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肢端肥大症的影像学诊断

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肢端肥大症的影像学诊断 Enyi Nwaneri, HMS III Gillian Lieberman, MD Radiologic Features of Acromegaly Enyi Nwaneri Harvard Medical School Year III Gillian Lieberman, MD March 2003 Our Patient • ID/CC A 29 year old Asian male visits his doctor because he notices that his hands ar...
肢端肥大症的影像学诊断
Enyi Nwaneri, HMS III Gillian Lieberman, MD Radiologic Features of Acromegaly Enyi Nwaneri Harvard Medical School Year III Gillian Lieberman, MD March 2003 Our Patient • ID/CC A 29 year old Asian male visits his doctor because he notices that his hands are getting bigger • HPI No major complaints except enlarging of hands, nose, jaw, and feet over the past 2-3 years • PE Significant enlargement of hands and feet, broad nose, enlargement of facial tissues, frontal bossing • LABS Growth Hormone: 18 ng/ml (nl 0-10 ng/ml) IGF-I: 738 ng/ml (nl 114-492 ng/ml) Enyi Nwaneri, HMS III Gillian Lieberman, MD 2 Diagnosis ACROMEGALY Enyi Nwaneri, HMS III Gillian Lieberman, MD 3 ACROMEGALY • Clinical syndrome that results from excessive secretion of growth hormone (GH) after fusion of the epiphyseal growth plates • Annual incidence is 3-4 per million people • Incidence is equal in men and women • Mean age at diagnosis is 40 to 45 years • Most common cause is GH secreting adenoma of the anterior pituitary Enyi Nwaneri, HMS III Gillian Lieberman, MD 4 Clinical Manifestations • Somatic effects due to excess GH and IGF-I which cause stimulation of growth of skin, connective tissue, cartilage, bone, viscera, and epithelial tissue • Onset is insidious with slow progression Enyi Nwaneri, HMS III Gillian Lieberman, MD From Motlitch ME. Clinical manifestations of acromegaly. Endocrinol Metab Clin North Am 1992; 21(3): p 599. 5 Clinical Manifestations • Acral and soft tissue overgrowth • Articular overgrowth • Enlarged viscera • Cardiovascular disease • Tumors • Hypogonadism Enyi Nwaneri, HMS III Gillian Lieberman, MD From http://www.library.uthscsa.edu/ms2/Pathology/Endocrine/ Labs/Lab%202/Lab%202.doc From Melmed S. Clinical Manifestations of Acromegaly. UpToDate 2003. 6 Anatomy and Relations of the Pituitary Gland Enyi Nwaneri, HMS III Gillian Lieberman, MD From Netter F. The Ciba Collection, Vol. 4. R.R. Donnelley & Sons Company. New York, 1965, p 6. 7 Relationship of the Pituitary Gland to the Cavernous Sinus Enyi Nwaneri, HMS III Gillian Lieberman, MD From Netter F. The Ciba Collection, Vol. 4. R.R. Donnelley & Sons Company. New York, 1965, p 7. 8 Relationship of the Pituitary Gland to the Sella Turcica Enyi Nwaneri, HMS III Gillian Lieberman, MD From http://www.mythos.com/webmd/Content.aspx? P=ENDOCC&E=6 Sella turcica Pituitary gland Optic chiasm Mamillary body 9 Relationship of the Pituitary Adenoma to the Sella Turcica Enyi Nwaneri, HMS III Gillian Lieberman, MD From Netter F. The Ciba Collection, Vol 1(2). CIBA Pharmaceutical Company. West Caldwell, NJ, 1986, p 122. 10 Imaging Modalities • Plain Radiographs • MRI • Somatostatin Receptor Scintigraphy Enyi Nwaneri, HMS III Gillian Lieberman, MD 11 Plain Radiographs • No longer routinely used for diagnosis of acromegaly • Good for demonstrating wide variety of radiologic changes of the skeleton in patients with acromegaly Enyi Nwaneri, HMS III Gillian Lieberman, MD 12 Plain Radiograph Features of Skeletal Involvement in Acromegaly Enyi Nwaneri, HMS III Gillian Lieberman, MD 13 Companion Patient A • 48 year old Italian woman presents to Mt. Auburn Hospital in 1956 with chief complaint of headache, weight gain, swollen hands, and changes in facial features • Skull radiographs reveal an enlarged sella turcicaÆ diagnosis of acromegaly made • Refuses treatment for 15 years • Finally presents to BI Hospital in 1971 for further work-up and treatment of her acromegaly • On exam noted to have thick, coarse skin, enlarged lips and ears, separated teeth, macroglossia, marked prognathism, frontal bossing, and enlarged hands and feet Enyi Nwaneri, HMS III Gillian Lieberman, MD 14 Changes in Skin Thickness Enyi Nwaneri, HMS III Gillian Lieberman, MD Courtesy of Ferris Hall, MD Prominence of soft tissues of the heel measuring 40 mm Pathophysiologic correlation: Excessive growth hormone Æ marked response by collagen tissue Æ connective tissue hyperplasia Æ increased thickness of skin (e.g. heel pad) Companion Patient AFindings: 15 Diagnostic Significance of Thickened Heel Pad Enyi Nwaneri, HMS III Gillian Lieberman, MD From Resnick D. Bone and Joint Disorders Vol. 3, 4th ed. W.B. Saunders Company. Philadelphia, 2002, p 2010. Measure shortest distance between calcaneus and plantar skin surface Values greater than 23 mm in men and 21.5 mm in women are suggestive of acromegaly Variation with body weight, race somewhat limits usefulness Æ development of more sophisticated calculations Enthesal proliferation of bone at insertion of Achilles tendon and plantar aponeurosis 16 Differential Diagnosis of Thickened Heel Pad 1) Acromegaly 2) Generalized edema (e.g. CHF, DVT, lymphedema) 3) Infection of soft tissues 4) Normal variant; genetic (especially Black and Polynesian males) 5) Obesity 6) Trauma 7) Long-term phenytoin therapy Enyi Nwaneri, HMS III Gillian Lieberman, MD 17 Abnormalities of the Skull Enyi Nwaneri, HMS III Gillian Lieberman, MD Courtesy of Ferris Hall, MD Companion Patient AFindings: Thickened cranial vault Enlarged sella turcica Prominence of the occipital protuberance Elongation and projection of mandible Enlarged frontal sinuses 18 Abnormalities of the Skull Enyi Nwaneri, HMS III Gillian Lieberman, MD From Chew F. Radiologic manifestations in the musculoskeletal system of miscellaneous endocrine disorders. Radiologic Clinics of North America 1991; 29(1): p 139. From Resnick D. Bone and Joint Disorders Vol. 3, 4th ed. W.B. Saunders Company. Philadelphia, 2002, p 2010. Findings: Increased prominence of mandible Increased thickness of clavaria Enlarged frontal sinus Enlarged supraorbital ridge 19 Abnormalities of the Skull Enyi Nwaneri, HMS III Gillian Lieberman, MD Pathophysiologic correlation: Presence of pituitary neoplasm Æ sella turcica abnormalities Excess growth hormone Æ stimulation of periosteal bone formation Æ mandibular enlargement, thickening of cranial vault, prominence of supraorbital ridges and facial structures 20 Abnormalities of the Hand and Wrist Enyi Nwaneri, HMS III Gillian Lieberman, MD Courtesy of Ferris Hall, MD Companion Patient AFindings: Widening of MCP joint spaces Soft tissue thickening Carpal cysts Pathophysiologic correlation: Excessive growth hormone Æ increased chondrocytic activity Æ proliferation of articular cartilage (cartilage hypertrophy) Æ widening of articular space Carpal cysts result suggest bone degeneration Broadened phalangeal tufts 21 Abnormalities of the Hand and Wrist Enyi Nwaneri, HMS III Gillian Lieberman, MD From Resnick D. Bone and Joint Disorders Vol. 3, 4th ed. W.B. Saunders Company. Philadelphia, 2002, p 2012. Findings: Enlargement of terminal phalangeal tuft (“spade- like”) Enlargement of terminal phalangeal base Soft tissue thickening Formation of pseudoforamina Pathophysiologic correlation: Stimulation of periosteal bone formation Æ enlargement of phalanges, phalangeal tufts especially 22 Abnormalities of the Foot Enyi Nwaneri, HMS III Gillian Lieberman, MD Findings: Soft tissue enlargement, widening of the MTP joints, prominence of terminal phalangeal tufts and bases, pseudoforamina From Resnick D. Bone and Joint Disorders Vol. 3, 4th ed. W.B. Saunders Company. Philadelphia, 2002, p 2015. 23 Differential Diagnosis of Phalangeal Tuft Enlargement 1) Normal variant Must take into account patient’s gender and occupation 2) Acromegaly Enyi Nwaneri, HMS III Gillian Lieberman, MD 24 Abnormalities of the Vertebral Column Enyi Nwaneri, HMS III Gillian Lieberman, MD Findings: Increase in thoracic vertebral body AP diameter due to bone formation on anterior aspect of vertebrae Findings: Increased concavity on posterior aspect of lumbar vertebral bodies (scalloped vertebrae)Æ related to excessive resorption of bone From Resnick D. Bone and Joint Disorders Vol. 3, 4th ed. W.B. Saunders Company. Philadelphia, 2002, p 2016. From Resnick D. Bone and Joint Disorders Vol. 3, 4th ed. W.B. Saunders Company. Philadelphia, 2002, p 2016. Companion Patient A Findings: “…suggestion of new bone formation involving the anterior aspects of the vertebral bodies.” 25 Companion Patient A Noted on plain radiographs to have degenerative disease in joints of shoulders, fingers, wrists, knees 26 Articular Abnormalities • Radiologic joint abnormalities seen most frequently in knee, glenohumeral joint, and hip • Two main categories: cartilage hypertrophy and cartilaginous and bony degeneration • Radiologic features of acromeglic degenerative changes (joint space narrowing, cyst formation, osteophyte formation) can be confused with primary degenerative joint disease (osteoarthritis) Enyi Nwaneri, HMS III Gillian Lieberman, MD 27 Acromegalic Arthropathy or Osteoarthritis? Enyi Nwaneri, HMS III Gillian Lieberman, MD • If joint space is wide Æ Acromegaly • If joint space is narrow Æ Differential diagnosis rests on distribution of arthropathy Acromegaly involves shoulders, elbows, and MCP joints, which are unusual sites for primary osteoarthritis 28 Additional Plain Radiograph Features of Skeletal Involvement • Abnormalities of the Thoracic Cage May appear enlarged due to elongation and enlargement of the anterior ribs (particularly first ribs) and medial clavicles Prominence of costochondral junction (acromegalic rosary) due to GH mediated reactivation of endochondral ossification • Abnormalities of the Pelvis Enlargement and “beaking” of pubis symphysis Bony proliferation at ligament attachment sites (enthesopathy) • Abnormalities of the Long Bones Narrowing of diaphysis due to predominant bone resorption Enyi Nwaneri, HMS III Gillian Lieberman, MD 29 Imaging Modalities • Plain Radiographs • MRI • Somatostatin Receptor Scintigraphy Enyi Nwaneri, HMS III Gillian Lieberman, MD 30 MRI • Method of choice in detecting pituitary adenomas • Can detect tumors as small as 2 mm in diameter • Can accurately identify dimensions and anatomic extent of tumor • Compared to CT Superior soft tissue contrast More sensitive in defining the extrasellar extent of the adenomas and in detecting cavernous sinus invasion Enyi Nwaneri, HMS III Gillian Lieberman, MD 31 Any Role for CT? • Postcontrast CT scans previously used for imaging of adenoma and assessment of bone erosions or cavernous sinus invasion • Unable to detect small adenomas due to poor contrast resolution and similar enhancing characteristics between adenomas and normal pituitary gland • Advantage over MRI Æ superior in demonstrating cortical bone Æ critical in cases of pituitary adenomas causing deformity or erosion of the sellar floor • CT scans may be more useful than MRI in selected patients in whom the bony anatomy of the floor of the sella turcica needs to be visualized Enyi Nwaneri, HMS III Gillian Lieberman, MD 32 Our Patient Enyi Nwaneri, HMS III Gillian Lieberman, MD Findings: 5 mm x 11 mm x 10 mm low signal, non-enhancing lesion in left side of pituitary consistent with adenoma From BIDMC PACS From BIDMC PACS 33 Imaging Modalities • Plain Radiographs • MRI • Somatostatin Receptor Scintigraphy Enyi Nwaneri, HMS III Gillian Lieberman, MD 34 Somatostatin Receptor Scintigraphy • Based on underlying principle that GH adenomas express somatostatin receptors • Radiolabeled octreotide is used as the detector allowing somatostatin receptor positive tissues to be visualized • Non-specific since somatostatin receptors are also widely expressed by normal endocrine tissue Æ high incidence of false positives • Physiologic distribution of octreotide includes the digestive tract Æ masking of abdominal tumors by normal octreotide uptake in this region • Implications for medical therapy Æ increased uptake on octreotide scan predicts success of treatment with somatostatin analogs Enyi Nwaneri, HMS III Gillian Lieberman, MD 35 Companion Patient B • 43 year old woman presents with clinical findings of acromegaly • Pituitary MRI was negative • Underwent octreotide scan for further work-up of possible pituitary adenoma • Visualization done after 4 and 24 hours Enyi Nwaneri, HMS III Gillian Lieberman, MD 36 Companion Patient B Enyi Nwaneri, HMS III Gillian Lieberman, MD Initial 4 hour whole body image Findings: Normal activity involving the kidneys, liver, and spleen. Normal accumulation in bladder. No evidence of focal abnormal increased tracer activity. Courtesy of Kevin Donohoe, MD 37 Companion Patient B Enyi Nwaneri, HMS III Gillian Lieberman, MD 24 hour whole body image Findings: Normal uptake visualized in thyroid, kidneys, liver spleen, and non-specific GI uptake Focal increased uptake at the region of the skull base Courtesy of Kevin Donohoe, MD 38 Companion Patient B Enyi Nwaneri, HMS III Gillian Lieberman, MD Courtesy of Kevin Donohoe, MD Left lateral view of head Findings: Normal uptake visualized in thyroid Localized uptake at base of skull corresponding to location of pituitary fossa Impression: Abnormal uptake of octreotide in region of the pituitary fossa, suggestive of adenoma 39 Our Patient • On 12/4/02 underwent selective transsphenoidal surgical resection of pituitary microadenoma • Pathology of showed strong staining for growth hormone • One day post-op GH level reduced to 2 ng/ml • One month post-op reports improvement in hand swelling • As of 1/29/03 GH levels reduced to less than 1 ng/ml and IGF-I level was 250 Enyi Nwaneri, HMS III Gillian Lieberman, MD 40 References • Chew F. Radiologic manifestations in the musculoskeletal system of miscellaneous endocrine disorders. Radiologic Clinics of North America 1991; 29(1):135-147. • Goldman AB. Some miscellaneous joint diseases. Seminars in Roentgenology 1982; 17(1):60-80. • Hordon LD. Rheumatic and bone disorders associated with acromegaly. UpToDate 2003. www.uptodateonline.com • Lieberman S, Bjorkengren A, Hoffman A. Rheumatological and skeletal changes in acromegaly. Endocrinol Metab Clin North Am 1992; 21(3):615-630. • Moran S, Patsalides A, Patronas N. A case of a hidden pituitary adenoma causing acromegaly. EndoTrends 2002; 9(1) 1,9-13. http://www.endocrinefellows.org/htmldocs/endotrends/v9i1.pdf • Melmed S. Clinical manifestations of acromegaly. UpToDate 2003. www.uptodateonline.com • Melmed S. Diagnosis of acromegaly. UpToDate 2003. www.uptodateonline.com • Melmed S. Treatment of acromegaly. UpToDate 2003. www.uptodateonline.com • Motlitch ME. Clinical manifestations of acromegaly. Endocrinol Metab Clin North Am 1992; 21(3):597. Enyi Nwaneri, HMS III Gillian Lieberman, MD 41 References Cont. • Netter F. The Ciba Collection, Vol. 4. R.R. Donnelley & Sons Company. New York, 1965. • Netter F. The Ciba Collection, Vol 1(2). CIBA Pharmaceutical Company. West Caldwell, NJ, 1986, p 122. • Resnick D. Bone and Joint Disorders, Vol. 3, 4th ed. W.B. Saunders Company. Philadelphia, 2002. • Snyder P. Causes, presentation, and evaluation of sellar masses. UpToDate 2003. www.uptodateonline.com • Staple TW, Melson GL, Evens RG. Miscellaneous soft tissue lesions of the extremities. Seminars in Roentgenology 1973; 8(1):117-127. • Subbarao K, Jacobson HG. Systemic disorders affecting the thoracic cage. Radiologic Clinics of North America 1984; 22(3):497-517. • http://www.library.uthscsa.edu/ms2/Pathology/Endocrine/Labs. • http://www.mythos.com/webmd/Content.aspx?P=ENDOCC&E=6 Enyi Nwaneri, HMS III Gillian Lieberman, MD 42 Special Thanks Ferris Hall, MD Kevin Donohoe, MD Eric Niendorf, MD Daniel Saurborn, MD Robert Silver, MD Gillian Lieberman, MD Pamela Lepkowski Larry Barbaras and Cara Lyn D’amour Enyi Nwaneri, HMS III Gillian Lieberman, MD 43 Radiologic Features of Acromegaly Our Patient Diagnosis ACROMEGALY Clinical Manifestations Clinical Manifestations Anatomy and Relations of the Pituitary Gland Relationship of the Pituitary Gland to the Cavernous Sinus Relationship of the Pituitary Gland to the Sella Turcica Relationship of the Pituitary Adenoma to the Sella Turcica Imaging Modalities Plain Radiographs Plain Radiograph Features of Skeletal Involvement�in Acromegaly Companion Patient A Changes in Skin Thickness� Diagnostic Significance of Thickened Heel Pad Differential Diagnosis of Thickened Heel Pad Abnormalities of the Skull Abnormalities of the Skull Abnormalities of the Skull Abnormalities of the Hand and Wrist Abnormalities of the Hand and Wrist Abnormalities of the Foot Differential Diagnosis of Phalangeal Tuft Enlargement Abnormalities of the Vertebral Column Companion Patient A Articular Abnormalities Acromegalic Arthropathy or Osteoarthritis? Additional Plain Radiograph Features of Skeletal Involvement Imaging Modalities MRI Any Role for CT? Our Patient Imaging Modalities Somatostatin Receptor Scintigraphy Companion Patient B Companion Patient B Companion Patient B Companion Patient B Our Patient References References Cont. Special Thanks
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