Abstract
Introduction
We present a case of three major lymphomesenteric cysts in a female patient with known basal cell nevus syndrome (BCNS or Gorlin–Goltz syndrome).
Discussion
Although those cysts have been reported to appear in this syndrome, the exact prevalence is not known. In this case, the patient suffered from severe and recurrent abdominal pain due to bowel obstruction.
Conclusion
By causing severe symptoms, cystic lymphangiomas are an important complication of the BCNS despite of their seldom occurrence. Surgeons confronted with intraabdominal masses in a patient with BCNS should always be aware of this manifestation of the disorder and plan interdisciplinary operations.
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References
Alqahtani A, Nguyen LT, Flageole H et al (1999) 25 years’ experience with lymphangiomas in children. J Pediatr Surg 34(7):1164–1168
Berry M, Monneins F, Delafolie A et al (1987) Diffuse cystic peritoneal lymphangiomatosis. Long-term clinical, ultrasonic and X-ray computed tomographic study. Apropos of a case. J Radiol 68(3):199–203
Bliss DP Jr, Coffin CM, Bower RJ et al (1994) Mesenteric cysts in children. Surgery 115(5):571–577
Block JB, Clendenning WE (1963) Parathyroid hormone hyporesponsiveness in patients with basal-cell nevi and bone defects. N Engl J Med 268:1157–1162
Chung JH, Suh YL, Park IA et al (1999) A pathologic study of abdominal lymphangiomas. J Korean Med Sci 14(3):257–262
Cohen MM Jr (2003) The hedgehog signaling network. Am J Med Genet A 123(1):5–28
Dalaker K (1978) Chylous cyst of the mesentery of the transverse colon. Acta Chir Scand 145:207
Evans DG, Ladusans EJ, Rimmer S et al (1993) Complications of the naevoid basal cell carcinoma syndrome: results of a population based study. J Med Genet 30(6):460–464
Földi M (1977) Erkrankungen des Lymphsystems, 1st edn. Baden-Baden, Brüssel (Witzstrock)
Gailani MR, Bale SJ, Leffell DJ et al (1992) Developmental defects in Gorlin syndrome related to a putative tumor suppressor gene on chromosome 9. Cell 69(1):111–117
Gorlin RJ (1995) Nevoid basal cell carcinoma syndrome. Dermatol Clin 13(1):113–125
Gorlin RJ (2004) Nevoid basal cell carcinoma (Gorlin) syndrome. Genet Med 6(6):530–539
Hahn H, Wicking C, Zaphiropoulous PG et al (1996) Mutations of the human homolog of Drosophila patched in the nevoid basal cell carcinoma syndrome. Cell 85(6):841–851
Hamdi A, Nouri A, Selmi M et al (1993) Abdominal cystic lymphangioma in children. Ann Chir 47(6):553–556
Hancock BJ, St-Vil D, Luks FI et al (1992) Complications of lymphangiomas in children. J Pediatr Surg 27(2):220–224 (discussion 224–226)
Johnson RL, Rothman AL, Xie J et al (1996) Human homolog of patched, a candidate gene for the basal cell nevus syndrome. Science 272(5268):1668–1671
Kimonis VE, Goldstein AM, Pastakia B et al (1997) Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 69(3):299–308
Knudson AG Jr (1971) Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci USA 68(4):820–823
Kronberger L (1970) A chyle cyst of the mesentery. Zentralbl Chir 95(41):1203–1206
Levanat S, Gorlin RJ, Fallet S et al (1996) A two-hit model for developmental defects in Gorlin syndrome. Nat Genet 12(1):85–87
Lorken M, Marnitz U, Manegold E, Schumpelick V (2001) Intra-abdominal lymphangioma. Chirurg 72(1):72–77
Marigo V, Davey RA, Zuo Y et al (1996) Biochemical evidence that patched is the Hedgehog receptor. Nature 384(6605):176–179
Mennecier D, Boucher E, Bey E et al (1999) Mesenteric cystic lymphangioma. Presse Med 28(12):629–631
Roisman I, Manny J, Fields S, Shiloni E (1989) Intra-abdominal lymphangioma. Br J Surg 76(5):485–489
Ros PR, Olmsted WW, Moser RP Jr et al (1987) Mesenteric and omental cysts: histologic classification with imaging correlation. Radiology 164(2):327–332
Shanley S, Ratcliffe J, Hockey A et al (1994) Nevoid basal cell carcinoma syndrome: review of 118 affected individuals. Am J Med Genet 50(3):282–290
Stone DM, Hynes M, Armanini M et al (1996) The tumour-suppressor gene patched encodes a candidate receptor for sonic hedgehog. Nature 384(6605):129–134
Stopinski J, Stephan S, Staib I (1994) Intra-abdominal cystic lymphangioma and mesenteric cysts as a cause of abdominal discomfort. Langenbecks Arch Chir 379(3):182–187
Taipale J, Cooper MK, Maiti T, Beachy PA (2002) Patched acts catalytically to suppress the activity of Smoothened. Nature 418(6900):892–897
Takiff H, Calabria R, Yin L, Stabile BE (1985) Mesenteric cysts and intra-abdominal cystic lymphangiomas. Arch Surg 120(11):1266–1269
Vargas-Serrano B, Alegre-Bernal N, Cortina-Moreno B et al (1995) Abdominal cystic lymphangiomas: US and CT findings. Eur J Radiol 19(3):183–187
Yang DM, Jung DH, Kim H et al (2004) Retroperitoneal cystic masses: CT, clinical and pathological findings and literature review. Radiographics 24(5):1353–1365
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Kayser, C., Kayser, G., Baier, P. et al. Surgery for cystic lymphangioma in Gorlin–Goltz syndrome. Langenbecks Arch Surg 392, 203–207 (2007). https://doi.org/10.1007/s00423-006-0126-5
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DOI: https://doi.org/10.1007/s00423-006-0126-5