. After the first cycle of chemotherapy (BFM95-NHL protocol), abdominal symptoms resolved; no other signs suggestive of intussusception were observed, as well as a significant reduction of the splenic mass was observed.
CONCLUSIONS: Burkitt lymphoma in pediatric patients can present as a well-defined splenic tumor, causing no splenomegaly. In addition, its management does not require surgery since it can be resolved with chemotherapy.

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Bartonella species are gram-negative coccobacilli that are globally recognized as significant pathogens causing zoonotic infections. Among Bartonella species, B. henselae, B. quintana, and B. bacilliformis are prominent pathogens causing infections in humans, often manifesting as infective endocarditis. Bartonella endocarditis can pose diagnostic challenges due to its indolent presentation and limitations of standard microbiological culture techniques to identify the organism. We report a case of a 23-year-old male, who initially presented with the manifestations of hepatosplenic T-cell lymphoma, later diagnosed with blood culture-negative endocarditis caused by B. henselae. The patient had a complicated clinical course including pancytopenia, hepatosplenomegaly, and severe aortic valve regurgitation. Despite negative blood cultures, diagnostic clues included persistent fevers and bicuspid aortic valve with abscess. High Bartonella IgG titers (>1:800) supported the diagnosis. This case underscores the importance of considering Bartonella species in patients with suspected endocarditis, particularly in those with predisposing factors and negative blood cultures. Diagnosis relies heavily on serologic assays due to low sensitivity of conventional culture methods. Treatment involves a multidisciplinary approach with antibiotics and surgical intervention for optimal outcomes. Timely recognition and management are crucial to mitigate the high mortality associated with Bartonella endocarditis, and we hope this article offers insight for clinicians.

Hemangiosarcoma is an aggressive tumour that most frequently occurs in larger, middle-aged dogs of certain breeds. The spleen is the most commonly affected organ. The aim of this prospective therapy study was to evaluate the clinical effect of autologous, monocyte-derived dendritic cell (DC) therapy in canine hemangiosarcoma stage II after splenectomy. Dogs (n=452) diagnosed with splenic hemangiosarcoma that underwent splenectomy were enrolled. Of these, 42 dogs with stage II entered the DC therapy study. The median survival time for the total group of 42 dogs was 203 days. The median survival for the group (n=34) that received the full DC therapy (≥3 vaccines) was 256 days, with a 29 % one-year survival rate and a hazard ratio of 0.30, adjusted to age and bodyweight (P=0.010). We further observed a significant increase in DC yield after each application and demonstrated that DC yield at the beginning of treatment is significantly related to patient survival. While further evidence is needed, we conclude that autologous, monocyte-derived DC therapy is a viable alternative to standard treatment methods of canine splenic stage II hemangiosarcoma.

BACKGROUND: Extraskeletal osteosarcoma is an extremely rare malignancy that accounts for 1% of soft tissue sarcoma and 4.3% of all osteosarcoma. Extraskeletal osteosarcoma can develop in a patient between the ages of 48 and 60 years. The incidence of extraskeletal osteosarcoma is slightly higher in male patients than in females.
METHODS: A 50-year-old Caucasian male patient presented with a 6-month history of intermittent lower-left back pain that limits his activity. Prior ultrasonography and abdominal computed tomography scan showed a diagnosis of kidney stone and tumor in the lower-left abdomen. The computed tomography urography with contrast revealed a mass suspected as a left retroperitoneal malignant tumor. Hence, the tumor was resected through laparotomy and the patient continued with histopathological and immunohistochemistry examination with the result of extraskeletal osteosarcoma.
CONCLUSIONS: Extraskeletal osteosarcoma presents diagnostic challenges requiring multimodal examination, including histological and immunohistochemistry analyses. This case underscores the aggressive nature and poor prognosis despite undergoing the current suggested treatment.

OBJECTIVE: Splenic lesions might exhibit overlapping imaging features, varying from benign entities like cysts and hemangiomas to malignancies such as lymphoma and angiosarcoma. This meta-analysis aims to delineate imaging characteristics that distinguish malignant from benign splenic lesions.
METHODS: Adhering to PRISMA guidelines, we searched PubMed, Scopus, and Web of Science for studies on imaging features differentiating malignant from benign splenic lesions. We extracted data on splenic pathology and imaging characteristics and assessed the methodological quality via QUADAS-2. Odds ratio meta-analyses were performed using STATA (Version 17.0, Stata Corp, College Station, TX).
RESULTS: Portal phase hypoenhancement, hypovascular enhancement pattern, diffusion restriction, and late phase hypoenhancement, with odds ratios above 10, highly indicate malignancy. Other features suggestive of malignancy include solid morphology, lymphadenopathy, presence of perisplenic fluid, arterial hypoenhancement, hypoechogenicity on ultrasound, splenomegaly, and presence of multiple lesions. In contrast, cystic morphology, hypervascular-washout and hypervascular-persistent pattern of enhancement, late phase hyperenhancement, anechogenicity on ultrasound, portal phase hyperenhancement, well-defined borders, and calcification are in favour of benign pathology.
CONCLUSIONS: The study underscores the critical role of contrast-enhanced and diffusion-weighted imaging in distinguishing malignant from benign splenic lesions, emphasizing the role of features like portal phase hypoenhancement and restricted diffusion in diagnosing malignancies. Additionally, the study emphasizes the value of contrast-enhanced ultrasound, which allows for the visualization of key contrast-enhancement patterns without the risk of ionizing radiation exposure.

BACKGROUND: Dogs with retroperitoneal hemangiosarcoma (HSA) exhibit variable postoperative median survival times (MST).
OBJECTIVE: To retrospectively evaluate the prognostic value of selected tumour-related factors, such as tumour size, rupture, invasion into adjacent tissue, involvement of lymph node and distant metastasis, they were analysed in dogs with retroperitoneal HSA.
METHODS: Ten dogs with retroperitoneal HSA managed solely with surgical excision were reviewed and compared with spleen (71) and liver (9) HSA. The Kaplan-Meier method and log-rank analysis were used compare MSTs between factors. Multivariable Cox proportional-hazard analysis was used to compare differences between arising sites.
RESULTS: Retroperitoneal HSA showed comparatively longer postoperative MST compared with that of spleen and liver HSA and demonstrated significantly longer MST (p = 0.003) for tumours ≥5 cm (195 days) than <5 cm (70 days). Spleen HSA revealed significantly shorter MSTs in involvement of distant lymph nodes (23 days) and distant metastasis (39 days) than those in negative (83 days, p = 0.002 and 110 days, p < 0.001, respectively). Liver HSA also revealed significantly shorter MST (16.5 days compared with 98 days, p = 0.003) for distant metastasis. Additionally, hazard ratios (HRs) and their forest plot for overall HSA revealed as poor prognostic factors, arising sites (spleen; HR 2.78, p = 0.016 and liver; HR 3.62, p = 0.019), involvement of distant lymph nodes (HR 2.43, p = 0.014), and distant metastasis (HR 2.86, p < 0.001), and as better prognostic factor of tumour size ≥5 cm (HR 0.53, p = 0.037).
CONCLUSIONS: In combination with overall HSA, retroperitoneal HSA shows comparatively longer postoperative MST compared to spleen and liver HSA, associated with tumour size ≥5 cm suggesting better prognostic factor.

Isolated splenic lymphangiomas are rare benign lesions mostly seen in children are exceptionally rare in adults, often discovered incidentally due to their typically asymptomatic nature. This case report elaborates on the surgical excision of a rare splenic cystic lymphangioma in a 33-year-old woman, underscoring the diagnostic and therapeutic challenges these tumors pose. The patient\'s symptoms, abdominal pain and a palpable mass led to imaging through ultrasound and CT, which revealed a cystic splenic lesion. Total splenectomy was performed, revealing a large cystic mass, with pathological examination confirming a cystic lymphangioma. This case emphasizes the necessity of considering splenic lymphangiomas in adult patients presenting with splenic lesions. It also highlights the critical role of surgical interventions for definitive diagnosis and to prevent complications such as rupture and hemorrhage, thereby emphasizing on the complexity of managing rare splenic tumors.