• Nenhum resultado encontrado

Radiol Bras vol.45 número4

N/A
N/A
Protected

Academic year: 2018

Share "Radiol Bras vol.45 número4"

Copied!
2
0
0

Texto

(1)

XI

Radiol Bras. 2012 Jul/Ago;45(4):XI–XII

Paulo Moraes Agnollitto1, Marcello Henrique Nogueira-Barbosa2

0100-3984 © Colégio Brasileiro de Radiologia e Diagnóstico por Imagem

WHICH IS YOUR DIAGNOSIS?

Agnollitto PM, Nogueira-Barbosa MH. Which is your diagnosis? Radiol Bras. 2012 Jul/Ago;45(4):XI–XII.

A female, 22-year-old patient presented with a soft tissue lesion adjacent to the cal-varia. Skull radiography was performed as part of the preoperative investigation for the lesion excision (Figure 1). In order to supplement the evaluation of incidental radiographic findings, the patient was submitted to computed tomography (CT) of the facial sinuses (Figures 2 and 3).

Study developed at Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (HCFMRP-USP), Ribeirão Preto, SP, Brazil. 1. MD, Resident of Radiology at Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Univer-sidade de São Paulo (HCFMRP-USP), Ribeirão Preto, SP, Brazil. 2. PhD, Professor, Division

of Radiology, Department of Medical Practice, Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil. Mailing Address: Dr. Paulo Moraes Agnollitto. Avenida Caramuru, 2300, República. Ribeirão Preto, SP, Brazil, 14030-000. E-mail: agno53@gmail.com

Figure 1. Skull radiography. Posteroanterior (A) and lateral (B) views.

A

B

Figure 2. Non-contrast-enhanced CT, bone window.

A

B

Figure 3. Coronal CT sections, bone window.

A

(2)

XII Radiol Bras. 2012 Jul/Ago;45(4):XI–XII Images Description

Figure 1. Skull radiography, posteroan-terior and lateral views demonstrating in-cidentally found osteoblastic lesion with projection into the facial sinuses and man-dible.

Figure 2. Non-contrast-enhanced axial CT sections, bone window, confirming the presence of multiple osteoblastic lesions in the facial sinuses. Such lesions project into the air spaces of the paranasal sinuses.

Figure 3. Coronal CT sections, bone window, demonstrating multiple osteoblas-tic lesions in the facial sinuses and man-dible. The lesion on the mandible shows exophytic growth. The findings are sugges-tive of multiple osteomas.

Diagnosis: Gardner’s syndrome.

COMMENTS

Gardner’s syndrome is a variant of fa-milial adenomatous polyposis which is a dominant autosomal disease characterized by the presence of multiple adenomatous polyps on the intestinal mucosa, particu-larly in the colon, with high potential for malignant transformation(1).

Gardner’s syndrome was originally de-scribed as a triad comprising: 1) adenoma-tous polyposis of the colon; 2) skull and

mandible osteomas; 3) epidermoid cysts. However, from the original description by Garder in 1953, the triad has been ex-panded to include other soft tissue abnor-malities such as desmoid tumors, seba-ceous cysts, lipomas, fibromas and sarco-mas(2). The incidence of this syndrome is estimated to be 1:14.000 births(3).

Osteomas are benign, slow-growing bone tumors, corresponding to the most common benign neoplasms of the nose and facial sinuses. Generally, they are asymp-tomatic and incidentally found at imaging studies, although they may run their course with symptoms, depending on their size and location. Typically, the growth of these bone tumors occurs towards inside the air space of a facial sinus or simply exophyt-ically in relation to the bone surface. In Gardner’s syndrome, osteomas presenta-tion is varied, generally preceding the on-set of colon polyps(4,5).

The genetic basis of this syndrome is re-lated to a mutation in the gene of colon adenomatous polyposis located in the long arm of the chromosome 5. The loss of the functions of this gene, which is a tumor suppressor, is considered to be the initial event in the development of adenomas(6,7). Differential diagnoses include pure fa-milial adenomatous polyposis, Turcot

syn-drome (an association of colon adenomas and encephalic tumors such as glioblas-toma multiforme and medulloblasglioblas-toma), Peutz-Jegher syndrome (consisting in mul-tiple hamartomatous polyps) and hereditary nonpolyposis colorectal cancer(6).

The management of these patients in-cludes total colectomy, since the malignant transformation of adenomas occurs in all of the cases. Familial screening is indi-cated(1–3).

REFERENCES

1. Juhn E, Khachemoune A. Gardner syndrome: skin manifestations, differential diagnosis and manage-ment. Am J Clin Dermatol. 2010;11:117–22. 2. Brodbeck AJ, Cruz JV, Camargo Filho SA, et al.

Síndrome de Gardner: apresentação de um caso e revisão da literatura. Rev Bras Colo-Proct. 1983; 3:95–7.

3. Costa JHG, Azevedo IF, Moreira H, et al. Síndrome de Gardner – descrição de um caso raro. Rev Bras Colo-Proct. 1986;6:131–5.

4. Alexander AAZ, Patel AA, Odland R. Paranasal sinus osteomas and Gardner’s syndrome. Ann Otol Rhinol Laryngol. 2007;116:658–62.

5. Wijn MA, Keller JJ, Giardiello FM, et al. Oral and maxillofacial manifestations of familial adenoma-tous polyposis. Oral Dis. 2007;13:360–5. 6. Half E, Bercovich D, Rozen P. Familial adenomatous

polyposis. Orphanet J Rare Dis. 2009;4:22. 7. Gómez-García EB, Knoers NVAM. Gardner’s

Imagem

Figure 2. Non-contrast-enhanced CT, bone window.

Referências

Documentos relacionados

Os resultados da pesquisa demostram que a Madeireira Larssen obteve grandes melhorias nos seus processos de gestão de pessoas após a implementação do Guia de Excelência

No capítulo 2, são apresentadas as prescrições teóricas para cálculo da força axial de compressão resistente de colunas sem furos para o modo de flambagem distorcional, presentes

1 Docente de Pós-graduação, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP; 2 Médico Assisten- te, Hospital das Clínicas da Faculdade

3 Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Hospital das Clínicas, Centro de Ciências das Imagens. e Física Médica, Ribeirão Preto

2 Universidade de São Paulo; Faculdade de Medicina de Ribeirão Preto; Hospital das Clínicas, Radiologia e Diagnóstico por Imagem, Ribeirão Preto SP, Brasil.. Correspondence:

Hospital das Clínicas – Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (HCFMRP-USP), and Cedirp – Radiologia e Diagnóstico por Imagem, Ribeirão Preto,

Tenured Professor at the Faculdade de Medicina de Ribeirão Preto (University of São Paulo School of Medicine in Ribeirão Preto) - USP - Ribeirão Preto (SP) Brazil2. The fact

Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo – FMRP/USP, University of São Paulo at Ribeirão Preto School of Medicine – Ribeirão Preto,