Clinical Review & Education

JAMA Surgery Clinical Challenge

Young Woman With Massive Splenomegaly Katherine E. Poruk, MD; Skye C. Mayo, MD, MPH; Peter Cornell, ACNP; Frederic E. Eckhauser, MD

Figure 1. Magnetic resonance image demonstrating splenomegaly.

An otherwise healthy woman in her early 20s was referred to our general surgery clinic for massive splenomegaly after developing a migratory, urticaric maculopapular rash over her body earlier in the year. On physical examination, the inferior tip of the spleen extended well below the costal margin into her left lower quadrant. In addition to the rash and spleQuiz at nomegaly, the patient also described several months of fatigue, early satiety, and back pain. However, she denied other constitutional symptoms such as fevers, night sweats, chills, arthralgias, or weight loss. She did not have any personal history of mononucleosis, Lyme disease, arthritis, or bleeding disorders. In addition, there was no family history of hematologic diseases or lymphoma. Findings from a hematologic workup were unremarkable. Crosssectional imaging was performed (Figure 1).


A. Splenic pelosis B. Echinococcal splenic cystic disease C. Splenic lymphangiomatosis D. Non-Hodgkins lymphoma

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Clinical Review & Education JAMA Surgery Clinical Challenge

Diagnosis C. Splenic lymphangiomatosis

Discussion Splenic lymphangiomatosis (SL) is a rare and often incidentally discovered condition characterized by diffuse replacement of the normal splenic parenchyma with benign cysts of various sizes.1 Patients areoftenasymptomaticuntilthespleenislargeenoughtocausesymptoms, such as pain or early satiety, or self-discovery of an abdominal mass. Only 12 other case reports of isolated SL without lymphangiomatosis involving other organs have appeared in the literature to A


Figure 2. A, Gross appearance of the spleen immediately after splenectomy. B, Pathologic sectioning of the spleen demonstrating multiple cysts.

ARTICLE INFORMATION Author Affiliations: Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland. Corresponding Author: Frederic E. Eckhauser, MD, Johns Hopkins Hospital, 600 N Wolfe St, Blalock 618, Baltimore, MD 21287 ([email protected]). Section Editor: Carl E. Bredenberg, MD.

2. Wadsworth DT, Newman B, Abramson SJ, Carpenter BL, Lorenzo RL. Splenic lymphangiomatosis in children. Radiology. 1997; 202(1):173-176.

8. Kwon AH, Inui H, Tsuji K, Takai S, Imamura A, Kamiyama Y. Laparoscopic splenectomy for a lymphangioma of the spleen: report of a case. Surg Today. 2001;31(3):258-261.

3. Spapen HD, Reynaert H, Debeuckelaere S, Achten E, Somers G. An unusual case of cystic lymphangiomatosis of the spleen. Neth J Med. 1990;37(1-2):24-26.

9. Barrier A, Lacaine F, Callard P, Huguier M. Lymphangiomatosis of the spleen and 2 accessory spleens. Surgery. 2002;131(1):114-116.

4. Gómez A, Toscano R, Sánchez E, Vara C. Splenic lymphangiomatosis. J Chir (Paris). 1992;129(1):35-37.

Published Online: June 11, 2014. doi:10.1001/jamasurg.2013.3209. Conflict of Interest Disclosures: None reported. REFERENCES 1. Morgenstern L, Bello JM, Fisher BL, Verham RP. The clinical spectrum of lymphangiomas and lymphangiomatosis of the spleen. Am Surg. 1992; 58(10):599-604.


date.1-12 The diagnosis of SL is often made only after infectious, hematologic, malignant, and congenital causes have been ruled out. In our patient, because of the extent of the compressive symptoms related to her massive spleen and an unclear diagnosis after an extensive laboratory workup, splenectomy was recommended. She underwentanopensplenectomythroughaleftsubcostalincision.Toremove the large spleen without extending the incision, the splenic artery was ligated early in the operation, allowing the spleen to decompress and decrease in size (Figure 2A). The final pathology showed diffuse lymphangioma of the spleen, which measured 20 × 12 × 10.5 cm and weighed 1225 g. Multiple cysts lined with flattened epithelium were seen (Figure 2B). The approach to splenomegaly can be complex. Many possible etiologies exist including congenital disorders, infection, inflammation, hematologic diseases, or malignancy. In some cases, the workup findings are negative, leading to the consideration of other diagnoses. In children, it can be seen with congenital metabolic disorders. Several viral, parasitic, and bacterial infections are also associated with spleen enlargement. Splenomegaly can also be seen with inflammatory disorders. The spleen can become enlarged by increased venous pressure due to obstructed blood flow. Primary splenic tumors can cause splenomegaly and can be divided into lymphoid and nonlymphoid tumors.13 Hodgkin and non-Hodgkin lymphoma, histiocytic lymphoma, and plasmacytoma comprise the most common primary lymphoid tumors. Vascular tumors are the most common nonlymphoid splenic tumors. Splenic lymphangiomatosis is a rare cause of splenomegaly. The spleen may be the only involved organ or it may be associated with multisystem lesions.2 It is rare to have lymphangiomatosis limited only to the spleen.2,5 Further, SL is believed to be due to a developmental failure of the lymphatic system, leading to the formation of lymphangiomas; as such, it is believed by some to be a congenital developmental defect.6,9 Cross-sectional imaging often demonstrates a low-density nonenhancing, thin-walled splenic cyst with crisply defined margins.3,6 It is a benign condition; patients followed up as far as 20 years have remained asymptomatic.2 At 1 month of follow-up, our patient returned to her normal activities without any symptoms. While SL remains a rare disorder, the diagnosis should be considered in young patients with splenomegaly.

5. Panich V. Splenic cystic lymphangiomatosis: an unusual cause of massive splenomegaly: report of a case. J Med Assoc Thai. 1994;77(3):165-168. 6. Bader TR, Ranner G, Klimpfinger M. Case report: CT appearance of capillary and cavernous lymphangiomatosis of the spleen in an adult. Clin Radiol. 1998;53(5):379-381. 7. Talarico C, Cerasoli V, Mancini B, et al. Lymphangiomatosis of the spleen: report of a clinical case. Ann Ital Chir. 2000;71(5):599-602.

10. Patti R, Iannitto E, Di Vita G. Splenic lymphangiomatosis showing rapid growth during lactation: a case report. World J Gastroenterol. 2010;16(9):1155-1157. 11. Ozel A, Uysal E, Dokucu AI, Erturk SM, Basak M, Cantisani V. US, CT and MRI findings in a case of diffuse lymphangiomatosis and cystic hygroma. J Ultrasound. 2008;11(1):22-25. 12. Pistoia F, Markowitz SK. Splenic lymphangiomatosis: CT diagnosis. AJR Am J Roentgenol. 1988;150(1):121-122. 13. Giovagnoni A, Giorgi C, Goteri G. Tumours of the spleen. Cancer Imaging. 2005;5(1):73-77.

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Young woman with massive splenomegaly.

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