Splenic hamartomas in children

Splenic hamartomas(SHs)are uncommon,benign vascular lesions of unclear etiology and are mostly found incidentally on abdominal images,at surgery,or at autopsy.Since the first case description,in 1861,less than 50 pediatric SH cases have been reported in the literature.In this article,we have performed an analysis of all SH cases in children published in the literature to date and presented our case of an 8-year-old male with SH.These lesions in children were shown to cause symptoms more often than in the adult population.The observed SH sizes in children ranged from a few millimeters to 18 cm,and the symptomatic lesions were mostly larger or multiple.The most common clinical finding was splenomegaly.Signs of hypersplenism were present in children with a single SH larger than 4.5 cm(diameter range:4.5-18.0 cm)and in those with multiple hamartomas,ranging from a few millimeters to 5 cm.Eighty percent of patients with available laboratory findings had hematological abnormalities such as anemia,thrombocytopenia,or pancytopenia.Other symptoms and signs included abdominal pain,recurrent infections,fever,night sweats,lethargy,growth retardation,and weight loss.The use of multiple imaging modalities may suggest the preoperative diagnosis of a splenic mass in children and determine the therapeutic approach.However,the final diagnosis of SH relies on histopathological evaluation.Surgery,including total or partial splenectomy(PS),is the mainstay of SH management.Milickovic M et al.Splenomas in children WJCC https://www.wjgnet.com 1910 April 16,2024 Volume 12 Issue 11 Although total splenectomy carries a greater risk of overwhelming post-splenectomy infection than PS it has remained the most performed surgical procedure in children with SH.In the majority of pediatric patients with symptomatic SH,resolution of symptoms and resolution or improvement of cytopenias occurred after surgical treatment.

Precocious Puberty in a 5-Year-Old Girl with a Giant Hypothalamic Hamartoma Discovered Perinatally: Case Report

Background: Hypothalamic hamartoma is a rare non-neoplastic lesion, typically identified in early childhood during investigations for precocious puberty and/or gelastic seizures. However, cases of diagnosis even earlier or during fetal development have been documented. Case Presentation: A newborn girl was diagnosed with hydrocephalus during pregnancy. An MRI revealed a large oval hypothalamic process, which suggested a hypothalamic hamartoma. At the age of 2, she experienced alternating crying and laughing seizures, followed by a decrease in visual acuity. Due to involvement of the optic pathways, surgery was not performed and she underwent antiepileptic medicines and gamma knife radiotherapy. At the age of 5 years and 3 months, she presented with breast development and laboratory tests confirmed central precocious puberty. Quarterly injections of GnRH agonists have since been administered with favorable results. Conclusion: Early-diagnosed hypothalamic hamartomas require close monitoring, by an experienced multidisciplinary, to promptly detect and treat potential complications, especially precocious puberty, and prevent any undesirable impact on final height.

Combined hamartoma of the retina and retinal pigment epithelium:A case report

BACKGROUND Combined hamartoma of the retina and retinal pigment epithelium(CHRRPE)is a rare congenital benign tumor which is commonly monocular.Typical CHRRPE comprises slightly raised lesions at the posterior pole,with proliferation membrane often leading to vascular distortion.In severe cases,macular edema,macular hole,retinal detachment or vitreous hemorrhage may occur.Patients with atypical clinical manifestations are prone to misdiagnosis by inexperienced ophthalmologists.CASE SUMMARY A 33-year-old man reported onset of right eye blurred vision for one week prior.Anterior segment and intraocular pressure were normal in both eyes.Left eye fundus photography was normal.Right eye ophthalmoscopy showed vitreous hemorrhage and off-white raised retinal lesions below the optic disc.Proliferative membranes on the lesion surfaces resulted in superficial retinal detachment and tortuosity and occlusion of peripheral blood vessels.A horseshoe-like tear in the temporal periphery was surrounded by retinal detachment.Optical coherence tomography revealed retinal thickening at the focal site with structural disturbance indicated by high reflectance.Right eye ultrasound showed retinal thickening at the lesion,stretching and uplifting of the proliferative membrane,with moderately patchy echo at the optic disc edge.Cytokines and antibodies were detected in vitreous fluids during the operation to rule out other diseases.Fundus fluorescein angiography(FFA)at postoperative follow-up led to final diagnosis of CHRRPE.CONCLUSION FFA is helpful in diagnosing retinal and retinal pigment epithelial combined hamartoma.In addition,other cytokine and etiological tests facilitate further differential diagnosis to rule out other suspected diseases.

Incidentally discovered asymptomatic splenic hamartoma misdiagnosed as an aneurysm:A case report

BACKGROUND Splenic hamartoma(SH)is a rare,benign vascular proliferation that is often found incidentally.It may be misdiagnosed as a splenic aneurysm or splenic malignancy.CASE SUMMARY A 21-year-old male patient was admitted to our hospital with a complaint of an incidentally discovered asymptomatic splenic space-occupying lesion for 2 wk.Abdominal computed tomography(CT)scan showed a circular low-density shadow in the hilum of the spleen.Contrast-enhanced CT revealed an aneurysm located in the hilum of the spleen before operation.Laparoscopic splenectomy was performed and postoperative pathology revealed the presence of SH.CONCLUSION Imaging studies are insufficient for the differential diagnosis of SH from other diseases,and laparoscopic splenectomy is a less invasive procedure and useful for the diagnostic purpose as well.

多重稳固的胰腺的 hamartomas : 一份案例报告和文学的评论

Non-neoplastic tumor-like lesions in the pancreas are uncommon.Here,we present a case of multiple solid pancreatic hamartomas in a 78-year-old Japanese woman.Her computed tomography revealed a pancreatic mass,measuring 1.8 cm in maximum diameter.However,no symptoms were found.She was not an alcoholic and had no history of pancreatitis.The patient underwent a pancreatoduodenectomy,and three well-demarcated solid nodules measuring 1.7 cm,0.4 cm,and 0.3 cm in diameter were found in the pancreatic head.Microscopically,the lesions were composed of non-neoplastic,disarranged acinar cells and ducts embedded in a sclerotic stroma with elongated spindle cells that lacked discrete islets.The stromal spindle cells were immunoreactive for CD34 and CD117.The histological diagnosis was multiple solid hamartomas of the pancreas.There has been no recurrence 30 mo after surgery.So far,18 cases of pancreatic hamartoma have been reported in the English literature,including our case.Six out of these 18 cases seemed to fit the criteria of solid pancreatic hamartoma.Although the number of cases was limited,solid pancreatic hamartomas seem to be benign tumor-like lesions,which are found incidentally in healthy middle-aged adults,but occasionally involve the whole pancreas,resulting in a poor prognosis.Solid pancreatic hamartoma was sometimes associated with minor pancreatic abnormality,and multiple small lesions other than the main tumors were detected in a small number of cases.From these findings,one may speculate that solid pancreatic hamartoma could be the result of a malformation during the development of the pancreas.

Symptomatic multinodular splenic hamartoma preoperatively suspected as metastatic tumor:A case report

Splenic hamartoma(SH)is a rare benign tumor usually detected accidentally,which is composed of an aberrant mixture of normal splenic elements.Here,we report a case of 54-year-old man who presented with symptomatic multinodular SH and was admitted initially for thrombocytopenia and anemia.Physical examination revealed that the patients had an anemic appearance and palpable spleen,extending 10 cm below the costal margin.Preoperative ultrasound and computed tomography(CT)indicated splenomegaly with multinodular lesions.On enhanced CT scanning,during the arterial phase,the lesions demonstrated inhomogeneous enhancement,and in the portal phase the lesions were more hyperdense than the splenic parenchyma.The images were highly suggestive of a metastatic tumor.Splenectomy was performed 1 wk later.The tumor was eventually diagnosed as SH according to the morphological features and immunohistochemical detection,by which CD34 was positive in lining cells and some spindle cells,vimentin was positive in the tumor,factor-Ⅷ-related antigen was positive multifocally in lining cells,and smooth muscle actin was positive in some spindle cells.Thrombocytopenia and anemia were cured after splenectomy.

Splenic hamartoma: A case report and review of the literature

Splenic hamartoma is a rare benign malformation, composed of an anomalous mixture of normal splenic elements, often found incidentally while working up other complaints or at autopsy. A splenic mass was incidentally found while evaluating the effects of a traffic accident in a 63-year-old woman. Abdominal computed tomography revealed a well-defined splenic mass with rim enhancement. The patient underwent splenectomy. The resected spleen contained a well-defined mass lesion measuring 3.5 cm × 3.0 cm. Microscopic examination revealed disorganized slit-like vascular channels lined by plump endothelial cells without atypia. The cells lining the vascular channels were positive for CD8, CD31, CD34 and vimentin. Endothelial cells that are positive for CD8 are a key feature that differentiates hamartoma from other vascular lesions of the spleen. Although this tumor is very rare, it must be included in the differential diagnosis of splenic mass-forming lesions.

Liver transplantation for a giant mesenchymal hamartoma of the liver in an adult: Case report and review of the literature

Mesenchymal hamartomas of the liver(MHLs) in adults are rare and potentially premalignant lesions, which present as solid/cystic neoplasms. We report a rare case of orthotopic liver transplantation in a patient with a giant MHL. In 2013, a 34-year-old female sought medical advice after a 2-year history of progressive abdominal distention and respiratory distress. Physical examination revealed an extensive mass in the abdomen. Computed tomography(CT) of her abdomen revealed multiple liver cysts, with the diameter of largest cyst being 16 cm × 14 cm. The liver hilar structures were not clearly displayed. The adjacent organs were compressed and displaced. Initial laboratory tests, including biochemical investigations and coagulation profile, were unremarkable. Tumor markers, including levels of AFP, CEA and CA19-9, were within the normal ranges. The patient underwent orthotopic liver transplantation in November 2013, the liver being procured from a 40-year-old man after cardiac death following traumatic brain injury. Warm ischemic time was 7.5 min and cold ischemic time was 3 h. The recipient underwent classical orthotopic liver transplantation. The recipient operative procedure took 8.5 h, the anhepatic phase lasting for 1 h without the use of venovenous bypass. The immunosuppressive regimen includedintraoperative induction with basiliximab and high-dose methylprednisolone, and postoperative maintenance with tacrolimus, mycophenolate mofetil, and prednisone. The recipient's diseased liver weighed 21 kg(dry weight) and measured 41 cm × 32 cm × 31 cm. Histopathological examination confirmed the diagnosis of an MHL. The patient did not experience any acute rejection episode or other complication. All the laboratory tests returned to normal within one month after surgery. Three months after transplantation, the immunosuppressive therapy was reduced to tacrolimus monotherapy, and the T-tube was removed after cholangiography showed no abnormalities. Twelve months after transplantation, the patient remains well and is fulfilling all normal activities. Adult giant MHL is extremely rare. Symptoms, physical signs, laboratory results, and radiographic imaging are nonspecific and inconclusive. Surgical excision of the lesion is imperative to make a definite diagnosis and as a cure. Liver transplantation should be considered as an option in the treatment of a non-resectable MHL.

Laparoscopic splenectomy for splenic hamartoma:Case management and clinical consequences

Splenic hamartoma is a rare benign tumor,and although minimally invasive surgery may be suitable for this condition,there have only been 2 previous reports of laparoscopic surgery.Here we report the third case of splenic hamartoma managed by laparoscopic splenectomy.A 37-year-old male was incidentally diagnosed by abdominal ultrasonography with a hypoechoic mass measuring 2.5 cm × 2.4 cm in the spleen.Color Doppler sonography showed multiple flow signals within the mass and contrast-enhanced computed tomography revealed strong enhancement of the lesion.On T1-and T2-weighted magnetic resonance images,the splenic mass was demonstrated as isointense and hyperintense respectively.Although a malignant tumor could not be ruled out,a hand-assisted laparoscopic splenectomy was performed because the splenic mass was limited in size and had not invaded adjacent organs.The pathological diagnosis was splenic hamartoma.The postoperative course was uneventful and the patient was discharged by the seventh postoperative day.Although splenic hamartomas have some specific imaging features,more reports and analyses of these cases are required to increase the reliability of the diagnosis and management.Hand-assisted laparoscopic splenectomy may play a pivotal role in the postoperative diagnosis and management of this condition.

Bile duct hamartomas-the von Meyenburg complex

Hamartomas of the bile duct (von Meyenburg complex) are benign neoplasms of the liver, constituted histologically cystic dilatations of the bile duct, encompassed by fibrous stroma. We report a 42-year-old female patient with symptomatic cholecystitis, whose gross and ultrasonic appearance suggestive of multiple liver metastases. Magnetic resonance imaging and liver biopsy are the gold standards for diagnosis of this rare hepatobiliary condition.

Retroperitoneoscopic Microwave Ablation of Renal Hamartoma:Middle-term Results

The safety and efficacy of retroperitoneoscopic microwave ablation (MWA) in the treatment of renal hamartoma were evaluated.From July 2007 to July 2009, a total of 16 cases of renal hamartoma were treated with retroperitoneoscopic MWA.Peri-and post-operative findings were observed.Middle-term efficacy was assessed by contrast-enhanced computerized tomography (CT) in follow-up period.All patients received MWA of 1-5 points.The mean operative time was 85 min and the mean blood loss was 65 mL.During a median follow-up of 16 months, no evidence of disease recurrence was observed despite of incomplete ablation in 1 case.Retroperitoneoscopic MWA is a relatively simple procedure with less impact to renal function and less complication.The outcome of middle-term follow-up is satisfactory.Thus, retroperitoneoscopic MWA appears to be a safe and effective technique for renal hamartoma in selected patients.

Optical coherence tomography-angiography of juxtapapillary hamartoma

Dear Editor,I＇m Dr. Gilda Cennamo from the Eye Clinic of Department of Neurosciences, Reproductive Sciences and Dentistry,University of Naples Federico II, Naples, Italy. I write to present four cases ofjuxtapapillary hamartoma evaluated with non-invasive optical coherence tomography-angiography （OCT-A）.

A Case of COPD with Left Endobronchial Hamartoma by Bronchoscopic Intervention

Objective: Exploration for chronic obstructive pulmonary disease with branched air lumen hamartoma by bronchoscoptic treatment. Material and Method: An old man was referred to our hospital with shortness of breath for many years. Clinical evidence emphysema on respiratory examination, lung function and chest X-ray gave rise to concern. He was diagnosed as COPD and treated with ICS + LABA. But the shortness of breath is still existed. Initial assessment by CT suggested a left lower lobe collapse. Then bronchoscope identified a solid abnormality in main left bronchus. The pathology showed a benign neoplasm. Because of intolerance of operation, the patient was treated by brochoscopic intervention. The endoscopic intervention included resection by electrosurgical snare, electrocautery, argon plasma coagulation (APC). Result: After intervention, the neoplasm was partly removed and the pathological result was endobrochial hematoma. After treatment, the patient’s panting had taken a turn for the better. After one month later, the patient’s lung function improved a lot. Conclusion: In conclusion, endobronchial hamartomas are one of benign neoplasms. It can cause persistent bronchial obstruction and recurrent pneumonias. In this case, invasive endoscopic treatment provides an excellent outcome.

Adult intussusception secondary to an ileum hamartoma

Intussusception is a rare condition in the adult population.However,in contrast to its presentation in children,an identifiable etiology is found in the majority of cases.Clinical manifestations of adult intussusception are non-specific and patients may present with acute,intermittent or chronic symptoms,predominantly those of intestinal obstruction.A 27-year-old male patient with recurrent abdominal pain secondary to intussusception is herein reported.The clinical presentation and ultrasonographic findings led to the diagnosis.At laparotomy,an ileal hamartoma was found as the lead point of the intussusception.Surgical management and histopathologic studies are described.A recurrent intestinal obstruction and classic ultrasound findings may lead to the diagnosis of intussusception but surgical exploration remains essential.The principle of resection without reduction is well established.

Portal hypertension in a patient with biliary hamartomas: A case report

BACKGROUND Biliary hamartomas(BH)are a rare benign disease caused by malformation of the intrahepatic bile ducts.BH are occasionally diagnosed,but often lack obvious clinical symptoms.They are usually diagnosed by biopsy and imaging tests in clinical practice.Few studies have reported the association of BH with portal hypertension.CASE SUMMARY A 40-year-old man was repeatedly admitted to our hospital due to hematochezia.The source of bleeding was considered to be gastroesophageal varices and portal hypertensive gastropathy by endoscopy.He had no history of hepatitis virus infection,alcohol abuse,drug-induced liver injury,or autoimmune liver disease.He underwent magnetic resonance imaging,which showed rounded,irregular,low-signal-T1 and high-signal-T2 lesions diffusely distributed on the liver,that were not communicated with the biliary system on magnetic resonance cholangiopancreatography.According to the imaging examination,the patient was considered to have a diagnosis of BH with portal hypertension.CONCLUSION Based on the present case report,BH may be a potential etiology of portal hypertension.

Large cystic-solid pulmonary hamartoma:A case report

BACKGROUND It now seems that all pulmonary hamartomas(PHs)are large cystic-solid lesions that are difficult to diagnose.However,few cases of large cystic-solid PHs have been reported.The present case report presents a large cystic-solid PH and provides a literature review of the imaging features,formation mechanism and histopathological basis of PHs.CASE SUMMARY A 53-year-old woman with no clinical symptoms underwent a chest computed tomography(CT)examination at our hospital.Nonenhanced CT images revealed a large,flat tumor with multiple air-containing cysts in the left thoracic cavity and a cystic part confined to the medial side of the tumor;the solid part of the tumor showed abundant fat and lamellar soft tissue components.Multiple small blood vessels were detected in the solid part of the tumor on contrast-enhanced CT images.Given the large size of the lesion,the patient elected to undergo surgery.Histological examination revealed PH.A detailed review of the patient’s CT imaging showed that the lesion had a small vascular pedicle to the left lower lobe,which was a clue to its lung tissue histological origin.According to immunohistochemical staining,the confined multiple air-containing cysts were caused by the entrapment of respiratory/alveolar epithelium.CONCLUSION This case shows the imaging manifestations of a large PH.Heightened awareness of its formation mechanism and histopathological basis may alert radiologists to consider this diagnosis in their daily workflow.

Fibrous hamartoma of infancy with bone destruction of the tibia:A case report

BACKGROUND Fibrous hamartoma of infancy(FHI)is a rare disease of infancy with unknown etiology.The disease mainly involves soft tissue,has no specific clinical manifestations,and is difficult to diagnose.At present,the diagnosis is mainly confirmed by histopathological examination,and the main treatment is surgical resection of the pathological tissue,which is prone to recurrence.CASE SUMMARY A five-month-old female patient was admitted to our hospital with swelling in the right calf.Two biopsies were performed in our hospital and another hospital,respectively,confirming the diagnosis as fibrous hamartoma.After exclusion of surgical contraindications,resection was performed with clear margins of 1 cm.Radiographic examination showed tumor recurrence more than four months after the operation,and surgery was performed again to extend the resection margins to 1.5 cm.The patient is recovering well,and after a follow-up of 36 mo,shows no signs of recurrence.CONCLUSION Our case report demonstrates that FHI should be considered in the differential diagnosis for a lower extremity mass with bone destruction.For FHI with bone destruction and unclear boundaries,excision margins of 1.5 cm could be superior to margins of 1 cm.

Intrahepatic multicystic biliary hamartoma:A case report

BACKGROUND Multicystic biliary hamartoma(MCBH)is a rare hamartomatous nodule of the liver,which has recently been described as a new category of hepatic nodular cystic lesion.Most of them are benign.The imaging findings are similar to those of many other hepatic cystic lesions,but MCBH also has some notable features,such as large cysts,smooth cyst walls,and lack of communication with the hepatic duct.Due to the non-specific radiology,preoperative diagnosis is difficult,and is usually diagnosed by postoperative pathology.Complete resection is the best treatment option,and the postoperative prognosis is good.CASE SUMMARY When the patients have MCBH,the symptoms may not very typical,and they require a combination of imaging and pathology for diagnosis.Under normal circumstances,the prognosis of MCBH is good.However,in patients with MCBH,more cases need to be observed for verification.CONCLUSION When the patients have MCBH,the symptoms may not very typical,and they require a combination of imaging and pathology for diagnosis.Under normal circumstances,the prognosis of MCBH is good.However,in patients with MCBH,more cases need to be observed for verification.

Fibrous Hamartoma of Infancy at the Hand: A Case Report with Complications

Fibrous Hamartoma of Infancy is a benign subdermal tumor most commonly presenting at the proximal appendages. Here we present a case of Fibrous Hamatoma of Infancy at the hand with discussion of its challenging excision and post-operative complications. This case highlights the delicate and challenging nature of pediatric hand surgery.

Respiratory Epithelial Adenomatoid Hamartoma of the Nasal Cavity: A Case Report

Respiratory epithelial adenomatoid hamartoma (REAH) is an uncommon lesion of the upper aerodigestive tract. It usually presents as a nasal or as a sinus or nasopharyngeal mass. The potential danger associated with REAH is the risk of misdiagnosis. Recognition and awareness of this benign lesion is necessary to distinguish it from inverted papilloma and adenocarcinoma. This is important to avoid aggressive surgery. We report a case of REAH diagnosed on histopathology.