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Milickovic M, Rasic P, Cvejic S, Bozic D, Savic D, Mijovic T, Cvetinovic S, Djuricic SM. Splenic hamartomas in children. World J Clin Cases 2024; 12:1909-1917. [PMID: 38660549 PMCID: PMC11036520 DOI: 10.12998/wjcc.v12.i11.1909] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/27/2024] [Revised: 03/06/2024] [Accepted: 03/22/2024] [Indexed: 04/11/2024] Open
Abstract
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. 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.
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Affiliation(s)
- Maja Milickovic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Petar Rasic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
| | - Sofija Cvejic
- Department of Radiology, The Children’s University Hospital, Belgrade 11000, Serbia
| | - Dejana Bozic
- Department of Clinical Pathology, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
| | - Djordje Savic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Tanja Mijovic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
| | - Sava Cvetinovic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
| | - Slavisa M Djuricic
- Faculty of Medicine, University of Banja Luka, Banja Luka 78000, Bosnia and Herzegovina
- Department of Clinical Pathology, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
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Sabra TA, Maher A, AlRashidi R, Ibrahim H. A splenic hamartoma: Adding a new case to the literature: A case report. Int J Surg Case Rep 2022; 90:106647. [PMID: 34979425 PMCID: PMC8732791 DOI: 10.1016/j.ijscr.2021.106647] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/09/2021] [Revised: 11/21/2021] [Accepted: 11/28/2021] [Indexed: 11/30/2022] Open
Abstract
Introduction Splenic tumours are relatively rare and include malignancies such as lymphomas, angiosarcomas, plasmacytomas, primary malignant fibrous histiocytomas, and splenic metastases. Benign tumours of the spleen such as hemangiomas, cysts, and inflammatory pseudotumours are very rare (Kaza et al., 2010, PisaniCeretti et al., 2012) [1], [2]. There are fewer than 160 cases of splenic hamartoma or splenomas having been reported in the literature (Basso et al., 2012) [3]. Only 20% of the cases were detected in children (Abramowsky et al., 2004) [4]. Although multi-modality imaging findings were described preoperatively, the final diagnosis was splenic hamartoma based on histology and immunohistochemistry. Case report Here, we report a case of a14 year old child left upper quadrant abdominal pain and worsening sickness. Multi-modality imaging detected a solid lesion of the spleen, who required splenectomy and was pathologically diagnosed as a splenic hamartoma. The postoperative course was uneventful. Discussion Splenic hamartoma is very rare. Only 20% of hamartomas occur in children. They are commonly found incidentally on imaging with no symptoms. Conclusion Splenic hamartoma is a benign vascular proliferative lesion that requires a multi-modality imaging studies for diagnosis and confirmed by histopathology. It must be included in the differential diagnosis of splenic mass forming lesions.
There are fewer than 160 cases of splenic hamartoma having been reported in the literature. Only 20% of the cases were detected in children. Although imaging findings were described preoperatively, the final diagnosis was splenic hamartoma based on histology. We report a case of solid lesion of the spleen, who required splenectomy and was pathologically diagnosed as splenic hamartomas.
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Affiliation(s)
| | - Ahmed Maher
- Pediatric surgery unit, Assiut university children hospital, Assiut, Egypt
| | | | - Hussein Ibrahim
- Pediatric surgery unit, Assiut university children hospital, Assiut, Egypt
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Franke D, Anupindi SA, Barnewolt CE, Green TG, Greer MLC, Harkanyi Z, Lorenz N, McCarville MB, Mentzel HJ, Ntoulia A, Squires JH. Contrast-enhanced ultrasound of the spleen, pancreas and gallbladder in children. Pediatr Radiol 2021; 51:2229-2252. [PMID: 34431006 DOI: 10.1007/s00247-021-05131-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/02/2021] [Revised: 04/30/2021] [Accepted: 06/10/2021] [Indexed: 12/14/2022]
Abstract
Gray-scale and color/power Doppler ultrasound (US) are the first-line imaging modalities to evaluate the spleen, gallbladder and pancreas in children. The increasing use of contrast-enhanced ultrasound (CEUS) as a reliable and safe method to evaluate liver lesions in the pediatric population promises potential for imaging other internal organs. Although CEUS applications of the spleen, gallbladder and pancreas have been well described in adults, they have not been fully explored in children. In this manuscript, we present an overview of the applications of CEUS for normal variants and diseases affecting the spleen, gallbladder and pancreas. We highlight a variety of cases as examples of how CEUS can serve in the diagnosis and follow-up for such diseases in children. Our discussion includes specific examination techniques; presentation of the main imaging findings in various benign and malignant lesions of the spleen, gallbladder and pancreas in children; and acknowledgment of the limitations of CEUS for these organs.
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Affiliation(s)
- Doris Franke
- Department of Pediatric Kidney, Liver and Metabolic Diseases, MHH, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
| | - Sudha A Anupindi
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.,Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
| | - Carol E Barnewolt
- Department of Radiology, Boston Children's Hospital, Harvard University, Boston, MA, USA
| | - Thomas G Green
- Department of Radiology, Crouse Hospital, Syracuse, NY, USA
| | - Mary-Louise C Greer
- Department of Diagnostic Imaging, The Hospital for Sick Children, Department of Medical Imaging, University of Toronto, Toronto, ON, Canada
| | - Zoltan Harkanyi
- Department of Radiology, Heim Pal National Pediatric Institute, Budapest, Hungary
| | - Norbert Lorenz
- Children's Hospital, Dresden Municipal Hospital, Teaching-Hospital of Technical University Dresden, Dresden, Germany
| | - M Beth McCarville
- Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN, USA
| | - Hans-Joachim Mentzel
- Section of Pediatric Radiology, Institute of Diagnostic and Interventional Radiology, University Hospital, Jena, Germany
| | - Aikaterini Ntoulia
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
| | - Judy H Squires
- Department of Radiology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
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Aguilera NS, Auerbach A. Hamartoma, choristomas and malformation of the spleen and lymph node. Semin Diagn Pathol 2018; 36:16-23. [PMID: 30482418 DOI: 10.1053/j.semdp.2018.11.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Affiliation(s)
- Nadine S Aguilera
- Department of Pathology, University of Virginia Health System, Charlottesville, VA, United States.
| | - Aaron Auerbach
- Joint Pathology Center, Silver Spring, MD, United States
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Serra F, Sorrentino L, Cabry F, Biondini D, Ceccarelli PL, Campanelli M, Gelmini R. First case of laparoscopic partial splenectomy in a child with hamartoma: Case report and review of the literature. Int J Surg Case Rep 2018; 53:140-143. [PMID: 30396124 PMCID: PMC6216043 DOI: 10.1016/j.ijscr.2018.09.018] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/18/2018] [Revised: 09/12/2018] [Accepted: 09/13/2018] [Indexed: 12/11/2022] Open
Abstract
INTRODUCTION To date, laparoscopic surgery has played a key role in the treatment of not only splenic hematologic pathologies but also solid ones. Hamartoma is a rare disease; only twenty percent of them are of pediatric relevance; it is a benign tumor, but radiological features never allow proper differentiation from malignant neoplasms. In children, hamartoma may be associated with other morbid conditions, such as sickle cell disease or other hematological alterations. PRESENTATION OF THE CASE We report a case of hamartoma in a 7-year-old child treated with partial laparoscopic splenectomy. After a multidisciplinary evaluation, the indication of laparoscopic splenectomy was decided; upon evaluating the age of the patient and the affected spleen portion, a partial splenectomy was proposed. The histological examination during surgery was performed to exclude any form of malignancy. The intraoperative frozen section of the specimen was negative for malignancies, and a partial splenectomy was performed. DISCUSSION Surgery remains the first choice in the definitive treatment of solid lesions of the spleen; minimally invasive technique, namely, laparoscopy, has set itself as the technique of choice for surgical treatment. In this case, the possibility of obtaining an intraoperative pathological diagnosis by frozen section of the specimen, confirming the benign nature of the lesion, allowed the surgeon to decide in favor of a laparoscopic partial splenectomy. CONCLUSION Partial laparoscopic splenectomy can be considered a safe, effective and reproducible alternative in patients suffering from benign solid diseases, safeguarding the hematological functions of the organ itself in pediatric age.
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Affiliation(s)
- Francesco Serra
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Lorena Sorrentino
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Francesca Cabry
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Diego Biondini
- Department of Pediatric Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Pier Luca Ceccarelli
- Department of Pediatric Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Michela Campanelli
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Roberta Gelmini
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
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Cheng N, Chen J, Pan Y, Jiang Y, Zhou J, Shao C. Splenic hamartoma with bizarre stromal cells: a case report and literature review. Diagn Pathol 2018; 13:8. [PMID: 29378604 PMCID: PMC6389155 DOI: 10.1186/s13000-018-0687-y] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2017] [Accepted: 01/15/2018] [Indexed: 12/11/2022] Open
Abstract
Background Splenic hamartoma is a rare benign vascular proliferative lesion composed of unorganized sinusoid-like channels lined with plump or flat endothelial cells and characterized by a CD8-positive immunophenotype of the lining cells. Scattered bizarre stromal cells can be found in some splenic hamartomas. The presence of splenic hamartoma with bizarre stromal cells is extremely rare and these bizarre cells make it possible to be regarded as a malignancy. Recognition of this rare histologic variant will help to avoid diagnostic confusion and overtreatment of this benign entity. Case presentation We report a case of a 40-year-old man with occasional left-sided waist back pain. A splenic space-occupying lesion was detected by ultrasound and magnetic resonance imaging. Microscopically bizarre large cells were scattered throughout the splenic hamartoma. The cells exhibited atypical nuclei, scarcely visible cytoplasm, and vesicular chromatin, and they did not form expansile clusters and lacked mitotic activity. An immunohistochemical panel was performed. The bizarre cells strongly expressed vimentin, and the Ki-67 index was very low. The lesion was diagnosed as a splenic hamartoma with bizarre stromal cells. Conclusions To the best of our knowledge, this is the first systematic review on a splenic hamartoma with bizarre stromal cells; only six cases have been described in the literature. Proper identification is important to secure adequate treatment.
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Affiliation(s)
- Na Cheng
- Department of Pathology, The Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Rd, Guangzhou, 510630, China
| | - Jianning Chen
- Department of Pathology, The Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Rd, Guangzhou, 510630, China
| | - Yuhang Pan
- Department of Pathology, The Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Rd, Guangzhou, 510630, China
| | - Ye Jiang
- Department of Pathology, The Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Rd, Guangzhou, 510630, China
| | - Jing Zhou
- Department of Pathology, The Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Rd, Guangzhou, 510630, China
| | - Chunkui Shao
- Department of Pathology, The Third Affiliated Hospital, Sun Yat-sen University, 600 Tianhe Rd, Guangzhou, 510630, China.
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Giambelluca D, Picone D, Re GL, Pappalardo S, Romeo P. Hamartoma of the spleen (splenoma) with calcifications in a child with beta-thalassemia: A case report. J Radiol Case Rep 2018; 11:7-12. [PMID: 29299089 DOI: 10.3941/jrcr.v11i5.3100] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
Splenic hamartoma (or splenoma) is a rare, benign, vascular tumor, often incidentally found at imaging, surgery or autopsy. Albeit usually asymptomatic and rare in children, when it occurs in the pediatric population it is more commonly symptomatic. We report a case of a 15-year-old girl with iron-deficiency anemia and beta-thalassemia, who had a large (10 × 8 × 7 cm) splenic lesion with calcifications, incidentally found during follow-up for splenomegaly and histologically characterized as hamartoma with calcified areas. The association of hamartoma and hematological disorders is a very unusual condition in children.
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Affiliation(s)
- Dario Giambelluca
- Department of Radiology, Policlinico Universitario Paolo Giaccone, Palermo, Italy
| | - Dario Picone
- Department of Radiology, Policlinico Universitario Paolo Giaccone, Palermo, Italy
| | - Giuseppe Lo Re
- Department of Radiology, Policlinico Universitario Paolo Giaccone, Palermo, Italy
| | | | - Placido Romeo
- Department of Radiology, S. Vincenzo Hospital, Taormina, Italy
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Illuminati G, Prezioso G, Pizzardi G, Pasqua R, Perotti B, Amatucci C, De Vincentiis L. Splenic hamartoma associated with abdominal discomfort and pain: Case report. INTERNATIONAL JOURNAL OF SURGERY OPEN 2018. [DOI: 10.1016/j.ijso.2018.07.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Elenga N, Labbé S, Leduc N, Sika A, Cuadro E, Long L, Njuieyon F, Kom-Tchameni R, Basset T. Asymptomatic multinodular splenoma (splenic hamartoma) in a child with sickle cell anemia. Int Med Case Rep J 2017; 10:233-236. [PMID: 28744165 PMCID: PMC5513840 DOI: 10.2147/imcrj.s125988] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Splenoma is a rare and benign malformation usually fortuitously diagnosed during imaging, surgery or, unfortunately, at autopsy. Although splenoma was first described in 1861, its association with hematological pathology is a very unusual condition in children. We report the case of an asymptomatic splenoma in an 8-year-old boy with sickle cell anemia, whose diagnosis was confirmed after conventional splenectomy.
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Affiliation(s)
- Narcisse Elenga
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Sylvain Labbé
- Pathological Anatomy Department, Cayenne Hospital, Cayenne Cedex, French Guiana
| | - Nicolas Leduc
- Pathological Anatomy Department, Cayenne Hospital, Cayenne Cedex, French Guiana
| | - Anicet Sika
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Emma Cuadro
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Laurence Long
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Falucar Njuieyon
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Rémi Kom-Tchameni
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Thierry Basset
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
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Ahyad RA, Lam CZ, Shearkhani O, Navarro OM. Incidental splenic nodules found on MR imaging done for assessment of iron overload in children. Pediatr Radiol 2017; 47:844-849. [PMID: 28409222 DOI: 10.1007/s00247-017-3842-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2016] [Revised: 02/06/2017] [Accepted: 03/21/2017] [Indexed: 10/19/2022]
Abstract
BACKGROUND MR imaging is used to assess iron overload in patients with hemoglobinopathies and in those who have undergone multiple blood transfusions. Sometimes splenic nodules are found incidentally on these examinations and this may cause diagnostic uncertainty. OBJECTIVE To determine the prevalence, imaging characteristics and evolution of splenic nodules found on MR imaging for iron overload evaluation. MATERIALS AND METHODS Retrospective review of all MR imaging examinations performed for iron overload assessment from 2005 to 2015 in a tertiary pediatric hospital. The presence of focal splenic nodules including number, size, signal characteristics and changes on follow-up MR imaging were recorded. Relevant patient clinical information including underlying hematological disease was also documented. RESULTS A total of 318 patients had MR imaging for iron overload assessment. Of these, 25 (8%) had at least one incidental splenic nodule. Sickle cell disease was present in 22 patients (88%) and thalassemia in 3 (12%). On intermediate-weighted spin-echo images, the nodules had high signal intensity compared to the remainder of the spleen in 23 patients (92%) and low signal intensity in the remaining 2 (8%). In all patients (100%) the nodules showed progressive loss of signal intensity with increasing echo time values. Follow-up MR imaging was performed in 20 (80%) patients, which showed an increase in the size of the splenic nodules in 7 patients (35%) stability in 11 (55%) and a decrease in size in 2 (10%). CONCLUSION It is not uncommon to find splenic nodules during MR evaluation of iron overload. In patients with sickle cell disease, most of these nodules are thought to represent preserved splenic tissue and appear hyperintense compared to the remainder of the spleen. They frequently remain stable on follow-up imaging, although about a third of them may show growth. Awareness of these nodules is important to avoid concern for potential malignancy and unnecessary investigations.
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Affiliation(s)
- Rayan A Ahyad
- Department of Medical Imaging, University of Toronto, Toronto, ON, Canada.,Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada
| | - Christopher Z Lam
- Department of Medical Imaging, University of Toronto, Toronto, ON, Canada.,Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada
| | - Omid Shearkhani
- Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada
| | - Oscar M Navarro
- Department of Medical Imaging, University of Toronto, Toronto, ON, Canada. .,Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada.
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Barrier A, Willy S, Slone JS. Extramedullary hematopoiesis of the liver in a child with sickle cell disease: A rare complication. Pediatr Int 2015; 57:770-2. [PMID: 26171586 DOI: 10.1111/ped.12647] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/21/2014] [Revised: 11/17/2014] [Accepted: 12/16/2014] [Indexed: 12/01/2022]
Abstract
We present the case of a 7-year-old Cameroonian girl with sickle cell disease (SCD) who presented with progressive abdominal distension, fever, severe anemia, respiratory distress, and fatigue. Abdominal ultrasound showed a 15.3 cm × 11.5 cm × 15.5 cm solid echogenic mass within the left lobe of the liver. Fine-needle aspiration showed features of extramedullary hematopoiesis (EMH). Despite transfusions, antibiotics, and initiation of hydroxyurea the patient died of respiratory failure during the hospital stay. There is a paucity of information on EMH in the pediatric sickle cell population, especially from resource-limited settings such as western Africa. EMH, however, is a known complication of SCD and should be considered in patients presenting with mass lesions in the setting of chronic anemia. With limited therapeutic interventions for EMH, including radiation and hydroxyurea, the emphasis should be on improving overall treatment of patients with chronic and untreated hemolytic anemia, especially in low-income countries.
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Affiliation(s)
| | - Simo Willy
- Mbingo Baptist Hospital, Mbingo, Cameroon
| | - Jeremy S Slone
- Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, Texas, USA
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Wang RT, Xu XS, Hou HL, Qu K, Bai JG. Symptomatic multinodular splenic hamartoma preoperatively suspected as metastatic tumor: A case report. World J Gastroenterol 2014; 20:10637-10641. [PMID: 25132786 PMCID: PMC4130877 DOI: 10.3748/wjg.v20.i30.10637] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/06/2014] [Revised: 01/26/2014] [Accepted: 05/05/2014] [Indexed: 02/06/2023] Open
Abstract
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.
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Zhang LF, Tou JF, Wang X, Gu WZ, Ma XH, Qin Q. Splenic hamartomas in two children. World J Surg Oncol 2014; 12:180. [PMID: 24906658 PMCID: PMC4068870 DOI: 10.1186/1477-7819-12-180] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2013] [Accepted: 05/19/2014] [Indexed: 02/03/2023] Open
Abstract
Hamartomas are extremely rare splenic benign tumours in children. We present two cases, both in boys (6 and 8 years old), with left upper quadrant abdominal pain that were otherwise asymptomatic. Both patients showed a splenic mass on preoperative ultrasonography and magnetic resonance imaging (MRI). One patient had a focal splenic mass that was identified preoperatively with contrasted computed tomography (CT) scans. Both patients underwent a total splenectomy. Although multi-modality imaging findings were described preoperatively, the final diagnosis in each case was splenic hamartoma based on histology and immunohistochemistry. The postoperative courses were uneventful.
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Affiliation(s)
| | | | | | | | | | - Qi Qin
- Department of General Surgery, Children Hospital, Zhejiang University School of Medicine, Hangzhou 310006, China.
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Pisani Ceretti A, Bislenghi G, Virdis M, Maroni N, Gatti A, Opocher E. Laparoscopic splenectomy for splenic hamartoma: a case report. Case Rep Gastrointest Med 2012; 2012:435802. [PMID: 23125940 PMCID: PMC3485498 DOI: 10.1155/2012/435802] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/16/2012] [Accepted: 09/30/2012] [Indexed: 12/11/2022] Open
Abstract
Hamartoma is a rare splenic benign tumor usually accidentally detected as a radiologic finding. Preoperative diagnosis poses a challenge and thus surgery becomes necessary to confirm the clinical suspicion. Laparoscopic splenectomy has gained consensus as a standard surgical procedure particularly for autoimmune hematological diseases. This former experience has allowed this technique to be extended to other splenic pathologies. Here we report a case of total laparoscopic splenectomy for a bulky splenic hamartoma in a young male patient.
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Affiliation(s)
| | - Gabriele Bislenghi
- Department of General Surgery, San Paolo Hospital, University of Milan, Via Cesariano 10, 20154 Milano, Italy
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Shah P, Robbani I, Khan AB, Shah OJ. Symptomatic splenoma in a child. Ann Saudi Med 2009; 29:236-8. [PMID: 19448371 PMCID: PMC2813654 DOI: 10.4103/0256-4947.51780] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
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von Herbay A, Barreiros AP, Ignee A, Westendorff J, Gregor M, Galle PR, Dietrich C. Contrast-enhanced ultrasonography with SonoVue: differentiation between benign and malignant lesions of the spleen. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2009; 28:421-434. [PMID: 19321670 DOI: 10.7863/jum.2009.28.4.421] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/27/2023]
Abstract
OBJECTIVE We investigated the ability of contrast-enhanced ultrasonography with SonoVue (Bracco SpA, Milan, Italy), a sulfur hexafluoride microbubble contrast agent, to reveal differences between benign and malignant focal splenic lesions. METHODS In a prospective study we investigated 35 lesions in 35 patients (24 male and 11 female; mean age +/- SD, 54 +/- 15 years) with focal splenic lesions detected by B-mode ultrasonography. After intravenous injection of 1.2 to 2.4 mL of SonoVue, the spleen was examined continuously for 3 minutes using low-mechanical index ultrasonography with contrast-specific software. The final diagnosis was established by histologic examination, computed tomography, or magnetic resonance imaging. RESULTS In 14 patients, the splenic lesions were malignant (metastasis, n = 6; non-Hodgkin lymphoma, n = 6; and Hodgkin lymphoma, n = 2). In 21 patients, the focal splenic lesions were benign (ischemic lesion, n = 6; echogenic cyst, n = 5; abscess, n = 4; hemangioma, n = 3; hematoma, n = 1; hemophagocytosis syndrome, n = 1; and splenoma, n = 1. Typical findings for benign lesions were 2 arrival patterns: no contrast enhancement (neither in the early nor in the parenchymal phase; P < .05) and the beginning of contrast enhancement in the early phase followed by contrast enhancement in the parenchymal phase 60 seconds after injection. In contrast, the combination of contrast enhancement in the early phase followed by rapid wash-out and demarcation of the lesion without contrast enhancement in the parenchymal phase (60 seconds after injection) was typical for malignant lesions (P < .001). CONCLUSIONS Contrast-enhanced ultrasonography is helpful in the differentiation between benign and malignant lesions of the spleen.
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Abstract
Splenic hamartoma is a rare, benign vascular proliferation that is often found incidentally while working up other complaints or at autopsy. Women more commonly present with symptoms related to mass effect than men. Histologic findings consist of unorganized vascular channels of varying width, with intervening red pulp-like disorganized stroma with or without lymphoid follicles. The endothelial cells are similar to those of normal splenic sinuses. Although rendering a diagnosis can be difficult, endothelial cells that are positive for CD8 are a key feature that differentiate hamartoma from other vascular lesions of the spleen. Clinical, radiologic, and histologic correlation is essential to ensure this benign lesion is not mistaken for malignancy.
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Affiliation(s)
- Hwajeong Lee
- Department of Pathology and Laboratory Medicine, Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI 48202, USA.
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Abstract
We presented a case of splenic hamartoma. A 12-year-old girl has had an abdominal pain since 9 years of age. Contrast-enhanced computed tomography scan at a local hospital revealed heterogeneous enhancement of a mass which is 5 cm in diameter, located near the surface of the spleen, diagnosed as hemangioma. She had an investigation with Levovist-enhanced ultrasonography and superparamagnetic iron oxide-enhanced magnetic resonance imaging on T2-weighted gradient-echo imaging, which showed a hypervascular echoic mass and a decrease of signal intensity. The imaging diagnosis was splenic hamartoma. She underwent laparoscopic splenectomy to prevent the spontaneous rupture. The splenic artery at the pancreas body was exposed, and doubly ligated by vessel clip and furthermore ligated by 3-0 silk. At the splenic hilum, the splenic artery and vein were dissected with the Endo GIA vascular linear cutting stapler. The spleen was captured into the Endocatch II retrieval bag and removed from a transverse lower abdominal (suprapubic) incision extended up 7 cm in length through a port site. In pathological findings, the tumor was compatible with hamartoma. Splenic hamartomas in pediatric patients have been described extremely rare. In clinical symptoms, spontaneously ruptured splenic hamartoma has been rarely reported.
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Affiliation(s)
- Yukihiro Tatekawa
- Department of Surgery, Nara Medical University, 840, Shijyo-cho, Kashiharashi, Nara 634-8522, Japan.
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Benkirane A, Berrebi D, Olaya N, Ferkdadji L, Chomette PP, Benkerrou M, Peuchmaur M. Hamartome de la rate (splénome) chez un enfant drépanocytaire. Ann Pathol 2007; 27:27-30. [PMID: 17568356 DOI: 10.1016/s0242-6498(07)88681-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
Hamartomas of the spleen or splenomas are uncommon benign lesions that predominantly occur in adults. We report a case of an 11-year-old girl with sickle cell anemia who had a single splenic 1.8 cm nodule incidentally found during splenectomy and histologically characterized by disorganized red pulp tissue without interspersed white pulp leading to the diagnosis of hamartoma. The association of hamartoma and hematological conditions is a very unusual condition in children.
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Affiliation(s)
- Amal Benkirane
- Anatomie et Cytologie pathologiques, Hôpital Robert Debré, 48, boulevard Sérurier 75019 Paris
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Abstract
Splenic hamartoma is a rare entity characterized by disorganized red pulp tissue in the absence of interspersed white pulp. We report 3 cases with scattered bizarre stromal cells, a feature not previously reported in the literature. The patients were adults who presented with abdominal pain (2 cases) or were incidentally found to have a splenic mass (1 case). The lesion was solitary, circumscribed, and unencapsulated, comprising disorganized slit-like, tubular, ectatic or cavernous vascular channels. There was a loose stroma that contained lymphocytes, plasma cells, siderophages, and fibrin exudate. Scattered haphazardly were isolated bizarre large cells with oval, convoluted, or multilobated nuclei; pale or smudged chromatin; and scanty cytoplasm. Mitotic figures were not found. The bizarre cells were negative for lymphoid, dendritic cell, histiocytic, myeloid, endothelial, epithelial, and melanocytic markers. Only rare bizarre cells stained for desmin in 1 case. It is important to recognize this morphologic variant of splenic hamartoma in order not to misinterpret the bizarre cells as being indicative of a malignancy.
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Affiliation(s)
- Wah Cheuk
- Department of Pathology, Queen Elizabeth Hospital, Hong Kong.
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