{"paper_id":"a27584b3-8e80-4127-9a4b-302f933ad26d","body_text":"Review began\n 01/25/2022 \nReview ended\n 02/07/2022 \nPublished\n 02/08/2022\n© Copyright \n2022\nKrzeczowski et al. This is an open access\narticle distributed under the terms of the\nCreative Commons Attribution License CC-\nBY 4.0., which permits unrestricted use,\ndistribution, and reproduction in any\nmedium, provided the original author and\nsource are credited.\nSplenic Cysts and the Case of Mistaken Identity\nRachel M. Krzeczowski \n \n, \nTheresa N. Jackson \n \n, \nWareef Kabbani \n \n, \nHeather M. Grossman Verner \n \n, \nPhillip\nSladek \n1.\n Clinical Research, Methodist Health System, Dallas, USA \n2.\n Medical Education, Methodist Health System, Dallas,\nUSA \n3.\n Surgical Pathology, Methodist Health System, Dallas, USA \n4.\n Surgical Acute Care, Methodist Health System,\nDallas, USA\nCorresponding author: \nPhillip Sladek, \nphillipsladek@mhd.com\nAbstract\nEndometriosis is a well-described pathology, with anatomic location of endometrial cell implantation\nextending both intraperitoneal and rarely extraperitoneal. Interestingly, previous reports indicated that the\nspleen enjoys immunity to endometriosis. Here, we present a patient with unremitting abdominal pain who,\nupon further workup, revealed multicystic disease of the spleen. The patient underwent an open\nsplenectomy with pathology revealing intraparenchymal endometriosis likely due to seeding from traumatic\nsplenorrhaphy. Two-week follow-up demonstrated resolution of symptoms and a well-healing incision with\nno postoperative complications.\nCategories:\n Obstetrics/Gynecology, Pathology, General Surgery\nKeywords:\n splenic cysts, general gynecology, endometriosis, splenectomy, trauma\nIntroduction\nSplenic cysts are a rare entity with only 7.6 cases reported for every 10,000 patients \n[1]\n. Fowler et al. provide\nthe current classification system of splenic cysts consisting of either primary (true) cysts with a cellular\nlining or secondary (false) cysts without a cellular lining \n[2]\n. Primary cysts are further subclassified as\nparasitic versus nonparasitic \n[2]\n. Splenic cysts can present as either unilocular or multilocular with or\nwithout intramural calcifications \n[3]\n. Nonparasitic cysts are typically unilocular, whereas parasitic cysts are\nmore often multilocular \n[4]\n. A detailed history and physical examination in combination with computed\ntomography (CT) can help narrow the etiologic differential diagnoses for patients presenting with splenic\ncysts.\nOn the other hand, endometriosis is a fairly common gynecologic pathology with a varied presentation that\nmay present in nearly all intraperitoneal organs \n[5-7]\n. Interestingly, past literature indicates that the spleen\nenjoys a unique immunity against this pathology \n[8]\n. Here, we present the first case of a female with\nendometriosis of the spleen who presented with unrelenting abdominal pain.\nCase Presentation\nA 36-year-old female presented with a one-week history of worsening chronic left upper quadrant\nabdominal pain with postprandial nausea and vomiting. Her medical history was remarkable for\nsplenorrhaphy following blunt traumatic assault 10 years previously. Additionally, the patient had traveled\nto the Caribbean six months prior to presentation. CT revealed an 8-cm multiloculated cystic mass with\nmural calcifications (Figure \n1\n). Given her past medical history, the differential diagnosis included both\nparasitic splenic cyst and pseudocyst. After negative serology for \nEchinococcus granulosus\n IgG antibodies, the\npatient underwent an open splenectomy without complications. Gross examination of the resected specimen\nshowed a benign 8-cm multilocular cyst. Microscopic examination revealed multiple cystic structures with\nglands and surrounding stroma. The glands were lined by mucin secreting columnar epithelium and showed\nluminal secretions. Stroma was compact and cellular. The tissue underwent H&E staining (Figure \n2\n) and\nimmunohistochemistry (Figure \n3\n), and the findings were consistent with endometriosis. The patient did well\npostoperatively. At her two-week follow-up, she had resolution of symptoms and a well-healing incision.\nWhile continual follow-up was recommended due to the chronic and recurrent nature of endometriosis, our\npatient was unfortunately lost to follow-up. Follow-up with an annual ultrasound scan of the abdomen for\nthe first few postoperative years would have allowed for recurrence surveillance.\n1\n2\n3\n1\n4\n \n Open Access Case\nReport\n \nDOI:\n 10.7759/cureus.22012\nHow to cite this article\nKrzeczowski R M, Jackson T N, Kabbani W, et al. (February 08, 2022) Splenic Cysts and the Case of Mistaken Identity. Cureus 14(2): e22012. \nDOI\n10.7759/cureus.22012\n\nFIGURE\n 1: Computer tomography demonstrating a multicystic splenic\ncyst\nThe spleen contains an 8-cm multiloculated cystic mass (star) with some mural calcifications (arrow) in the cysts.\n2022 Krzeczowski et al. Cureus 14(2): e22012. DOI 10.7759/cureus.22012\n2\n of \n5\n\nFIGURE\n 2: H&E stain of the splenic tissue revealed histology consistent\nwith endometrial cells\nA: Low-power image showing splenic parenchyma with areas of fibrosis, evidence of past hemorrhage\n(cholesterol clefts (arrow)) and adjacent cystically dilated glandular structures (star) (H&E, ×20). B: Low-power\nimage showing cystically dilated glandular structures within the spleen. Notice the condensed stroma with\nhemorrhage surrounding the glands (H&E, ×40). C: Endometrial-type glands with focal ciliated epithelium (arrow)\n(H&E, ×400). D: Note the endometrial stroma comprised of spindle cells with scant cytoplasm and ill-defined cell\nborders (star) surrounding endometrial-type glands (arrow) (H&E, ×400).\n2022 Krzeczowski et al. Cureus 14(2): e22012. DOI 10.7759/cureus.22012\n3\n of \n5\n\nFIGURE\n 3: Immunohistochemistry demonstrates normal endometrial\ntissue with positive staining for Pax-8, WT-1, estrogen receptor, and\nCD10\nImmunohistochemistry defined the endometrial cells with positive Pax-8 (A), WT-1 (B), estrogen receptor staining\nfor epithelial cells of the endometrium (C), and CD10 staining for normal endometrial stroma (D). This is consistent\nwith endometrial tissue.\nDiscussion\nEndometriosis is a common gynecological pathology impacting 6%-10% of reproductive-aged women. Its\npresentation most commonly includes pelvic pain, dysmenorrhea, dyspareunia, and infertility.\nEndometriosis is a chronic disease and requires a life-long management plan that can range from medical to\nsurgical therapy. Medical therapy includes nonsteroidal anti-inflammatory drugs (NSAIDs) for\nendometriosis-related pain, continuous hormonal contraceptives, or gonadotropin-releasing hormone\n(GnRH) agonists, whereas surgical options include excision or ablation \n[5]\n.\nThe pathogenesis of endometriosis has long been debated. The most widely accepted pathogenetic\nmechanism is the transplantation theory of Sampson, which proposes multiple avenues for seeding of\nendometriosis, including lymphatic dissemination, vascular dissemination, iatrogenic transplantation, and\nretrograde menstruation \n[8]\n. The most common modality for ectopic endometriosis is overwhelmingly\nretrograde menstruation, resulting in the implantation of endometrial cells within the pelvic and abdominal\ncavity \n[6]\n. Implantation sites are well documented. Anatomic distribution includes ovaries, fallopian tubes,\nuterus, sigmoid colon, small bowel, omentum, and other sites \n[6,7]\n. Rarely, extraperitoneal endometriosis of\nthe lung, urinary system, peripheral nervous system, and central nervous system have been described \n[9,10]\n.\nIt is likely that this patient’s history of splenorrhaphy provided an implantation site for her endometriosis. \nSplenorrhaphy has been reported in surgical literature since the 1800s \n[11]\n. However, splenic salvage did not\nbecome popularized until the 1970s \n[12]\n. By 1980, it was the standard of care for traumatic grade II-IV\nsplenic injuries due to concern for overwhelming post-splenectomy infection and ill-defined postoperative\nimmunologic deficits. The technique itself is diverse and includes topical hemostatic agents, electrocautery,\nmesh application, partial resection, and omental patching \n[11]\n. It is possible that, in this case, omental\npatching from previous splenorrhaphy provided a splenic implantation site for this patient’s endometriosis.\nAs modern imaging has improved, the landscape of traumatic splenic injury has again shifted to primarily\nnonoperative management for hemodynamically stable trauma patients, and splenorrhaphy has since been\n2022 Krzeczowski et al. Cureus 14(2): e22012. DOI 10.7759/cureus.22012\n4\n of \n5\n\nabandoned \n[12,13]\n. The change in management for traumatic splenic injury and the paucity of splenic\nendometriosis suggests that this may be an exclusive case unlikely to be reencountered.\nConclusions\nOur literature review revealed that endometriosis of the spleen has not yet been described. While\nendometrial splenic implants by retrograde menstruation seem plausible, the unique presentation of\nintraparenchymal endometriosis implies a multifactorial etiology. Given the patient’s history of\nsplenorrhaphy, we suspect that retrograde menstruation coupled with iatrogenic seeding during splenic\nrepair is the most likely explanation. The current management of traumatic splenic injury has evolved away\nfrom splenorrhaphy. Therefore, we believe that this may remain a unique case unlikely to be encountered in\nthe future.\nAdditional Information\nDisclosures\nHuman subjects:\n Consent was obtained or waived by all participants in this study. \nConflicts of interest:\n In\ncompliance with the ICMJE uniform disclosure form, all authors declare the following: \nPayment/services\ninfo:\n All authors have declared that no financial support was received from any organization for the\nsubmitted work. \nFinancial relationships:\n All authors have declared that they have no financial\nrelationships at present or within the previous three years with any organizations that might have an\ninterest in the submitted work. \nOther relationships:\n All authors have declared that there are no other\nrelationships or activities that could appear to have influenced the submitted work.\nReferences\n1\n. \nKarfis EA, Roustanis E, Tsimoyiannis EC: \nSurgical management of nonparasitic splenic cysts\n. 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Pediatr Surg Int.\n2003, 19:617-27. \n10.1007/s00383-003-0972-y\n13\n. \nStassen NA, Bhullar I, Cheng JD, et al.: \nSelective nonoperative management of blunt splenic injury: an\nEastern Association for the Surgery of Trauma practice management guideline\n. J Trauma Acute Care Surg.\n2012, 73:S294-300. \n10.1097/TA.0b013e3182702afc\n2022 Krzeczowski et al. Cureus 14(2): e22012. DOI 10.7759/cureus.22012\n5\n of \n5","source_license":"CC0","license_restricted":false}