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.

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.

Multiple solid pancreatic hamartomas:A case report and review of the literature

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.

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.

Esophageal early basaloid squamous carcinoma with unusual narrowband imaging magnified endoscopy findings

Basaloid squamous carcinoma (BSC) is a rare variant of esophageal cancer. There are very few reports of “early” BSC. Here we report a case of early BSC with unusual findings by narrowband imaging magnified endoscopy (NBI-ME). A 70-year-old man with a middle thoracic esophageal tumor was referred to our hospital. White-light endoscopy revealed a reddish depressed lesion 5 mm in diameter having a subepithelial tumor-like prominence with a gentle rising slope. NBI-ME revealed irregular loop-shaped microvessels coexistent with thick irregularly branched non-looped vessels. Iodine staining revealed a pale brown lesion. We performed endoscopic submucosal dissection for diagnostic treatment. Histologic examination showed the proliferation of basal cell-like hyperchromatic tumor cells in the lamina propria and with slight invasion into the submucosa at a depth of 320 μm. The tumor cells formed solid nests and microcystic structures, containing an Alcian blue-positive mucoid matrix. The surface was covered with squamous epithelium without cellular atypia. Thin vessels were observed in the intra-epithelial papilla and thick vessels were observed around the solid nests beneath the epithelium. Based on these findings together, we diagnosed the lesion as BSC. In this case, the NBI-ME findings differed from those of typical squamous cell carcinoma in that both non-invasive cancer-like irregular loop-shaped microvessels coexisted with massively invasive cancer-like thick non-looped vessels. We speculate that the looped and non-looped vessels observed by NBI-ME histologically corresponded to thin vessels in the intra-epithelial papilla and thick vessels around the tumor nests, respectively. These NBI-ME findings might be a feature of early esophageal BSC.

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.

Basket pattern blood flow signals discovered in a case of splenic hamartoma by power Doppler ultrasonography

We present the gray-scale ultrasonography （GSUS）, power Doppler ultrasonography （PDUS）, abdominal computed tomography （CT）, and magnetic resonance imaging （MRI） findings for a case of splenic hamartoma in a 27-year-old man, showing a φ 50 mm homogeneous, iso- and hypo-echoic splenic mass with evidence of a small plural cystic lesion. This splenic hamartoma showed increased vascularity on power Doppler sonograms. PDUS showed multiple circular blood flow signals inside the mass （i.e. a basket pattern）, which was consistent with the small plural cystic lesion shown by GSUS. Spectral analysis also confirmed arterial and venous flow. CT scans showed that the mass had lowdensity relative to the normal spleen and MRI showed that the mass was isodense, relative to the normal spleen. Therefore, CT and MRI are not useful for the diagnosis of splenic hamartoma. Ultrasonography can be used to diagnose splenic hamartoma without administration of a contrast matedal and therefore is an indispensable method for the diagnosis of splenic hamartoma.

Imaging findings of biliary hamartomas

AIM： To evaluate the imaging findings of biliary hamartomas （yon Meyenburg complexes, VMCs） and discuss the differential diagnosis with other related diseases. METHODS： Imaging findings of biliary hamartomas on ultrasonography （US）, computed tomography （C-T）, magnetic resonance imaging （MRI）, MR cholangiopancreatography （MRCP）and hepatobiliary scintigraphy were retrospectively analyzed in six patients. RESULTS： On ultrasound images, five of the six cases showed multiple small hyper- and hypo-echoic lesions with comet-tail echoes, especially when magnified by US with the usage of zoom function. In all the six cases, multiple tiny hypodense lesions less than 10 mm in diameter were revealed as scattered throughout the liver with no enhancement on CT. These tiny lesions were demonstrated to be hyper- and hypo-intensity on T2- and TI-weighed images, respectively, in three patients who underwent MRI examinations. MRCP was performed in two patients, and clearly showed multiple tiny irregular- and round-shaped hyper-intensity lesions. MRCP and hepatobiliary scintigraphy showed normal appearances of intra- and extra-hepatic bile ducts in two and one patients, respectively. CONCLUSION： Imaging modalities are useful in the diagnosis and differential diagnosis of VMCs. A correct diagnosis might be obtained when typical imaging findings are present even without a histological confirmation.

Three cases of Solitary Peutz-Jeghers-type hamartomatous polyp in the duodenum

A solitary Peutz-Jeghers-type hamartomatous polyp in the duodenum is rare, and few reports have described its characteristic endoscopic features. We describe three cases of solitary Peutz-Jeghers-type hamartomatous polyp and their endoscopic findings in detail. The polyp in all of our three cases showed an irregularly lobular or nodular surface, whereas adenomas often show a regularly nodular or granular surface. The color of the polyp was whitish in all of our cases. In the present cases, close observation by endoscopy revealed that the solitary Peutz-Jeghers-type hamartomatous polyps looked whitish because of the presence of diffusely scattered white spots on the surface of the polyps. Duodenal polyps that exhibit the aforementioned endoscopic characteristics may be diagnosed as Peutz-Jeghers-type hamartomatous polyps and treated by polypectomy because of the malignant potential.

A case of EMRC for basaloid squamous carcinoma of the cervical esophagus

Basaloid squamous carcinoma (BSC) of the esophagus is a rare esophageal tumor. A 79-year-old man with a history of proximal gastrectomy for gastric adenocarcinoma in 2000 was followed-up by esophagogastroduodenoscopy (EGD) annually. In June 2010, EGD revealed a new protruding lesion in the cervical esophagus. The small lesion was approximately 5 mm in size. A biopsy specimen showed poorly differentiated squamous cell carcinoma. We performed endoscopic mucosal resection using a cap-fi tted endoscope (EMRC). The histological diagnosis of the endoscopically resected specimen was BSC and the invasion depth was limited to the muscularis mucosae. Horizontal and vertical margins were negative. We report the case of superfi cial BSC in the cervical esophagus successfully resected by EMRC.

Spontaneous rupture of splenic hamartoma in a patient with hepatitis C virus-related cirrhosis and portal hypertension:A case report and review of the literature

Spontaneous rupture is a rare complication of splenic hamartoma. A review of the literature revealed only four such cases. To the best of our knowledge, this is the first report of spontaneous rupture of splenic hamartoma associated with liver cirrhosis and portal hypertension. A 53-year-old woman, who was followed up for aortic dissection and hepatitis C virus （HCV）-related liver cirrhosis, was referred with sudden left chest and shoulder pain. An abdominal ultrasound showed intraabdominal bleeding, and computed tomography indicated rupture of a splenic tumor. Emergent splenectomy was carried out. The postoperative course was uneventful, and the patient was discharged on the 13th postoperative day. Pathology revealed the tumor to be a ruptured splenic hamartoma. The non-tumorous splenic parenchyma revealed congestive changes. We consider that the presence of liver cirrhosis and portal hypertension are risk factors for spontaneous rupture of the splenic hamartoma.

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）.

Bile duct hamartomas(von Mayenburg complexes) mimicking liver metastases from bile duct cancer:MRC findings

We present a case of a 72-year-old man with a common bile duct cancer, who was initially believed to have multiple liver metastases based on computed tomography findings, and in whom magnetic resonance cholangiography （MRC） revealed a diagnosis of bile duct hamartomas. At exploration for pancreaticoduodenectomy, liver palpation revealed disseminated nodules at the surface of the liver. These nodules showed gray-white nodular lesions of about 0.5 cm in diameter scattered on the surface of both liver lobes, which were looked like multiple liver metastases from bile duct cancer. Frozen section of the liver biopsy disclosed multiple bile ducts with slightly dilated lumens embedded in the collagenous stroma characteristics of multiple bile duct hamartomas （BDHs）. Only two reports have described the MRC features of bile duct hamartomas. Of all imaging procedures, MRC provides the most relevant features for the imaging diagnosis of bile duct hamartomas.

The value of endoscopic ultrasonography on diagnosis and treatment of esophageal hamartoma

Objective:To examine the values of endoscopic ultrasonography (EUS) on diagnosis and treatment of esophageal hamartoma. Methods:We compared and analyzed various kinds of imaging examinations such as barium esophagram, con-trast-enhanced computed tomography (CT) and conventional gastroscopy in retrospectively reviewing the clinical data of an esophageal hamartoma patient seen in our clinic in the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China. Having received various imaging examinations, this patient was finally diagnosed with esophageal hamartoma and underwent gastroscopic resection of hamartoma with the diagnostic information obtained from EUS. The patient had been regularly followed up for 13 months after treatment. Results: Barium esophagram, CT and conventional gastroscopy detected the lesion, but were unable to distinguish it from common esophagopolypus and other submucosal lesions, and unable to determine etiopathogenisis. EUS detected the hamartoma and identified its internal structure, echo, exact size, depth of invasion, origin and the relationship between adjacent tissues and organs, differentiating the lesion from other submucosal tumors and clearly defining the diagnosis. EUS-guided fine needle aspiration (FNA) also helped to identify the etiological diagnosis. Conclusion: EUS was superior to other imaging means in diagnosis and treatment of hamartoma.

DIAGNOSIS AND TREATMENT OF PULMONARY HAMARTOMA

From 1970 to 1997, 67 patients with pulmonary hamartoma were operated on in our hospital. There were 38 men and 29 women with a M/F ratio of 1. 3: 1. The mean age was 47 years (range 21 to 82 years). The peak incidence was 40 to 60 years. 39% Patients had pulmonary symptoms: hemoptysis, cough, phlegm and chest pain. All were solitary. 68. 7% tumors were located in right lung and 64. 2% in upper or middle lobe. 80. 6% hamartomas were less than 3 cm in transverse diameter. On chest films, the opacity was homogeneous in 79.1% cases, heterogeneous in 20.9% and calcifications in 9.0%. The vast majority of shadows were heterogeneous on CT films. Operation is the best treatment for hamartomas. Postoperative mortality was 1. 5%. There was none recurrences and canceration during the follow-up (mean 14 years).

Mucosal Schwann cell “Hamartoma”:A new entity?

Schwannoma is a well-described,benign nerve sheath tumor of the soft tissue,but is rare in the gastrointestinal tract.Gastrointestinal schwannomas are often incidentally discovered as small polypoid intraluminal lesions.In this report,we describe the clinicopathologic and immunohistochemical features of a distinctive neural mucosal polyp composed of a diffuse cellular proliferation of uniform bland spindled cells in the lamina propria that entraps the colonic crypts.Immunohistochemical analysis revealed strong and diffuse positivity for the S-100 protein.To avoid confusion of these solitary colorectal polyps containing pure spindled Schwann cell proliferation in the lamina propria with neural lesions that have significant association with inherited syndromes,it is better to use the designation "mucosal Schwann hamartoma".