Ann Hepatobiliary Pancreat Surg.  2023 Feb;27(1):1-5. 10.14701/ahbps.22-062.

Littoral cell angiomas: Benign lesion with a penchant for visceral malignancies

• Gulati S
• Jeon H
• Vijay A

• KMID: 2539508
• DOI: http://doi.org/10.14701/ahbps.22-062

Abstract

Littoral cell angiomas are rare vascular tumors of the spleen. Because of their rarity, unclear etiopathogenesis, and association with other malignancies, these tumors can pose diagnostic and therapeutic challenges. Due to paucity of published literature on this entity often limited to case reports, relevant data on this topic were procured and synthesized with the aid of a comprehensive Medline search in addition to oncologic, pathologic, radiologic, and surgical literature review on littoral cell angiomas. This article provides an in-depth review into postulated etiopathogenesis, pathology, clinical manifestations, associated malignancies, and prognostic features of littoral cell angiomas.

Keyword

Spleen; Splenic neoplasms; Littoral cell angioma; Surgery; Cells

Figure

• 

  Fig. 1 Early contrast-enhanced helical computed tomography scan showing enlarged spleen containing innumerable small, focal, low-density lesions.

• 

  Fig. 2 Serial sections of spleen showing that the parenchyma is notable for multiple hemorrhagic cystic lesions diffusely spread throughout the parenchyma.

• 

  Fig. 3 Histologic specimen showing normal splenic parenchyma (large arrow) and adjacent vascular proliferation of cavernous, blood-filled spaces (small arrow) (H&E, ×20).

Reference

1. Falk S, Stutte HJ, Frizzera G. 1991; Littoral cell angioma. A novel splenic vascular lesion demonstrating histiocytic differentiation. Am J Surg Pathol. 15:1023–1033. DOI: 10.1097/00000478-199111000-00001. PMID: 1928554.

2. Gakenheimer-Smith L, Mohlman J, VandenHeuvel K, Jackson WD, Thomsen W, Stevenson A, et al. 2018; A novel presentation of littoral cell angioma and lymphatic malformations in a neonate. Pediatrics. 141(Suppl 5):S520–S525. DOI: 10.1542/peds.2017-2782. PMID: 29610184.

3. Priego P, Rodríguez Velasco G, Griffith PS, Fresneda V. 2008; Littoral cell angioma of the spleen. Clin Transl Oncol. 10:61–63. DOI: 10.1007/s12094-008-0155-3. PMID: 18208795.

4. Español I, Lerma E, Fumanal V, Palmer J, Roca M, Domingo-Albós A, et al. 2000; Littoral cell angioma with severe thrombocytopenia. Ann Hematol. 79:46–49. DOI: 10.1007/s002770050009. PMID: 10663622.

5. Schneider G, Uder M, Altmeyer K, Bonkhoff H, Gruber M, Kramann B. 2000; Littoral cell angioma of the spleen: CT and MR imaging appearance. Eur Radiol. 10:1395–1400. DOI: 10.1007/s003300000345. PMID: 10997426.

6. Zhang YH, Liu LM, Wang WP, Ding H, Wang XN, Xia HS. 2013; Littoral cell angioma of the spleen: sonographic-pathologic comparison. J Ultrasound Med. 32:691–697. DOI: 10.7863/jum.2013.32.4.691. PMID: 23525396.

7. Veillon DM, Williams RB, Sardenga LJ, Harrison GK, Cotelingam JD. 2000; 'Little' littoral cell angioma of the spleen. Am J Surg Pathol. 24:306–307. DOI: 10.1097/00000478-200002000-00023. PMID: 10680900.

8. Truong V, Finch R, Martin B, Buzacott K, Singh M, Patel B. 2019; Littoral cell angioma of spleen. ANZ J Surg. 89:E158–E159. DOI: 10.1111/ans.14193. PMID: 29024432.

9. O'Malley DP, Kim YS, Weiss LM. 2015; Distinctive immunohistochemical staining in littoral cell angioma using ERG and WT-1. Ann Diagn Pathol. 19:143–145. DOI: 10.1016/j.anndiagpath.2015.02.007. PMID: 25792460.

10. Tan YM, Chuah KL, Wong WK. 2004; Littoral cell angioma of the spleen. Ann Acad Med Singap. 33:524–526.

11. Mühlfeld AS, Eitner F, Perez-Bouza A, Knuechel R, Heintz B, Floege J. 2008; Littoral cell angioma of the spleen mimicking posttransplantation lymphoma in a 63-year-old renal transplant patient. Am J Kidney Dis. 52:e11–e14. DOI: 10.1053/j.ajkd.2008.01.033. PMID: 18479795.

12. Gardner JA, Devitt K. 2017; Incidental littoral cell angioma in refractory immune thrombocytopenic purpura. Blood. 129:1564. DOI: 10.1182/blood-2016-10-748772. PMID: 28302694.

13. Erçin C, Gürbüz Y, Hacihanefioğlu A, Turgut Karakaya A. 2005; Multiple littoral cell angioma of the spleen in a case of myelodysplastic syndrome. Hematology. 10:141–144. DOI: 10.1080/10245330400026121. PMID: 16019460.

14. Mac New HG, Fowler CL. 2008; Partial splenectomy for littoral cell angioma. J Pediatr Surg. 43:2288–2290. DOI: 10.1016/j.jpedsurg.2008.07.031. PMID: 19040956.

15. Cordesmeyer S, Pützler M, Titze U, Paulus H, Hoffmann MW. 2011; Littoral cell angioma of the spleen in a patient with previous pulmonary sarcoidosis: a TNF-α related pathogenesis? World J Surg Oncol. 9:106. DOI: 10.1186/1477-7819-9-106. PMID: 21929754. PMCID: PMC3187736.

16. Gorman D, Bergstrom R. 2016; Littoral cell angioma in a patient with Ehlers-Danlos syndrome on biologic immunosuppression for psoriatic arthritis. Int J Case Rep Images. 7:800–804.

17. Collins GL, Morgan MB, Taylor FM 3rd. 2003; Littoral cell angiomatosis with poorly differentiated adenocarcinoma of the lung. Ann Diagn Pathol. 7:54–59. DOI: 10.1053/adpa.2003.50009. PMID: 12616475.

18. Roldan-Vasquez E, Roldan-Vasquez A, Jarrin-Estupiñan X, Roldan-Crespo J. 2021; Case report: Infrequent littoral cell angioma of the spleen. Int J Surg Case Rep. 85:106242. DOI: 10.1016/j.ijscr.2021.106242. PMID: 34333257. PMCID: PMC8346658.

19. Ziske C, Meybehm M, Sauerbruch T, Schmidt-Wolf IG. 2001; Littoral cell angioma as a rare cause of splenomegaly. Ann Hematol. 80:45–48. DOI: 10.1007/s002770000223. PMID: 11233776.

20. Gupta MK, Levin M, Aguilera NS, Pastores GM. 2001; Littoral cell angioma of the spleen in a patient with Gaucher disease. Am J Hematol. 68:61–62. DOI: 10.1002/ajh.1151. PMID: 11559940.

21. Forest F, Duband S, Clemenson A, Peoc'h M. 2010; Traumatic subcapsular splenic hematoma revealing littoral cell angioma and Gaucher's disease. Ann Hematol. 89:1061–1062. DOI: 10.1007/s00277-010-0909-1. PMID: 20155266.

22. Antón-Pacheco J, Ayuso RM, Cano I, Martinez MA, Cuadros J, Berchi FJ. 2000; Splenic littoral cell angioma in an infant. J Pediatr Surg. 35:508–509. DOI: 10.1016/S0022-3468(00)90225-2. PMID: 10726700.

23. Sarandria JJ, Escano M, Kamangar F, Farooqui SO, Montgomery E, Cunningham SC. 2014; Littoral cell angioma: gastrointestinal associations. Gastrointest Cancer Res. 7:63–64.

24. Johansson J, Björnsson B, Ignatova S, Sandström P, Ekstedt M. 2015; Littoral cell angioma in a patient with Crohn's disease. Case Rep Gastrointest Med. 2015:474969. DOI: 10.1155/2015/474969. PMID: 25705528. PMCID: PMC4326338.

25. Nagarajan P, Cai G, Padda MS, Selbst M, Kowalski D, Proctor DD, et al. 2011; Littoral cell angioma of the spleen diagnosed by endoscopic ultrasound-guided fine-needle aspiration biopsy. Diagn Cytopathol. 39:318–322. DOI: 10.1002/dc.21384. PMID: 21488173.

26. Bhatt S, Huang J, Dogra V. 2007; Littoral cell angioma of the spleen. AJR Am J Roentgenol. 188:1365–1366. DOI: 10.2214/AJR.06.1157. PMID: 17449783.

27. Pilz JB, Sperschneider T, Lutz T, Loosli B, Maurer CA. 2011; Littoral cell angioma in main and accessory intrapancreatic spleen presenting as splenic rupture. Am J Surg. 201:e15–e17. DOI: 10.1016/j.amjsurg.2009.11.013. PMID: 20409532.

28. Steensma DP, Morice WG. 2000; Littoral cell angioma associated with portal hypertension and resected colon cancer. Acta Haematol. 104:131–134. DOI: 10.1159/000039747. PMID: 11154990.

29. Opatrny V, Treska V, Waloschek T, Molacek J. 2020; Littoral cell angioma of the spleen: a case report. SAGE Open Med Case Rep. 8:2050313X20959874. DOI: 10.1177/2050313X20959874. PMID: 33088569. PMCID: PMC7543100.

30. Bhavsar T, Wang C, Huang Y, Karachristos A, Inniss S. 2011; Littoral cell angiomas of the spleen associated with solid pseudopapillary tumor of the pancreas. World J Gastrointest Pathophysiol. 2:53–56. DOI: 10.4291/wjgp.v2.i3.53. PMID: 21860837. PMCID: PMC3158892.

31. Heese J, Bocklage T. 2000; Specimen fine-needle aspiration cytology of littoral cell angioma with histologic and immunohistochemical confirmation. Diagn Cytopathol. 22:39–44. DOI: 10.1002/(SICI)1097-0339(200001)22:1<39::AID-DC11>3.0.CO;2-Q.

32. Floyd JD, Kaplan PA, Sauter ER, Perry MC, Doll DC. 2005; Patients with unusual bladder malignancies and a rare cause of splenomegaly. Case 3. Littoral cell angioma of the spleen in a patient with previous lymphoma. J Clin Oncol. 23:4460–4462. DOI: 10.1200/JCO.2005.05.066. PMID: 15994157.

33. Melzer N, Barth PJ, Müller KM, Foss HD, Krug U, Schilling M, et al. 2012; Rapidly progressive B-cell dominated inflammatory neuropathy and littoral cell angioma of the spleen associated with plasmablastic B-cell lymphoma. Leuk Lymphoma. 53:1242–1244. DOI: 10.3109/10428194.2011.640677. PMID: 22098375.

34. Marzetti A, Messina F, Prando D, Verza LA, Vacca U, Azabdaftari A, et al. 2015; Laparoscopic splenectomy for a littoral cell angioma of the spleen: case report. World J Clin Cases. 3:951–955. DOI: 10.12998/wjcc.v3.i11.951. PMID: 26601099. PMCID: PMC4644898.

35. Chatelain D, Bonte H, Guillevin L, Balladur P, Flejou JF. 2002; Small solitary littoral cell angioma associated with splenic marginal zone lymphoma and villous lymphocyte leukaemia in a patient with hepatitis C infection. Histopathology. 41:473–475. DOI: 10.1046/j.1365-2559.2002.14313.x. PMID: 12405918.

36. Yano H, Imasato M, Monden T, Okamoto S. 2003; Hand-assisted laparoscopic splenectomy for splenic vascular tumors: report of two cases. Surg Laparosc Endosc Percutan Tech. 13:286–289. DOI: 10.1097/00129689-200308000-00014. PMID: 12960796.

37. Harmon RL, Cerruto CA, Scheckner A. 2006; Littoral cell angioma: a case report and review. Curr Surg. 63:345–350. DOI: 10.1016/j.cursur.2006.06.011. PMID: 16971207.

38. Shah S, Wasnik A, Pandya A, Bude RO. 2011; Multimodality imaging findings in image-guided biopsy proven splenic littoral cell angioma: series of three cases. Abdom Imaging. 36:735–738. DOI: 10.1007/s00261-011-9697-x. PMID: 21318377.

39. Mohan V, Jones RC, Drake AJ 3rd, Daly PL, Shakir KM. 2005; Littoral cell angioma presenting as metastatic thyroid carcinoma to the spleen. Thyroid. 15:170–175. DOI: 10.1089/thy.2005.15.170. PMID: 15753678.

40. Yaney A, Jones D, Perry KA, Jhawar SR. 2022; Rare case of littoral cell angioma recognised on CT simulation for adjuvant radiation treatment for early stage breast cancer. BMJ Case Rep. 15:e248167. DOI: 10.1136/bcr-2021-248167. PMID: 35131803.

41. Kranzfelder M, Bauer M, Richter T, Rudelius M, Huth M, Wagner P, et al. 2012; Littoral cell angioma and angiosarcoma of the spleen: report of two cases in siblings and review of the literature. J Gastrointest Surg. 16:863–867. DOI: 10.1007/s11605-011-1773-6. PMID: 22068970.

42. Ben-Izhak O, Bejar J, Ben-Eliezer S, Vlodavsky E. 2001; Splenic littoral cell haemangioendothelioma: a new low-grade variant of malignant littoral cell tumour. Histopathology. 39:469–475. DOI: 10.1046/j.1365-2559.2001.01242.x. PMID: 11737304.

43. Fernandez S, Cook GW, Arber DA. 2006; Metastasizing splenic littoral cell hemangioendothelioma. Am J Surg Pathol. 30:1036–1040. DOI: 10.1097/00000478-200608000-00016. PMID: 16861977.

44. Takayoshi K, Doi G, Tsuruta N, Yoshihiro T, Nio K, Tsuchihashi K, et al. 2018; Successful chemotherapeutic treatment for metastatic littoral cell angioma: a case report. Medicine (Baltimore). 97:e0378. DOI: 10.1097/MD.0000000000010378. PMID: 29642193. PMCID: PMC5908586.

45. Hu ZQ, A YJ, Sun QM, Li W, Li L. 2011; The splenic littoral cell angioma in China: a case report and review. World J Surg Oncol. 9:168. DOI: 10.1186/1477-7819-9-168. PMID: 22172167. PMCID: PMC3271992.

46. Wang Z, Zhang L, Zhang B, Mu D, Cui K, Li S. 2015; Hemangioendothelioma arising from the spleen: a case report and literature review. Oncol Lett. 9:209–212. DOI: 10.3892/ol.2014.2693. PMID: 25435960. PMCID: PMC4246698.

47. Wheelwright M, Spartz EJ, Skubitz K, Yousaf H, Murugan P, Harmon JV. 2021; Primary angiosarcoma of the spleen, a rare indication for splenectomy: a case report. Int J Surg Case Rep. 82:105929. DOI: 10.1016/j.ijscr.2021.105929. PMID: 33957408. PMCID: PMC8113851.