The curious case of a man with freckles and obscure gastrointestinal bleed

Abstract

1. Castleman B, Towne VW. CASE records of the Massachusetts General Hospital Weekly Clinicopathological Exercises: Case 40011. N Engl J Med. 1954;250:26–30. 2. Kimura T, Inoue T, Katayama K, Hirose K, Imamura Y, Yamaguchi A. Mesenteric Castleman’s disease: report of a case. Surg Today. 2002;32:651–4. 3. Rosai J. Rosai and Ackerman’s surgical pathology. 9th ed. New Delhi: Elsevier; 2004:1905–8. 4. Francis ND, Hollowood K, Gabriel R. Angiofollicular lymph node hyperplasia. J Clin Pathol. 1988;41:353–4. 5. Cesarman E, Knowles DM. Kaposi’s sarcoma-associated herpesvirus: a lymphotropic human herpesvirus associated with Kaposi’s sarcoma, primary effusion lymphoma, and multicentric Castleman’s disease. Semin Diagn Pathol. 1997;14:54–66. 6. Frizzera G. Castleman’s diesease: more questions than answers. Hum Pathol. 1985;156:202–5. 7. Yoshizaki K, Matsuda T, Nishimoto N, Kuritani T, Taeho L, Aozasa K, et al. Pathogenic significance of interleukin-6 (IL-6/ BSF-2) in Castleman’s disease. Blood. 1989;74:1360–7. unremarkable except for multiple cutaneous neurofibromas, axillary freckles and cafe au lait spots (Figure 1). Haematological tests revealed microcytic hypochromic anaemia and faecal occult blood was positive. Both upper GI endoscopy (UGIE) and colonoscopy were normal. CT angiogram of the abdomen (Figure 2) revealed the presence of two masses 3 cm × 2 cm in size, enhancing well with contrast, located in the proximal and mid jejunum and supplied by branches of the superior mesenteric artery. After obtaining an informed consent and preoperative packed cell transfusions, the patient was taken up for diagnostic laparoscopy. Findings at laparoscopy included two jejunal tumours as described above and additional