<strong>June</strong> <strong>2007</strong>KUWAIT MEDICAL JOURNAL 173Mucinous or macrocystic cystadenoma presentin 40-50 year old females and are malignant orpotentially malignant. These lesions have largercysts (more than 2 cm in diameter) and areunilocular or multilocular. Thick septae, solidmural nodules and peripheral coarse calcificationsare present [2,8,9] .Intraductal papillary mucinous tumors are rare.Two-thirds of the patients are men, with a peak ageof incidence at sixty. Most patients present withpancreatitis. The lesion is characterized by markeddistension of the pancreatic duct with a larg eamount of mucus, leading to cyst formation. CTscan may reveal a cystic mass of the pancreas or adilated pancreatic duct which may be similar tochronic pancreatitis [3] .Cystic non-functioning islet cell tumors aresmall in size ranging from 4 mm to 2 cm and arevery vascular. They also have a different enhancingpattern with intravenous contrast [2] .Pseudocysts, though inflammatory in nature,deserve a mention in the list of diff e re n t i a ldiagnosis. They are the sequel of acute or chronicpancreatitis, trauma or pancreatic cancer. They areusually located within the pancreas but can presentin the retroperitoneum, mediastinum or even theparenchyma of the liver, kidney or spleen. They aresingle, unilocular with internal echoes due to fluidor debris. They lack a solid component and areencapsulated by fibrous tissue [8, 9] .The SPEN presents at a younger age than theabove mentioned tumors. They generally lackinternal septations and multiple loculations. Thel a rge size, the well-defined capsule, theheterogeneous mixed solid and cystic pattern andthe hemorrhagic component seen as high signalintensity on T1 weighted imaging serve todistinguish the SPEN from other cystic pancreaticneoplasms.With the appropriate clinical setting, theimaging findings can be highly suggestive for thediagnosis of SPEN. This tumor should be primarilyconsidered in a young female presenting with alarge, well-defined cystic mass in the pancreas,with heterogeneous pattern. An accurate diagnosisis invaluable, since total surgical resection has anexcellent prognosis.REFERENCES1. Mergo PJ, Helmberger TK, Buetow PC, Helmberger RC,Ros PR. Pancreatic Neoplasms: MR imaging and pathologiccorrelation. Radiographics 1997; 17:281-301.2. Buetow PC, Buck JL, Pantongrag-Brown L, Beck KG, RosPR, Adair CF. Solid and Papillary neoplasm of the pancreas:imaging and pathologic correlation in 56 cases. Radiology1996; 199:707-711.3. Demos TC, Posniak HV, Harmath C, Olson MC, Aranha G.Cystic lesions of the pancreas. AJR 2002; 179:1375-1388.4. Bennet GL, Hann LE. Pancreatic Ultrasonography. SurgClin N Am 2001; 81:259-277.5. Dong PR, Lu DSK, Degregario F, Fell SC, Au A., Kadell BM.Solid and papillary neoplasm of the pancreas: radiologicalpathologicalstudy of five cases and review of literature.Clin Radiol 1996; 51:701-705.6. Hammond N, Miller FH, Sica GT, Gore RM. Imaging ofcystic diseases of the pancreas. Radiol Clin N Am 2002;40:1243-1262.7. Megibow AJ, Lavelle MT, Rofsky NM. MR imaging of thepancreas. Surg Clin N Am 2001; 81:307-320.8. Megibow AJ, Lavelle MT, Rofsky NM. Cystic tumors of thePancreas: The radiologist. Surg Clin N Am 2001; 81:489-495.9. Sarr MG, Kendrick ML, Nagorney DM, Thompson GB,Farley DR, Farnell MB. Cystic Neoplasms of the Pancreas -Benign to Malignant epithelial Neoplasms. Surg Clin N Am2001; 81:497-506.
174KUWAIT MEDICAL JOURNAL <strong>June</strong> <strong>2007</strong>Case ReportSAPHO: an Unusual Cause of Pulmonary NodulesNufoud Al- Shammari 1 , Alexander J Henderson 2 , Cameron TC Kennedy 3 , Simon C Langton Hewer 41Department of Pediatrics, Pulmonary Unit, Mubarak Al-Kabeer Hospital, Kuwait2,3,4Departments of Pediatric Respiratory Medicine and Pediatric Dermatology, Bristol Royal Hospital for Sick Children,Bristol BS2 8BJ, UKABSTRACTSAPHO is a clinical syndrome comprising of synovitis,acne, pustulosis, hyperostosis and osteitis. We report acase of SAPHO presenting in a child re f e r red forinvestigations of persistent radiological abnormalities inKuwait Medical Journal <strong>2007</strong>, <strong>39</strong> (2):174-176KEYWORDS : child, pulmonary nodules, lung disease, SAPHOthe chest. Pulmonary involvement in SAPHO has beenreported rare l y, is asymptomatic and may be morecommon than has been described.INTRODUCTIONSAPHO is a clinical syndrome comprising ofsynovitis, acne, pustulosis, hyperostosis and osteitis.Pulmonary involvement has been described rarelybut this cause of multiple nodular lesions on chestradiography should be differentiated from otherpossible diagnoses as it follows a benign course andrequires no specific therapy. Here we present a caseof SAPHO presenting in a child re f e r red forinvestigation of persistent radiological abnormalitiesin the chest.Case presentationA ten-year-old boy was assessed for persistentlung X-ray abnormalities eleven months after aprimary illness with myalgia and an erythematousrash with pustular eruptions on the soles of bothfeet. He made an intial recovery but two monthslater he developed a widespread, papular eruptionwith pustule formation. This was most florid on theexposed areas of the limbs and was associated witharthralgia, abdominal pain and muscle weakness.He was investigated for an infective cause of thisillness but cultures and serology were not positive.During the ensuing six months the rash relapsedand remitted.Four months after his initial symptoms, hedeveloped swelling of the left angle of hismandible. A dental abscess was diagnosed and hehad extraction of a primary molar. Cytologicalexamination of material from the site demonstratednon-specific, chronic inflammatory changes. Onemonth later he developed another swelling over themedial end of his left clavicle. This persisted forone month and resolved spontaneously. Chest X-rays taken six weeks apart showed multiple lesionsin the left lower lobe and costophrenic angle. Hehad no respiratory symptoms at this time but theradiological lesions persisted for six months and hewas referred to us for advice.Examination demonstrated a swelling over theleft sternoclavicular joint (Fig. 1) and scaly papuleswith occasional pustules distributed mainly on histrunk but with peripheral lesions (Fig. 2). Biopsy ofa skin lesion demonstrated mild, superficial,perivascular dermatitis. He had no pulmonarysymptoms or signs and his pulmonary functionwas normal. Computed tomography of the chest(Fig. 3) showed areas of density at both lung basesthat were consistent with areas of radiologicalabnormalities on plain chest X-ray (Fig. 4). Therewas no evidence of interstitial lung disease. Thelesions were judged to be previous areas ofi n f a rction with subsequent residual fibro s i s .Extensive investigations, including autoimmuneprofile, atypical pneumonia screen, Borrelia titers,T and B cell subsets, neutrophil function, antiHiband Tetanus titer and Mantoux test did notdemonstrate any evidence of autoimmune disease,immune deficiency or infection. His lung functiontests were normal and bone scan showed areas inhis right clavicle consistent with osteitis.We believe that the combination of osteitis of theclavicle and plantar pustulosis in our patient isconsistent with SAPHO syndrome.Address correspondence to:Nufoud Al - Shammari, Department of Pediatrics, Pulmonary Unit, Mubarak Al - Kabeer Hospital, P.O Box: 43787, Code: 32052, Hawalli,Kuwait. Tel: 965-5318502, Fax : 965-5311430, E-mail: nufoud_q8@hotmail.com