Localized amyloidosis is characterized by amyloid protein deposition restricted to one organ or tissue without systemic involvement. Gastrointestinal manifestations of localized amyloidoma are unusual, which makes amy...Localized amyloidosis is characterized by amyloid protein deposition restricted to one organ or tissue without systemic involvement. Gastrointestinal manifestations of localized amyloidoma are unusual, which makes amyloidoma restricted to the rectum a very rare diagnosis requiring a high index of suspicion. We present a rare account for rectal amyloidoma with an unusual presentation of obstructive symptoms and its treatment using a sophisticated surgical modality, transanal endoscopic microsurgery(TEM), which resulted in complete excision of the lesion without hospitalization and complications. The successful treatment for thisrectal amyloidoma using TEM emphasizes the need to broaden its application in the treatment of various rectal lesions while preserving organ function and decreasing recurrence.展开更多
Amyloidosis is commonly systemic,occasionally organlimited,and rarely a solitary localized mass.The latter,commonly referred to as tumoral amyloidosis,is described as occurring in nearly every organ/tissue.Only a few ...Amyloidosis is commonly systemic,occasionally organlimited,and rarely a solitary localized mass.The latter,commonly referred to as tumoral amyloidosis,is described as occurring in nearly every organ/tissue.Only a few reports of gastric amyloidosis exist today.We describe a 72 year-old black male from Barbados presenting with 3 d of diffuse abdominal pain.His medical history included Non-Hodgkin's Lymphoma diagnosed five years ago,status-post six rounds of cyclophosphamide,adriamycin,vincristine,prednisone chemotherapy,and currently was in remission.On computed tomography scan of the abdomen,thickening and calcification of the gastric wall was noted along with pneumatosis.On esophagogastroduodenoscopy,a large circumferential friable mass was seen from the gastroesophageal junction to the body.A large nonbleeding 3 cm polyp was also seen in post bulbar area of duodenum.Biopsies were stained with Congo red and gave green birefringence under polarized light,consistent with tumoral amyloidosis.Positron emission tomography scan revealed diffuse gastric mucosa uptake compatible with gastric malignancy without metastatic foci.Treatment for gastric amyloidomas has presently been one of observation or,at most,resection of the amyloid mass.It is not known if our patient required the same approach or if this warranted the re-institutionof chemotherapy for Non-Hodgkin's Lymphoma.Until more reports of tumoral amyloidosis are made known,treatment as well as prognosis remain uncertain.展开更多
A tumor-like presentation of systemic amyloidosis in the nasopharynx is exceedingly rare. A male of 52-year-old with a recurrent mass in the nasopharynx underwent surgical resection 2 years ago. Immunohistochemical ex...A tumor-like presentation of systemic amyloidosis in the nasopharynx is exceedingly rare. A male of 52-year-old with a recurrent mass in the nasopharynx underwent surgical resection 2 years ago. Immunohistochemical examination revealed M protein band and kappa immunoglobulin light chain. The sections of the biopsy of the mass in the nasopharynx and anterior abdominal wall were stained with Congo red and examined with microscopy. Amyloid deposits were found. It was diagnosed as systemic amyloidoma. Colchicine, melphalan and prednisone were administered. The mass decreased its size in the 8 months of follow-up. This case suggests that a solitary tumor in the nasopharynx might be an early sign of systemic amyloidosis because of the slow progression of the disease. The existence of a solitary amyloidoma in any location of the body should lead the doctor to look for other symptoms and signs of systemic amyloidosis.展开更多
<strong>Introduction: </strong>Primary deposition of amyloid can take several forms including amyloidoma organ restricted among which is brain. Brain amyloidoma can mimic several lesions and offers a diagn...<strong>Introduction: </strong>Primary deposition of amyloid can take several forms including amyloidoma organ restricted among which is brain. Brain amyloidoma can mimic several lesions and offers a diagnostic challenge to the physician. The present work reports an additional case and summarizes the salient characteristics of previously published cases. <strong>Case Report: </strong>A 61-year-old, woman experienced paroxystic auditory hallucinations followed at times by generalized tonico-clonic seizures. Examination revealed a mild left pyramidal weakness. Head CT scan and MRI revealed a lobular lesion of the right atrium with contrast enhancement. The diagnosis of amyloidoma Lambda light chain (λLC) type was performed by means of a stereotactic biopsy. The patient was treated by 3 cures of high dose methotrexate (15 mg/m<sup>2</sup>). At 24 months follow up the patient remains stable clinically and biologically. The 2 years follow-up MRI showed the same findings. <strong>Conclusion: </strong>No guidelines can be suggested for brain amyloidoma treatment;the “wait and see” was the mind-set in the literature. However, a non-tumour like pattern of a cerebral mass developing near the ventricle with a fine, irregular radiating margin enhancement and lack of oedema, and mass effect are important MRI clues for brain amyloidoma diagnosis. The diagnosis is histological after biopsy or excision.展开更多
文摘Localized amyloidosis is characterized by amyloid protein deposition restricted to one organ or tissue without systemic involvement. Gastrointestinal manifestations of localized amyloidoma are unusual, which makes amyloidoma restricted to the rectum a very rare diagnosis requiring a high index of suspicion. We present a rare account for rectal amyloidoma with an unusual presentation of obstructive symptoms and its treatment using a sophisticated surgical modality, transanal endoscopic microsurgery(TEM), which resulted in complete excision of the lesion without hospitalization and complications. The successful treatment for thisrectal amyloidoma using TEM emphasizes the need to broaden its application in the treatment of various rectal lesions while preserving organ function and decreasing recurrence.
文摘Amyloidosis is commonly systemic,occasionally organlimited,and rarely a solitary localized mass.The latter,commonly referred to as tumoral amyloidosis,is described as occurring in nearly every organ/tissue.Only a few reports of gastric amyloidosis exist today.We describe a 72 year-old black male from Barbados presenting with 3 d of diffuse abdominal pain.His medical history included Non-Hodgkin's Lymphoma diagnosed five years ago,status-post six rounds of cyclophosphamide,adriamycin,vincristine,prednisone chemotherapy,and currently was in remission.On computed tomography scan of the abdomen,thickening and calcification of the gastric wall was noted along with pneumatosis.On esophagogastroduodenoscopy,a large circumferential friable mass was seen from the gastroesophageal junction to the body.A large nonbleeding 3 cm polyp was also seen in post bulbar area of duodenum.Biopsies were stained with Congo red and gave green birefringence under polarized light,consistent with tumoral amyloidosis.Positron emission tomography scan revealed diffuse gastric mucosa uptake compatible with gastric malignancy without metastatic foci.Treatment for gastric amyloidomas has presently been one of observation or,at most,resection of the amyloid mass.It is not known if our patient required the same approach or if this warranted the re-institutionof chemotherapy for Non-Hodgkin's Lymphoma.Until more reports of tumoral amyloidosis are made known,treatment as well as prognosis remain uncertain.
文摘A tumor-like presentation of systemic amyloidosis in the nasopharynx is exceedingly rare. A male of 52-year-old with a recurrent mass in the nasopharynx underwent surgical resection 2 years ago. Immunohistochemical examination revealed M protein band and kappa immunoglobulin light chain. The sections of the biopsy of the mass in the nasopharynx and anterior abdominal wall were stained with Congo red and examined with microscopy. Amyloid deposits were found. It was diagnosed as systemic amyloidoma. Colchicine, melphalan and prednisone were administered. The mass decreased its size in the 8 months of follow-up. This case suggests that a solitary tumor in the nasopharynx might be an early sign of systemic amyloidosis because of the slow progression of the disease. The existence of a solitary amyloidoma in any location of the body should lead the doctor to look for other symptoms and signs of systemic amyloidosis.
文摘<strong>Introduction: </strong>Primary deposition of amyloid can take several forms including amyloidoma organ restricted among which is brain. Brain amyloidoma can mimic several lesions and offers a diagnostic challenge to the physician. The present work reports an additional case and summarizes the salient characteristics of previously published cases. <strong>Case Report: </strong>A 61-year-old, woman experienced paroxystic auditory hallucinations followed at times by generalized tonico-clonic seizures. Examination revealed a mild left pyramidal weakness. Head CT scan and MRI revealed a lobular lesion of the right atrium with contrast enhancement. The diagnosis of amyloidoma Lambda light chain (λLC) type was performed by means of a stereotactic biopsy. The patient was treated by 3 cures of high dose methotrexate (15 mg/m<sup>2</sup>). At 24 months follow up the patient remains stable clinically and biologically. The 2 years follow-up MRI showed the same findings. <strong>Conclusion: </strong>No guidelines can be suggested for brain amyloidoma treatment;the “wait and see” was the mind-set in the literature. However, a non-tumour like pattern of a cerebral mass developing near the ventricle with a fine, irregular radiating margin enhancement and lack of oedema, and mass effect are important MRI clues for brain amyloidoma diagnosis. The diagnosis is histological after biopsy or excision.
