Congenital intrathoracic accessory spleen is a very rare trick of nature: a case report

Abstract

Background: Congenital intrathoracic accessory spleen (CIAS) refers to a developmental anomaly resulting in the presence of splenic tissue within the chest. The differential diagnoses for the resulting mass are pulmonary malformations, or lesions with malignant potential. To our knowledge, only four cases of presumed CIAS have been described in literature to date, and no cases were reported in the United States. Case presentation: We report on a 14-year-old Caucasian female with a left chest mass discovered incidentally on a CT scan performed following an all-terrain vehicle accident. Following resection, the mass was diagnosed as a CIAS. Conclusions: From our review of literature, we found that CIAS can pose a diagnostic dilemma as it is rare, difficult to distinguish from pulmonary sequestration, or malignancy, and biopsy is often inconclusive. Resection is required to rule out malignancy and determine the diagnosis. Pediatric thoracic surgeons should consider CIAS in their differential for an intrathoracic mass with an inconclusive biopsy.

Preoperative angioembolisation of a mediastinal accessory ectopic spleen: A case report and review of the literature

“…This case illustrates the difficulty in diagnosis of such a rare and previously unreported entity. Multiple imaging tests and modalities were insufficient and ultimately, in line with previously reported cases, the patient required surgery for a definitive diagnosis [ [6] , [7] , [8] , [9] , 11 ]. The use of denatured red blood cell or Sulphur colloid radionuclide studies was not entertained in our patient, but could have proved diagnostic as is the case in splenosis.…”

“…Congenital ectopic thoracic spleen represents an exceedingly rare clinical entity with only 5 cases reported in the literature [ [6] , [7] , [8] , [9] , 11 ]. Four of these case reports described tissue within the left hemithorax [ 6 , 7 , 9 , 11 ] and 1 within the right hemithorax [8] . To our knowledge, this case represents the first reported incidence of congenital accessory splenic tissue within the middle mediastinum.…”

“…It is most commonly located within the perisplenic region and rarely measures larger than 40mm in maximal dimensions [ 4 , 5 ]. Less frequently, other locations have been reported including within the retroperitoneum, intrapancreatic, left intragonadal, and very rarely intrathoracic [6] , [7] , [8] , [9] , [10] , [11] . In the case of accessory intrathoracic splenic tissue, it has been postulated that aberrant migration into the pleural cavity may occur via incomplete pleuroperitoneal folds [8] .…”

Thoracic Splenosis - A necessary differential diagnosis for pleural based nodules with history of thoracoabdominal trauma

“…The condition is usually a result of joint traumatic injury to the abdomen and diaphragm, 1 and very rarely is thought to have a congenital origin as well. 2 The condition usually remains asymptomatic and is only discovered as an incidental finding, however its diagnosis is necessary owing to the range of differentials associated with it, including malignant conditions.…”