Abdominal splenosis: An often underdiagnosed entity

Abdominal splenosis: An often underdiagnosed entity

Rev Esp Med Nucl. 2011;30(2):97-100 Clinical note Abdominal splenosis: An often underdiagnosed entity J.L. Vercher-Conejero, a,* P. Bello-Arqués, a ...

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Rev Esp Med Nucl. 2011;30(2):97-100

Clinical note

Abdominal splenosis: An often underdiagnosed entity J.L. Vercher-Conejero, a,* P. Bello-Arqués, a L. Pelegrí-Martínez, b I. Hervás-Benito, a J.L. Loaiza-Góngora, a M. Falgas-Lacueva, a C. Ruiz-Llorca, a R. Pérez-Velasco, a A. Mateo-Navarro a Servicio de Medicina Nuclear, Hospital Universitario La Fe, Valencia, Spain Servicio de Radiodiagnóstico, Hospital Universitario La Fe, Valencia, Spain

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abstract

History of the article: Received 17 February 2010 Accepted 10 April 2010

Splenosis is defined as the heterotopic autotransplantation of splenic tissue because of a ruptured spleen due to trauma or surgery. It is a benign and incidental finding, although imaging tests may sometimes orient toward malignancy simulating renal tumors, abdominal lymphomas, endometriosis, among other. We report the case of a 42-year old male in whom a MRI was performed after a study due to abdominal pain. Multiple enlarged lymph nodes were observed in the abdomen, suggestive of lymphoproliferative disease. As an important background, splenectomy was carried out due to abdominal trauma at age 9. After several studies, it was decided to perform a 99mTc-labeled heat-damaged red blood cell scintigraphy that showed multiple pathological deposits distributed throughout the abdomen, and even the pelvis, being consistent with splenosis. © 2010 Elsevier España, S.L. and SEMNIM. All rights reserved.

Keywords: 99m-Technetium Heat-damaged redbloodcell Splenosis Radionuclide scintigraphy Trauma Abdominal nodules Computed tomography Magnetic resonance

Esplenosis abdominal: una entidad frecuentemente infradiagnosticada resumen

Palabras clave: Tecnecio-99m Hematíes desnaturalizados Esplenosis Gammagrafía Trauma Nódulos abdominales Tomografía computarizada Resonancia magnética

La esplenosis se define como el autotrasplante heterotópico de tejido esplénico como resultado de una rotura del bazo por trauma o cirugía. Es una condición benigna y de hallazgo casual, aunque en ciertas ocasiones las pruebas de imagen puedan orientar a malignidad simulando tumores renales, linfomas abdominales y endometriosis, entre otros. Presentamos el caso de un varón de 42 años al que, tras un estudio por dolor abdominal, se le realiza una resonancia magnética en la que se observan múltiples adenopatías en el abdomen que pueden orientar a un síndrome linfoproliferativo. Como antecedente importante, presenta esplenectomía por trauma abdominal a los 9 años. Tras varios estudios, se decide realizar una gammagrafía con hematíes desnaturalizados marcados con tecnecio-99m que muestra múltiples depósitos patológicos distribuidos por todo el abdomen e, incluso, la pelvis, siendo este hallazgo compatible con esplenosis. © 2010 Elsevier España, S.L. y SEMNIM. Todos los derechos reservados.

Introduction Splenosis is an acquired condition defined as the autotransplantation of viable splenic tissue in different anatomical compartments of the body. This situation is usually produced following rupture of the spleen due to trauma or in patients in whom therapeutic splenectomy has been performed.1 Splenic implants are usually multiple and may be of intraperitoneal or extraperitoneal localization. They are most often found on serous surfaces of the small intestine, the omentum, the large intestine, the mesenteruim and the retroperitoneum among other locations. Those found outside the peritoneum have been reported in the lung, the liver, the pericardium, and one exceptional case has even been described in the brain.2

* Corresponding author. E-mail: [email protected] (J.L. Vercher-Conejero).

The identification of these implants is generally asymptomatic and usually occurs after laparotomy, laparoscopy or imaging tests such as computerized tomography (CT) for other reasons. This finding may sometimes lead to confusion and suggests malignancy if the history of the patient is not taken into account, thereby leading to the performance of often unnecessary invasive techniques. The present case depicts such a setting. Clinical case We present the case of a 42-year-old male who consulted in the digestive disease outpatient clinic for suspicion of cholestasis with an increase in transaminases and alkaline phosphates (AP). Magnetic resonance (MR) of the abdomen was performed showing multiple nodules of up to 15 mm in diameter localized in the anterior mesenterium which were considered as mesenteric adenopathies (fig. 1). Another nodular formation of 25 mm in diameter was also found in the retroperitoneum adjacent to the posterior diaphragmatic

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J.L. Vercher-Conejero et al / Rev Esp Med Nucl. 2011;30(2):97-100

Figure 1. Magnetic resonance (coronal plane of SSFSE sequence potentiated in T2). The presence of multiple nodules (arrows) of approximately 15 mm in diameter were observed which were isotense to the abdominal musculature in the anterior mesenterium.

insertion. In addition, a solid elongated image of 45 × 21 mm superoexternal to the left kidney was described with signal intensity similar to the nodular images observed. Complete CT study was recommended to rule out a lymphoproliferative syndrome. The patient was admitted to the Department of Internal Medicine of our hospital and the case was investigated. The only personal history of the patient was that the patient reported having undergone splenectomy following intense abdominal trauma at the age of 9 years. On physical examination small soft nodules of approximately 15 mm were palpated distributed along the surgical scar and the remainder of the examination was normal. Blood analysis was normal and tumor markers were negative with a persistent increase in gammaglutamyl transpeptidase and AP. Chest and abdominal radiographies did not show any finding of pathological significance. Thoracoabdominal CT with contrast demonstrated multiple lobulated nodular lesions with homogeneous uptake of contrast in the abdomen. (figs. 2 and 3) as well as a residual small sized spleen (fig. 4). Taking the report of the CT and the history of abdominal traumatism during childhood into account, splenosis was considered. To confirm this diagnostic suspicion scintigraphy with denaturalized erythrocytes (DE) marked with technetium (Tc) 99m was performed demonstrating multiple foci of hyperuptake in the splenic region, the peritoneal cavity (fig. 5) and even foci in the pelvic region (fig. 6) compatible with the clinical suspicion of splenosis. The patient was discharged with a definitive diagnosis of splenosis and slightly elevated gammaglutamyl transpeptidase and AP values with the most probable cause being non alcoholic steatohepatitis while awaiting confirmation by liver biopsy. The asymptomatic situation of the patient allowed an expectant attitude without treatment to be maintained. Discussion In 1883 Graffini and Tizziani observed the presence of tissue with the color and consistence identical to splenic tissue in dogs. Later,

Figure 2. Computerized tomography (venous phase and axial plane). Several infradiagmatic, supramesocholic nodules located in the upper abdomen with homogeneous uptake of contrast (arrows).

Figure 3. Computerized tomography (venous phase and axial plane). Well delimited nodular lesion of oval morphology located in the mesenteric fat, the mesogastrium and posterior to, albeit not in contact with, the abdominal rectal muscle and is enhanced following the administration of i.v. contrast (arrow).

Von Kuttner was the first to identify the sowing of splenic tissue in the peritoneal cavity which explained the mechanism of autotransplantation of splenic tissue, although it was not until 1939 that the term splenosis was first introduced when Buchbinder and Lipkoff3 described the case of a woman who, on exploratory laparotomy for a history of endometriosis, presented multiple peritoneal implants. Histological study demonstrated that these nodules were formed of splenic tissue.



J.L. Vercher-Conejero et al / Rev Esp Med Nucl. 2011;30(2):97-100

Figure 4. Computerized tomography (venous phase and axial plane). In the topography of the spleen with a superoexternal localization to the left kidney a solid image of 45 × 21 mm in diameter may be seen which seems to be related to the splenic remains (arrow). A nodular image can also be observed adjacent to the posterior diaphragmatic insertion of the right side (arrow head).

Figure 5. Scintigraphy with technetium 99m and denaturalized erythrocytes. The anterior projection of the abdomen in the late phase shows multiple deposits of activity in the splenic area, the middle abdominal region and another focus inferior to the liver.

Up to 67% of the patients presenting splenic rupture may develop splenosis.1 The interval of time between the initial trauma and the diagnosis varies from 3-45 years with an average interval of 21 years.4 The real incidence is not well known since the finding of this entity is usually accidental. Ectopic splenic tissue may be found in two different ways: a) splenunculi or accessory spleen (AS) or b) splenosis. The AS are congenital and are found in the left side of the dorsomesogastric during

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Figure 6. Scintigraphy with technetium 99m and denaturalized erythrocytes. The posterior projection of the abdomen in the late phase shows several foci located in the upper poster abdominal region and the pelvis.

the period of fetal development located near the gastrosplenic or splenopancreatic ligament. To the contrary, splenosis is an acquired condition defined as the autoimplantation of splenic tissue in a heterotopic localization which may include any anatomical structure.2 The characteristics of AS and splenosis are somewhat different. The splenenculi are normally few in number, six or less. On the other hand, with splenosis a greater number may be found, with up to 300 nodules having been described.3 The AS have a normal splenic histology with blood obtained from a branch of the splenic artery. On the other hand, the blood in splenosis is provided by the surrounding tissues and vessels depending on the localization7. (falten refs. 5 y 6). In addition, histologically the nodules in splenosis demonstrate a distorted architecture without helium and an altered capsule.2 The mechanism by which splenosis is produced begins with splenic rupture whether by trauma or after surgery. It is thought that the dispersion of damaged splenic pulp continues with a process of implantation in the adjacent cavities. A second mechanism is the hematogenic propagation suggested in cases of intracranial and intrahepatic splenosis.4 This later case could also be produced by portal dissemination with growth of the nodules in response to tissue hypoxia. Thoracic splenosis may be due to traumatic rupture of the diaphragm and the subsequent passage of the splenic pulp to the thoracic cavity.4,5 Splenic function may be partially or totally substituted based on the quantity and vascularization of the implanted splenic tissue. Total splenectomy would produce immunological alterations and modifications in the peripheral blood which, due to transitory thrombocytosis, would allow Howell-Jolly bodies, Heinz bodies and siderocytes to be observed.2 Thus, the absence of visualization of these cellular alterations may also be indicative of splenosis. Most people with splenosis are usually asymptomatic during years without the need for surgical treatment. On occasions, manifestations of splenosis may include abdominal pain, intestinal obstruction due to adherences and digestive hemorrhage among others.2 The presence of this symptomatology may lead to confusion and imitate processes such as endometriosis, angiomas, lymphomas, urological tumors,6 Kaposi sarcomas in cutaneous splenosis or hepatocarcinomas in the case of intrahepatic splenosis and even metastatic lesions.

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It is therefore very important to have reliable non invasive diagnostic techniques which are sensitive and specific but also with a low index of false positives.8 The low density of splenic tissue makes visualization in conventional radiology difficult. Computerized tomography demonstrates the number, the shape and the size but not the possible origin as previously commented in the clinical case, and standard MR may not be very useful in the differential diagnosis of the finding. Nonetheless, recent studies have remarked the use of MR with ferumoxide, a superparamagentic contrast9 sequenced in T2 and particularly in cases of intrahepatic splenosis which aids in differentiating between hepatic tumors and splenic tissue. To date, the diagnostic technique of choice continues to be scintigraphy whether with Tc-99m sulphur colloid (SC) or with Tc99m-HD or with platelets marked with indium 111. These are based on the phagocytic capacity of the cells of the reticulendothelial system. On comparison between scintigraphy with SC and that with HD, Gunes et al. observed that scintigraphy with HD had a diagnostic performance of up to 32 % greater than that achieved with SC.10 Another reason for the greater diagnostic precision of scintigraphy with HD may be because the spleen only receives 10 % of the SC injected while this value rises to up to 90 % with HD. Scintigraphy with HD has also shown to be more sensitive than SC in early splenosis in the presence of little splenic tissue of functional hyposplenism.10 The diagnostic precision may even by greater if, in addition, tomographic images by single photon emission are performed.10 Another advantage of the tests with radioisotopes is the possibility of carrying out a complete body study in which the presence of foci of uptake suggestive of ectopic splenic tissue may be observed. As reported in the literature, scintigraphy with Tc-99m-HD is a reliable, easily applicable, non invasive technique with a high positive predictive value and is both highly specific and profitable.10,11

In the present case, the use of scintigraphy with HD allowed the diagnosis of splenosis to be achieved as well as the ruling out other diseases,12 in addition to avoiding the unnecessary use of invasive diagnostic techniques.

References 1. Livingston CD, Levine BA, Lecklitner ML, Sirinek KR. Incidence and function of residual splenic tissue following splenectomy for trauma in adults. Arch Surg. 1983;118:617-20. 2. Fleming CR, Dickson ER, Harrison Jr EG. Splenosis: Autotransplantation of sple- nic tissue. Am J Med. 1976;61:414-9. 3. Buchbinder JH, Lipkoff CJ. Splenosis: Multiple peritoneal splenic implants following abdominal injury. Surgery. 1939;6:927-34. 4. Yammine JN, Yatim A, Barbari A. Radionuclide imaging in thoracic splenosis and a review of the literature. Clin Nucl Med. 2003;28:121-3. 5. Puyalto P, Sánchez JJ, Olazábal Á. Esplenosis intratorácica: a propósito de un caso. Radiología. 2007;49:436-9. 6. Pérez Fentes D, Pazos González G, Blanco Parra M, Pubul Núñez V, Toucedo Caamaño V, Puñal Pereira A, et al. Esplenosis simulando una masa renal izquierda. Arch Esp Urol. 2009;62:396-9. 7. Carr NJ, Turk EP. The histological features of splenosis. Histopathology. 1992;21:549-53. 8. Stewart CA, Skimura IT, Siegel ME. Scintigraphic demonstration of splenosis. Clin Nucl Med. 1986;11:161-4. 9. Berman AJ, Zahalsky MP, Okon SA, Wagner JR. Distinguishing splenosis from renal masses using ferumoxide-enhanced magnetic resonance imaging. Uro- logy. 2003;62:748. 10. Castellani M, Cappellini MD, Cappelletti M, Fedriga E, Reschini E, Cerino M, et al. Tc-99m sulphur colloid scintigraphy in the assessment, of residual splenic tissue after splenectomy. Clin Radiol. 2001;56:596-8. 11. Wedemeyer J, Gratz KF, Soudah B, Rosenthal H, Strassburg C, Terkamp C, et al. Splenosis: An important differential diagnosis in splenectomized patients presenting with abdominal masses of unknown origin. Z Gastroen- terol. 2005;43:1225-9. 12. Rubio Garay M, Belda Sanchís J, Iglesias Sentís M, Gimferrer Garolera JM, Catalán Biel M, Callejas Pérez MA. Diagnóstico no invasivo de la esplenosis torácica postraumática. Arch Bronconeumol. 2004;40:139-40.