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Highlights • Laparoscopic middle segment splenectomy for central splenic hemangioma is a new technique. • Laparoscopic middle segment splenectomy for central splenic hemangioma is very useful for some selected patients. • In patients with splenic artery bifurcation point far from the splenic hilus parenchyma and in those cases where the blood supply of the upper and lower pole splenic segments can be ensured, when the splenic tumor is resected, performing LPS and retaining some upper and lower splenic segments is possible to ensure sufficient residual spleen., Introduction Laparoscopic surgery plays an important role in the treatment of splenichematologic pathologies and solid lesions. Splenic hemangioma is the most common benign tumor of the spleen. In patients with benign splenic space-occupying lesions, laparoscopic partial splenectomy (LPS) has a lower incidence of postoperative complications than laparoscopic total splenectomy (LTS). Currently, no uniform standard for the indication of LPS is available. Presentation of case Herein, we report a case of hemangioma in a 23-year-old woman treated with LPS. After multidisciplinary evaluation, laparoscopic splenectomy was indicated in this case; upon evaluating the age of the patient and the affected spleen portion, a middle segment splenectomy was proposed, with preservation of the upper and lower pole. The intraoperative frozen section of the specimen was negative for malignancies. Discussion Surgery remains the first choice in the treatment of solid lesions of the spleen. In this case, the volume of splenic hemangioma was large and accumulated in the upper and lower segments of the spleen. After the middle branch of splenic artery was cut off, the tumor was completely contained in the middle of upper and lower ischemic lines splenic segment. We think that the central type of benign splenic space occupying is not an absolute contraindication of LPS. For the patients whose splenic artery bifurcation point is far from the splenic hilus parenchyma and in those cases where the blood supply of the upper and lower poles of the splenic segments can be ensured, when the splenic tumor is resected, performing LPS and retaining some parts of the upper and lower splenic segments is possible to ensure sufficient residual spleen.

A 59-year-old woman with newly diagnosed pulmonary nodules underwent both 18F-FDG and 68Ga-FAPI PET/CT. Both studies showed similarly increased uptake in pneumonia. However, only 68Ga-FAPI PET/CT showed increased uptake in splenic hemangioma, whereas FDG uptake in the splenic lesion is low. Our case illustrates that splenic hemangioma can also reveal increased FAPI activity.

Vascular neoplasms are among the most common conditions affecting the spleen. The majority of these are idiopathic, benign in nature and asymptomatic and therefore treated with a conservative management. Only rare cases cause splenomegaly and/or chronic consumption coagulopathies, thus requiring splenectomy. Among these, the most common is splenic hemangioma, followed by littoral cell angioma and lymphangioma. Peliosis is a peculiar tumor-like non-neoplastic vascular lesion that diffusely affects the spleen and frequently presents with concomitant hepatic involvement. As a distinctive feature, peliosis can occur as a secondary manifestation of infections, malignancies and in individuals using certain drugs. On the opposite spectrum of clinical behavior lies splenic angiosarcoma, a vascular endothelial malignancy with aggressive presentation and poor prognosis. In some cases the endothelial nature of this neoplasm may not be evident on routine histologic examination and immunohistochemistry is used to disclose such phenotype. The term hemangioendothelioma is rarely used to describe borderline vascular neoplasms which appear more aggressive than conventional hemangiomas, but that do not entirely fulfill the diagnostic criteria for angiosarcoma. Some of these neoplasms coexpress endothelial and histiocytic markers and therefore have been proposed as the borderline counterpart of littoral cell angioma. The existence of hemangioendothelioma as a diagnostic entity per se is debated and this diagnosis should be rendered with caution. The current review aims at highlighting the main histologic features of vascular neoplasms and non-neoplastic vascular lesions of the spleen.

Purpose The purpose of this study was to explore the safety and feasibility of laparoscopic partial splenectomy in treating splenic benign lesions (SBL). Materials and methods A total of 16 SBL patients clearly diagnosed before surgery (8 cases of splenic cysts, 5 cases of splenic lymphangioma, and 3 cases of splenic hemangioma) underwent 2D or 3D laparoscopic partial splenectomy from October 2013 to August 2016. The diameters of the SBL were 5 to 17 cm with an average of 8.7 cm; 12 cases were located in the upper pole of the spleen, and 4 cases were located in the lower pole of the spleen. Results All 16 patients successfully completed laparoscopic partial splenectomy, and no patients required conversion to an open operation. There were 3 cases of resection using the 3D laparoscope. There were 14 cases of resection of half of the spleen and 2 cases of partial resection of the lower spleen. The operation time was 110 to 195 minutes with an average time of 157 minutes. The amount of intraoperative blood loss was 80 to 200 mL, and no patients required blood transfusion. The postoperative abdominal drainage time was 3 to 5 days, and no abdominal bleeding, pancreatic leakage, subphrenic infection, or other complications occurred; the postoperative hospital stay lasted 5 to 6 days. Conclusions Laparoscopic partial splenectomy is safe and feasible for the treatment of splenic benign lesions.

Objective: A case is presented of a large splenic hemangioma masquerading as an adrenal tumor.Methods: Case report and literature review.Results: A 65-year-old woman with a history of lower-extremity edema and refractory hypertension was incidentally found to have an apparent 8.5-cm left-adrenal mass on magnetic resonance imaging, suspicious for adrenal cortical carcinoma. An adrenal functional evaluation was unrevealing. The patient underwent laparoscopic resection of the mass with splenectomy, as the tumor was inseparable from the spleen. Final pathology revealed a 9 × 8 × 7 cm splenic hemangioma. Splenic hemangioma is a rare tumor, associated with thrombocytopenia and anemia, which can rupture and bleed. Management varies from observation to partial or total splenectomy. Several factors, including the patient's history, presentation, and imaging, contributed to the incorrect pre-operative diagnosis of an adrenal tumor.Conclusion: Adrenal incidentalomas are common. However, it is important to be aware of other visceral tumors that can masquerade as an adrenal incidentaloma.Abbreviations: CD cluster of differentiation; CT computed tomography; FNA fine-needle aspiration

Highlights • Few cases of splenic hamartomas associated with thrombocytopenia have been reported. • Imaging findings in splenic hamartomas are non-specific, variable, and making definitive preoperative diagnosis difficult. • Surgery is necessary for diagnosis when malignancy cannot be ruled out. • Surgery may also improve symptoms of hypersplenism, including thrombocytopenia., Introduction Hamartomas are rare, benign tumors of the spleen. Few cases of splenic hamartomas associated with thrombocytopenia have been reported. Presentation of case An asymptomatic 64-year-old man with myelodysplastic syndrome was found to have a splenic tumor. Laboratory tests were significant for thrombocytopenia, with a platelet count of 7.8 × 104/μL. Ultrasonography showed splenomegaly (10.8 × 6.6 cm), and a hypoechoic splenic mass (8.0 × 7.0 cm). Color doppler ultrasound revealed blood flow within the mass, and the mass density was homogeneous on abdominal computed tomography (CT). Contrast-enhanced CT showed heterogeneous enhancement of the splenic mass during the arterial phase. Positron emission tomography (PET)-CT showed no significant fludeoxyglucose (FDG) accumulation within the mass. The differential diagnosis included splenic hamartoma, splenic hemangioma, splenomegaly associated with extramedullary hematopoiesis, and malignant tumor, including solitary splenic metastasis. A laparoscopic splenectomy was performed due to the possibility of malignancy, the presence of thrombocytopenia, and the risk of splenic rupture. The resected specimen showed a localized, well-demarcated, 8.0 × 7.0 cm splenic mass. Histological examination revealed abnormal red pulp proliferation and the absence of normal splenic structures. The patient’s post-operative course was uneventful. His platelet count improved on post-operative day 1 and he was discharged on post-operative day 9. He remained in good health with a normal platelet count one month after surgery. Discussion Making definitive preoperative diagnosis is difficult in splenic hamartomas. Surgery is necessary for diagnosis when malignancy cannot be ruled out. Conclusions Surgery may also improve symptoms of hypersplenism, including thrombocytopenia.

A 62-year-old man with prostate cancer underwent both F-PSMA-1007 and F-fluorocholine PET/CT studies for biochemical recurrence. F-PSMA-1007 PET/CT outperformed F-fluorocholine PET/CT in detecting lymph node metastases in the pelvis and retroperitoneum, whereas the former showed a focus of increased uptake in the spleen not seen on F-fluorocholine. The F-PSMA-1007 -avid lesion corresponded to a splenic hemangioma, which was initially detected in an MRI scan 10 years ago, unchanged in size. This case shows different features of F-PSMA-1007 and F-fluorocholine uptake, in an incidentally detected splenic hemangioma, alerting PET/CT reports for possible pitfall.

A 73-year-old man underwent a Ga-PSMA-HBED-CC 11 PET/CT for evaluation of rising PSA in the context of previous radical retropubic prostatectomy and salvage radiotherapy for prostatic adenocarcinoma. There was no definite PSMA avid recurrence in the prostatic bed. Intense PSMA avid uptake was seen in the anterior aspect of the spleen. Further characterization with multiplanar multisequence pre- and post-IV contrast MRI of the upper abdomen with arterial, portal venous, and delayed phase acquisitions revealed a lesion with appearances consistent with a splenic hemangioma.

Objective We discuss current knowledge and management of splenic hamartoma, an uncommon form of benign tumor. Summary background data A splenic hamartoma is a rare form of benign splenic mass, often found incidentally while working up other complaints, and is typically treated by surgical resection of the mass. In this case, we discuss the management of an incidentally found splenic hamartoma that was treated with laparoscopic partial splenectomy. Methods The patient presented in the Emergency Department complaining of periumbilical pain after having been struck in the abdomen with a soccer ball the previous day. Following a physical exam and blood work, an ultrasound was performed that revealed a hypoechoic area within the spleen. The patient received a diagnosis of gastroenteritis and an appointment for follow-up at 2 months. At 2-month follow-up, an ultrasound indicated that the mass had grown. The mass appeared consistent with a splenic hemangioma, so the patient was scheduled for laparoscopic partial splenectomy. Results The mass was completely resected without any complications. The patient had an uncomplicated postoperative course. Conclusions When there are no other indications for a total splenectomy and malignancy is unlikely, laparoscopic partial splenectomy appears to be a reasonable treatment modality for a splenic hamartoma.

A 69-year-old man with biochemical recurrence after radical prostatectomy underwent 6 months of androgen deprivation therapy. Upon discontinuation of androgen deprivation therapy, serum prostate-specific antigen rose again to 0.69 ng/mL, and F-DCFPyL PET/CT was performed to identify the site of recurrence. However, only an intense focus of uptake in the spleen could be found. Subsequent contrast-enhanced MRI findings were pathognomonic for splenic hemangioma.

RNA-sequencing of a splenic hemangioma with the karyotype 45~47,XX,t(3;6)(q26;p21) showed that this translocation generated a chimeric TBL1XR1-HMGA1 gene. This is the first time that this tumor has been subjected to genetic analysis, but the finding of an acquired clonal chromosome abnormality in cells cultured from the lesion and the presence of the TBL1XR1-HMGA1 fusion in them strongly favor the conclusion that splenic hemangiomas are of a neoplastic nature. Genomic PCR confirmed the presence of the TBL1XR1-HMGA1 fusion gene, and RT-PCR together with Sanger sequencing verified the presence of the fusion transcripts. The molecular consequences of the t(3;6) would be substantial. The cells carrying the translocation would retain only one functional copy of the wild-type TBL1XR1 gene while the other, rearranged allele could produce a putative truncated form of TBL1XR1 protein containing the LiSH and F-box-like domains. In the TBL1XR1-HMGA1 fusion transcript, furthermore, untranslated exons of HMGA1 are replaced by the first 5 exons of the TBL1XR1 gene. The result is that the entire coding region of HMGA1 comes under the control of the TBL1XR1 promoter, bringing about dysregulation of HMGA1. This is reminiscent of similar pathogenetic mechanisms involving high mobility genes in benign connective tissue tumors such as lipomas and leiomyomas.

Tumors of spleen are rare. The most common benign tumor of spleen is hemangioma. Most splenic hemangiomas (SH) tend to be discovered in mid-30s to mid-50s. SH are discovered incidentally in most of the cases as they are usually asymptomatic. Small asymptomatic SH can be managed with observation. The treatment options for large hemangiomas are embolization of the splenic arterial branch, splenectomy by laparotomy or laparoscopy. Partial splenectomy can be done if the lesion is small and located at the poles of the spleen. We present a rare case of splenic hemangioma in a 10-year-old boy who presented with a painless palpable mass in the left upper abdomen. On CT scan—A large well-defined cystic lesion with few thin enhancing septa within it is seen involving the spleen. Laparotomy was done followed by Partial splenectomy. The histopathology report was suggestive of Cavernous Hemangioma of spleen.

Littoral-cell angioma (LCA), a primary angioma which clinically belongs to splenic hemangioma, can be mostly found in normal spleen red sinus shore cells of reticuloendothelial cell system. The cells of LCA strongly express endothelial and tissue cell associated antigens that indicate a dual differentiation characteristic; whereas only endothelial cell markers are positive in normal spleen red sinus shore cells. Diagnosis of LCA relies on histopathology. Regular follow-up is needed to monitor recurrence and metastasis.

This study is a retrospective examination of diseases in collared peccaries that were diagnosed by the Veterinary Pathology Laboratory, Universidade Federal Rural do Semiárido. Necropsy and histological examination were performed from 2005 to 2010. Of the 50 necropsied collared peccaries, 24% died due to restraint and capture myopathy; 18% died from trauma; and the remainder was diagnosed with splenic hemangioma (6%), enterolithiasis (6%), gastritis (6%), gastric ulcer (4%), intestinal volvulus (4%), gastric volvulus (2%), mammary carcinoma (2%), polycystic kidney disease (2%), pyometra (2%), and suppurative bronchopneumonia (2%). Twelve animals remained undiagnosed, seven of which (14%) were in advanced autolytic condition and five of which (10%) had no gross or microscopic lesions that were compatible with disease. This paper describes illnesses that have not been reported in the collared peccary, focusing on their clinical and pathological aspects. Este trabalho teve por objetivo realizar um estudo retrospectivo sobre as doenças de catetos diagnosticadas pelo Laboratório de Patologia Veterinária da Universidade Federal Rural do Semi-Árido, através do exame de necropsia e histológico no período de 2005 a 2010. Dos 50 catetos submetidos à necropsia, as percentagens das enfermidades diagnosticadas foram: miopatia por captura e contenção (24%), morte por traumas (18%), hemangioma do baço (6%), enterolitíase (6%), gastrite (6%), úlcera gástrica (4%), vólvulo intestinal (4%), vólvulo gástrico (2%), carcinoma mamário (2%), doença renal policística (2%), piometra (2%) e broncopneumonia supurativa (2%). Onze (22%) animais permaneceram sem diagnóstico, dos quais sete (10%) apresentavam avançado estado autolítico e cinco (14%) não foram observadas lesões macroscópicas e microscópicas compatíveis com nenhuma enfermidade. Este estudo apresenta relatos de doenças ainda não descritas em catetos, com enfoque nos aspectos clínicos e patológicos.

A 36-year-old man who presented with a nosebleed and anemia was referred to our hospital. Laboratory test results showed platelet depletion, decreased levels of fibrinogen, and increased fibrinogen degeneration products. CT showed a 13-cm splenic tumor. T2 -weighted MRI revealed a high-intensity mass. We preoperatively diagnosed splenic hemangioma with chronic disseminated intravascular coagulation and scheduled an operation to relieve the disseminated intravascular coagulation. We also performed hand-assisted laparoscopic splenectomy to ensure easy handling of the splenomegaly. The resected specimen microscopically consisted of hemorrhages and hemangiomatous lesions, and multiple angiomatoid nodules were scattered and separated by fibrocollagenous stroma with inflammatory cells. Three types of vessels (capillaries, sinusoids and small veins) were contained in the angiomatoid nodules, and the pathological diagnosis was sclerosing angiomatoid nodular transformation. The results of this case suggest that we should consider sclerosing angiomatoid nodular transformation in the differential diagnosis of patients with splenic tumors, as sclerosing angiomatoid nodular transformation with hemangiomatous features may cause coagulation disorders for which splenectomy should be performed.

Splenic hemangiomas (SHs) are the most common benign neoplasms of the spleen. However, they are rare in the newborn period. We present an extremely rare case of congenital SH complicated by Kasabach-Merritt syndrome.A 2.93 kg male infant was delivered at term with a prenatal diagnosis of a left infrarenal mass diagnosed by ultrasound at 35 weeks of gestation. Magnetic resonance imaging demonstrated a well-defined splenic mass with multiple flow voids and scattered areas of high intensity suggestive of hemorrhage. He developed anemia, thrombocytopenia, and coagulopathy which required transfusion with packed red cells, platelets, cryoprecipitate, and fresh frozen plasma. Excision biopsy of the spleen led to resolution of anemia, thrombocytopenia, and coagulopathy. The diagnosis of SH was confirmed by histopathology. At 2 months outpatient follow-up, the patient was growing well without any evidence of tumor recurrence.Congenital SH is a rare entity that can be fatal if the potential complication of Kasabach-Merritt syndrome is not anticipated, evaluated, and promptly treated. Our patient had a favorable outcome with early surgical excision of the SH.

Spleen vascular tumors such as hemangiomas, albeit rare, can present during neonatal period with unexplained circulatory shock. We present a case of a newborn with refractory hypovolemic shock and acute abdomen that underwent emergency splenectomy due to spontaneous rupture of a splenic hemangioma.

Extramedullary hematopoiesis (EMH) is the production of mature blood elements outside of the bone marrow and can occur as a compensatory result of a marrow replacing process or from marrow space occupying lesions such as tumor or marrow fibrosis. EMH can also be induced by factors elicited by neoplasms, such as vascular endothelial growth factor (VEGF). Usually, EMH is a diffuse process most commonly observed in lymph nodes, liver, and spleen. Rarely, EMH can form a mass lesion. Although the spleen is a common site for diffuse EMH, it is a rare location for a mass forming EMH. Hemangiomas are the most common benign tumors of the spleen. A case of a discrete, 8 cm lesion was noted incidentally on CT scan in a 59-year-old man with no significant past medical history. Endoscopic ultrasound guided fine-needle aspiration (EUS FNA) biopsy was performed and cytologic examination revealed trilinear hematopoiesis, with the most distinctive elements being megakaryocytes and erythroid precursors. A diagnosis of EMH was made. On resection, the mass was a hemangioma with EMH. EUS guided FNA is a useful tool for diagnosing splenic masses. Awareness of EMH, both as a mass forming lesion and a feature associated with benign and malignant vascular lesions is important, especially in patients with hematologic malignancies or marrow replacing processes.

LTHOUGH unusual, hemangioma is the most common type of primary splenic neoplasm. Usually, splenic hemangioma  appears as solid mass, but sometimes it presents cystic component as well, which is difficult to discriminate from some other lesions, such as abscess, simple cyst, parasitic cyst, and lymphangioma. Preoperative diagnosis of splenic hemangioma mainly depends on imaging study (e.g. ultrasonography, CT, MRI). As the laparoscopic technique develops, laparoscopic splenectomy is becoming the standard treatment for benign tumor of the spleen. Compared with that procedure, laparoscopic partial splenectomy (LPS) has some unique advantages except for higher requirements for surgical skills. We described in this report a successful LPS in a patient who was misdiagnosed as splenic cyst initially.

Received March 2, 2010, from the Department of Ultrasound, Derriford Hospital, Plymouth, England. Revision requested April 2, 2010. Revised manuscript accepted for publication April 26, 2010. Address correspondence to Irving T. Wells, MBBS, BSc, MRCP, Peninsula Radiology Academy, Plymouth International Business Park, Plymouth, Devon PL6 5WR, England. E-mail: tobywells@doctors.net.uk Abbreviations CT, computed tomography e present a case in which contrastenhanced sonography was used to differentiate a benign splenic lesion from a possible isolated splenic metastasis in a patient with known malignancy. A 57-year-old patient with prostate cancer was referred for imaging before resection. His prostatic tumor was thought to be locally confined on magnetic resonance imaging, but staging computed tomography (CT) showed a focal splenic mass (Figure 1) without evidence of metastatic disease elsewhere. A sonographic examination with a sulfur hexafluoride microbubble contrast agent (SonoVue, Bracco SpA, Milan, Italy) was performed for further evaluation of the splenic lesion. Gray scale sonography showed a 7-cm well-defined slightly hyperechoic mass containing a few small cystic areas occupying most of the spleen (Figure 2A). Power Doppler analysis showed predominantly peripheral blood flow but also some central flow (Figure 2B). On contrast-enhanced sonography, the lesion showed enhancement in the arterial (Figure 2C) and portal venous (Figure 2D) phases and retained contrast on delayed images (Figure 2E). Its enhancement profile was similar to that of adjacent splenic parenchyma. The diagnoses based on the sonographic appearances were of a giant splenic hemangioma or hamartoma. A radiolabeled red cell scan was performed, which showed homogeneous uptake within the spleen, further supporting the diagnosis of a nonmalignant lesion.

Rationale: Complete loss of splenic function increases infection and cardiovascular disease risks, so there is growing emphasis on spleen-preserving treatments, such as laproscopic partial splenectomy (LPS). However, LPS carries higher risk for hemorrhage. Sequential splenic embolization can obliterate the perilesional vascular bed while preserving flow through healthy tissue, substantially reducing risk of uncontrolled hemorrhage during LPS. Preoperative partial splenic embolization (PSE) may soften the spleen and reduce its size, which enhances space exposure for laparoscopic operation. Furthermore, immediate LPS guaranties these effects of PSE and prevents abscess, non-traumatic splenic rupture, post-embolization syndrome, and other complications. In light of these advantages, we conducted combined PSE and LPS for a case of hemangioma. Patient concerns: The patient presented with left abdominal discomfort of >1 year. Diagnoses: Ultrasound examination at the outpatient clinic identified a space-occupying lesion in the spleen. Contrast-enhanced computed tomography scan of the upper abdomen revealed a hypodense lesion, approximately 33 × 21 mm in size, located in the upper pole of the spleen, suggesting possible hemangioma. Interventions: The patient was treated by preoperative PSE followed by LPS. Outcomes: Treatment resulted in only mild intraoperative hemorrhage, fast postoperative recovery, and no recurrence during follow-up. And the postoperative histopathology confirmed splenic cavernous hemangioma. Lessions: Preoperative PSE combined with LPS is an effective therapy for elective patients that minimizes intraoperative hemorrhage during laparoscopic surgery, reduces surgical risk, and enhances surgical safety.

Apesar de a esplenectomia ser eficaz na abordagem terapêutica de pacientes com hemangioma esplênico, esse procedimento é acompanhado de elevada morbidade e até mortalidade, principalmente devido à sepse, quando realizado em crianças e adolescentes com sistema imunitário ainda imaturo. Para prevenir os efeitos adversos da asplenia, propõe-se neste artigo a esplenectomia parcial, com a retirada apenas da região do hemangioma, mantendo o restante do baço e preservando suas importantes funções.

Objective Ultrasound contrast agents in conjunction with contrast specific imaging techniques, are increasingly accepted in clinical use for diagnostic imaging in several organs. Contrast enhanced sonography (CES) of second-generation contrast media have shown a spleen-specific uptake of the microbubble contrast agent. The aim of this review is to illustrate indications for the use of CES in patients with suspected (peri-)splenic pathology. Methods This review based on the experience of transcutaneous CES in 200 patients with (peri-)splenic pathology diagnosed by B-mode sonography at an internal medicine center. CES studies were performed with a contrast-devoted unit (Acuson, Sequoia, Siemens medical solution) that had contrast-specific, continuous-mode software. A low mechanical index was used. A sulfur hexafluoride-based microbubble contrast medium (Sonovue®, Bracco SpA, Milan, Italy) was injected. Results On our experience, there are several clinical conditions which may show an diagnostic advantage of CES in comparison to B-mode US. CES should be performed to investigate: (1) the perisplenic tumor to diagnose or exclude accessory spleen, (2) the small-sized spleen to diagnose functional asplenia/hyposplenia, (3) the inhomogenous spleen of unknown cause to diagnose focal lesions within the spleen, (4) the incidentally found hypoechoic splenic tumor to diagnose high vascular splenic hemangioma, (5) focal lesions suspect for splenic abscess, hematoma, infarction to confirme diagnosis, and (6) patients with abdominal trauma to diagnose or exclude splenic injuriy. Conclusion CES is of diagnostic value in several clinical circumstances to diagnose accessory spleen, functional asplenia, small-sized splenic involvement, high vascular splenic hemangioma, and vascular splenic pathology like splenic infarction, splenic abscess, and splenic laceration.

Introduction Splenic hamartoma is a primary benign tumor of the spleen, which is often found incidentally. Splenic hamartomas are very rare, with approximately 150 cases documented in the literature to date. They represent benign vascular proliferation. Histological findings consist of disorganized stroma and vascular channels of varying width, with or without lymphoid follicles. Case presentation We present the case of a 39-year-old Greek woman, with no significant medical history, who was diagnosed incidentally with an enormous splenic hamartoma on computed tomography, finally confirmed by surgery and histopathology. Hamartomas are benign lesions, and it is important to differentiate them from malignancy. Conclusion Hamartoma represents a rare vascular entity characterized by a cluster of differentiation 8-positive immunophenotype. It is usually asymptomatic but large hamartomas may present with symptoms such as hemopoetic disorders, which resolve after splenectomy. It is important for radiologists to be able to differentiate splenic hamartoma from malignant entities.

症例は63歳の男性.主訴は呼吸苦,黒色便と腹部膨満感.身体所見と血液検査で貧血を認め,鉄剤の経口投与を開始.5日後下痢と腹部膨満感を訴えて再度来院.腹部超音波検査で腹水を認め,利尿剤を開始したところ腹部膨満感は軽快.しかし,5日後腹部膨満感が再度出現したため当院受診.呼吸苦と顔色不良および黒色便を認め,消化管出血を疑い同日入院.血液検査で貧血を認めたが,上部消化管内視鏡検査では出血源を認めず.同日腹痛,血圧の低下を認め,貧血の増悪あり濃厚赤血球を投与.腹部単純CTで腹水を認め,その2日後の試験穿刺で血性腹水を確認.腹腔内出血と診断したが出血源は同定できず,同日状態が悪化し死亡した.病理解剖を行い,脾血管腫破裂痕を認め,同部からの出血による出血性ショックと診断した.生前に診断困難で,剖検により脾血管腫破裂と診断した1例を経験したので報告する., Non-traumatic rupture of the splenic tumor is a rare condition. We report a case of the non-traumatic rupture of splenic hemangioma in a 63 year-old male patient. His clinical presentations were dyspnea, abdominal fullness and tarry stool, which made us suspect gastrointestinal bleeding. However, no bleeding focus was observed on esophago-gastro-duodenoscopy. Ascites was detected on computed tomography and abdominal parencesis showed that it was bloody. On the basis of these observations, we diagnosed his illness as the intraabdominal bleeding, but could not clarify the origin of the bloody ascites. The patient was dead from hypovolemic shock due to intraabdominal bleeding. Pathological examination showed that the intraabdominal bleeding was caused by non-traumatic rupture of splenic hemangioma and that his tarry stool was caused by ischemic colitis.

Hemangiomas involving the spleen are rare and seldom symptomatic. Treatment options for large lesions usually consist of splenectomy, embolization, or both. Antiangiogenic treatment has not been reported previously as an effective alternative for this type of lesion. We report our experience of successfully using glucocorticoids in an infant with a large hemangioma of the spleen.

Primary vascular neoplasms of the spleen constitute the majority of nonhematolymphoid splenic tumors. The benign primary vascular tumors include hemangioma, hamartoma, and lymphangioma, whereas those of variable or uncertain biologic behavior include littoral cell angioma, hemangioendothelioma, and hemangiopericytoma. The primary malignant vascular neoplasm of the spleen is angiosarcoma. Peliosis is a rare lesion of unknown cause that is usually found incidentally in asymptomatic patients but may be associated with hematologic or metastatic disease. Although these vascular neoplasms of the spleen are uncommon, their importance lies in that they must be differentiated from the more common neoplastic disorders of the spleen, such as lymphoma and metastasis. The most common echogenic solid or complex cystic mass in an asymptomatic patient is splenic hemangioma. However, the imaging appearance of splenic hemangiomas may be complex, and differentiation of these lesions from malignant disease may not be possible. The diagnosis of splenic hamartoma may be suggested when findings of increased blood flow on color Doppler images are seen in association with a homogeneous solid echogenic mass. A large subcapsular solitary cystic abnormality discovered incidentally in a child in association with internal septations and tiny mural nodules favors the diagnosis of lymphangioma. Any invasion of the surrounding splenic parenchyma by a splenic lesion should indicate a more aggressive or malignant process. Evaluation of a focal splenic abnormality identified on sonograms should be followed up with computed tomography or magnetic resonance imaging with and without contrast material enhancement. Splenectomy may be required for definitive evaluation of a splenic mass with atypical features.