Gorham-Stout syndrome: the radiologic-pathologic correlation as a diagnostic pathway when bone is vanishing

http://dx.doi.org/10.1590/0100-3984.2017.0167 Rômulo Florêncio Tristão Santos1,a, Tiago Kojun Tibana1,b, Isa Félix Adôrno1,c, Edson Marchiori2,d, Thiago Franchi Nunes1,e 1. Universidade Federal de Mato Grosso do Sul (UFMS), Campo Grande, MS, Brazil. 2. Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil. Correspondence: Dr. Edson Marchiori. Rua Thomaz Cameron, 438, Valparaiso. Petrópolis, RJ, Brazil, 25685-120. Email: edmarchiori@gmail.com. a. 0000-0002-8679-7369; b. 0000-0001-5930-1383; c. 0000-0002-2106-1211; d. 0000-0001-8797-7380; e. 0000-0003-0006-3725. Received 19 September 2017. Accepted after revision 9 October 2017. expiration. The treatment is generally supportive, including respiratory therapy, appropriate antibiotic therapy for the recurrent infections, and smoking cessation. In conclusion, Mounier-Kuhn syndrome should be considered in patients who present with bronchiectasis accompanied by abnormal dilatation of the trachea and the main bronchi. The preferred diagnostic imaging method is CT of the chest.

Gorham-Stout syndrome: the radiologic-pathologic correlation as a diagnostic pathway when bone is vanishing

Dear Editor,
A 34-year-old previously healthy man presented with a 12-month history of progressive polyarthralgia and edema of the hips, right ankle, and intercostal spaces. He reported no history of trauma. Conventional radiography revealed several mixed lesions (predominantly osteolytic lesions) in the pelvic ring, proximal femur, distal femur, distal tibia, both tali, and lumbar vertebral bodies, as well as unconsolidated fractures of the costal arches, with no periosteal reaction or associated soft tissue changes ( Figure 1). The initial hypotheses of multifocal osteolysis were secondary osteolytic conditions such as infection, cancer (primary or metastatic), inflammatory disorders, and endocrine disorders. The results of laboratory tests (complete blood count, protein profiles, parathyroid hormone level, ionic calcium level, and phosphate level) were normal, as were those of computed tomography staging. After the primary hypothesis of multifocal osteolysis had been excluded, the radiological hypothesis of Gorham-Stout syndrome was proposed. The patient was submitted to surgical biopsy of the right iliac bone, and the histopathology demonstrated spongy bone marrow tissue, the bone marrow having been replaced by fibrovascular tissue with innumerable capillary and cavernous vessels, cortical bone resorption, and reactive immature bone, consistent with massive osteolysis (Figure 1).
Initially described as "missing bone disease with intraosseous vascular alterations" (1,2) , the monocentric form of osteolysis of a bone or contiguous area of bone, with a predilection for the axial skeleton, was subsequently dubbed Gorham-Stout syndrome. The process is usually monostotic, although there have been a few reports of cases in which it was polyostotic (3) . In decreasing order of frequency, it affects the scapula, proximal end of the humerus, femur, rib, iliac bone, ischium, and sacrum (3) . It is currently known by a variety of names, including massive osteolysis, idiopathic osteolysis, vanishing bone disease, disappearing bone disease, phantom bone disease, spontaneous bone absorption, progressive bone atrophy, bone hemangiomatosis, and lymphangiomatosis of bone.
Gorham-Stout syndrome primarily affects children and young adults, the affected individuals presenting nonspecific complaints such as pain and joint edema. The pathological mechanisms proposed to date are multifactorial, with no known triggering factor. The histopathological hallmark is the replacement of normal bone by fibrous tissue with aggressive, expansile, non-neoplastic proliferation of capillary or cavernous blood vessels (3-7) , a process that can culminate in the replacement of the entire bone by fibrous tissue (7) . Histopathologically and radiologically, the syndrome initially presents as foci of rarefaction in the bone marrow and subcortical bone, with slow, irregular progression that can result in effacement of the diaphysis of the bones, narrowing of the involved ends, and, in some cases, the complete disappearance of the bone. Pathological fractures that do not consolidate are common and are characteristic of the syndrome (1,8) . The pathological-radiological diagnosis proposed by Heffez et al. (6) is based on specific criteria, including positive biopsy findings for angiomatous tissue and an osteolytic pattern Intestinal obstruction by a phytobezoar in a patient with a history of gastroplasty

Dear Editor,
We report the case of a 28-year-old female patient with a one-year history of gastroplasty who was suffering from abdominal pain one day after eating a large amount of jackfruit. The physical exam revealed diffuse pain on palpation, positive abrupt decompression and absence of bowel sounds. Computed tomography (CT) of the abdomen and pelvis showed signs of intestinal obstruction. Intraoperatively, intraluminal content, consistent with a phytobezoar (a jackfruit "bolus"), was observed impacting the distal anastomosis of the gastric bypass ( Figure 1).
A bezoar is a mass of exogenous undigested material that accumulates in the gastrointestinal tract, usually in the stomach or ileus, and causes intestinal obstruction (1) . Bezoars are associated with predisposing factors such as poor mastication, psychiatric disorders, and impaired gastric motility.