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Unilateral retroperitoneal fibrosis presenting as an abdominal mass
AJG – September, 2000
Because of the elevated hepatic iron stores, the HFE gene test was performed utilizing tissue from the donor liver. The HFE gene test was also performed on the recipient utilizing whole blood. Results: The donor liver biopsy at the time of transplantation showed moderate to marked hemosiderosis in hepatocytes, with a hepatic iron index of 2.9, compatible with pre-cirrhotic HHC. The recipient’s postoperative course was remarkable for an episode of rejection, which responded to standard treatment with steroids. He also developed persistently abnormal serum aminotransferases and rapidly progressive recurrent hepatitis C. His serum transferrin saturation, ferritin and hepatic iron stores remained persistently elevated. Two years post-OLT his serum ferritin is 1885 mg/L, and he has bridging fibrosis with a hepatic iron index of 2.7. HFE testing confirmed that the donor was homozygous for the C282Y mutation but negative for H63D mutation, and the recipient did not have the C282Y or H63D mutations. The patient is currently undergoing weekly phlebotomy, which he is tolerating very well, with gradual improvement in his liver enzymes. Conclusions: 1) We describe a case of the inadvertent transplantation of a liver from a donor with HHC into a recipient with ESLD secondary to hepatitis C. The recipient has developed recurrent histologic hepatitis C and persistent iron overload. 2) Iron overload in our patient may be contributing to progressive liver damage. 3) Phlebotomy may be helpful prior to starting other treatment for recurrent hepatitis C.
499 A new cause for Zollinger-Ellison Syndrome: Non-small cell lung cancer Saif Alaa Abou, Shojamanesh Homayoun, Gibril Fathia, Jensen* Robert T. National Institutes of Health, Bethesda, MD, United States. Purpose: We report the first case of Zollinger-Ellison syndrome (ZES) caused by a cancer of the lung. Methods: A 60 yo male presented with severe diarrhea associated with nausea, vomiting, abdominal pain and weight loss for 2 months. On EGD he had multiple post-bulbar duodenal ulcers. His symptoms were totally controlled with lansoprazole 30 mg bid. Upon further evaluation in our institution he was found to have hypergastrinemia with hyperchlorhydria. His secretin stimulation test was negative so were his tumor localization studies. However, a CT scan showed a 4-cm left lower lobe (LLL) mass with mediastinal adenopathy. Fine needle aspiration of the lung mass revealed atypical cells that stained positive for chromogranin A (Cg A) and synaptophysin suggesting neuroendocrine tumor. Apart from an elevated serum gastrin and serum Cg A all other hormonal assays were normal including serum gastrin-releasing peptide (GRP). Results: The patient underwent a thoracotomy with lobectomy of the LLL and resection of 21 lymph nodes (LN) of which 18 had tumor invasion. The histologic diagnosis was metastatic neuroendocrine carcinoma of the lung (large cell type). Immunohistochemical studies showed tumor cells to stain positive for gastrin, synaptophysin and CgA. Fasting serum gastrin level decreased immediately post-operatively from 1000 to 100 pg/ml (normal ⬍200 pg/ml). Also serum CgA level decrease postoperatively to normal range. The patient subsequently received adjuvant radiation treatment. Conclusions: Until recently all gastrinomas producing Zollinger-Ellison syndrome were intra-abdominal. Recently two cardiac gastrinomas were described and this case is the second extra-abdominal location of a tumor causing ZES. Many lung cancer types are known to produce peptides with hormonal activity including GRP, vasopressin and occasionally gastrin. This led some authors to suggest using these peptides as a tumor marker and as a tool to follow-up treatment response after surgery. Large cell neuroendocrine lung cancer is a member of the neuroendocrine lung cancer family that also include carcinoid (typical and atypical) and small cell lung cancer. In some cohort studies, there is an association of peptic ulcer disease with lung cancer, smoking was thought to be the common predisposing factor. However, gastric acid hypersecretion and peptic ulcer disease as a result of a peptide produced by lung cancer has not been reported.
Abstracts
2557
This case raises the possibility. This phenomenon may be more common than previously reported.
500 Unilateral retroperitoneal fibrosis presenting as an abdominal mass Aboyoussef MD M, Bahl MD V, Stept MD L, Evans MD S. UPMC Shadyside, Pittsburgh, PA. A 47-year-old black male previously in good health presented with three weeks of left flank pain associated with constipation. His past medical history is significant for diabetes mellitus type 2 for five years. His only medication is metformin. He has never had any abdominal surgeries. Preliminary work up revealed increase in creatinine to 1.6. Later, a CAT scan of the abdomen and pelvis revealed left hydroureter and hydronephrosis, with soft tissue fullness, 4 cm in the largest diameter, surrounding the left ureter. A stent was placed in the left ureter, which led to the relief of his pain. A laparotomy was performed, with lysis of adhesions around the ureter. Final pathology of an abdominal lymph node and tissue from the mass revealed retroperitoneal fibrosis. After ten days of hospital admission, his creatinine decreased to 0.9 and his bowel habits returned to normal. He was discharged with outpatient follow-up. Six months later he remains asymptomatic and follow-up CT scan shows no evidence of recurrence. Discussion: An interesting case of unilateral retroperitoneal fibrosis presenting as a retroperitoneal mass responding well to surgical lysis of adhesions and no pharmacotherapy.
501 A case of PNH and intestinal ischemia Tonya L. Adams, MD, David E. Fleischer, MD, Gustavo Marino, MD, Eileen Rusnock, MD, Lee Li, MD. Georgetown University Medical Center, Washington, D.C., USA. Thrombotic complications, particularly microthrombi involving intra-abdominal veins leading to intestinal ischemia, have remained a major cause of morbidity in patients with paroxysmal nocturnal hemoglobinuria (PNH). While intestinal ischemia has been postulated to be the cause of recurrent bouts of abdominal pain in this population, direct antemortem evidence for this complication is scarcely documented in the literature. We describe a case of PNH in a patient who presented with abdominal distress 3 years after the initial diagnosis was established. Clinical features and a combination of diagnostic modalities, including radiography, serial endoscopies and histology were used to make the prompt diagnosis of intestinal ischemia. This is the first case in which the electronic microscopy of the gastrointestinal lesion is described. Our patient was successfully treated with conservative measures and anticoagulation.
502 Intraductal papillary mucin tumor (IPMT): Clinical characteristics in a cohort of young patients Adler MD Douglas G, Chari MD Suresh T, Dahl RN Tamela, Pearson MD FACG Randall K. Mayo Clinic, Rochester, MN. Introduction: IPMT is a neoplastic disorder of the pancreatic ducts characterized by an ectatic ductal system due to a mucin secreting, papillary mucosa. Typically, patients present late in life with symptoms compatible with chronic pancreatitis or malignancy. The diagnosis is often delayed due to confusion of early clinical symptoms with other disorders. Invasive carcinoma is often present at diagnosis and benign IPMT is considered pre-malignant. We report the clinical characteristics of 5 patients under the age of 50 with histologically proven IPMT. Methods: Patients with IPMT were identified through the Mayo Clinic Pancreas Clinic and Surgical Pathology Database from 1982 to the present. Information abstracted from charts of consenting patients is reported.
Because of the elevated hepatic iron stores, the HFE gene test was performed utilizing tissue from the donor liver. The HFE gene test was also performed on the recipient utilizing whole blood. Results: The donor liver biopsy at the time of transplantation showed moderate to marked hemosiderosis in hepatocytes, with a hepatic iron index of 2.9, compatible with pre-cirrhotic HHC. The recipient’s postoperative course was remarkable for an episode of rejection, which responded to standard treatment with steroids. He also developed persistently abnormal serum aminotransferases and rapidly progressive recurrent hepatitis C. His serum transferrin saturation, ferritin and hepatic iron stores remained persistently elevated. Two years post-OLT his serum ferritin is 1885 mg/L, and he has bridging fibrosis with a hepatic iron index of 2.7. HFE testing confirmed that the donor was homozygous for the C282Y mutation but negative for H63D mutation, and the recipient did not have the C282Y or H63D mutations. The patient is currently undergoing weekly phlebotomy, which he is tolerating very well, with gradual improvement in his liver enzymes. Conclusions: 1) We describe a case of the inadvertent transplantation of a liver from a donor with HHC into a recipient with ESLD secondary to hepatitis C. The recipient has developed recurrent histologic hepatitis C and persistent iron overload. 2) Iron overload in our patient may be contributing to progressive liver damage. 3) Phlebotomy may be helpful prior to starting other treatment for recurrent hepatitis C.
499 A new cause for Zollinger-Ellison Syndrome: Non-small cell lung cancer Saif Alaa Abou, Shojamanesh Homayoun, Gibril Fathia, Jensen* Robert T. National Institutes of Health, Bethesda, MD, United States. Purpose: We report the first case of Zollinger-Ellison syndrome (ZES) caused by a cancer of the lung. Methods: A 60 yo male presented with severe diarrhea associated with nausea, vomiting, abdominal pain and weight loss for 2 months. On EGD he had multiple post-bulbar duodenal ulcers. His symptoms were totally controlled with lansoprazole 30 mg bid. Upon further evaluation in our institution he was found to have hypergastrinemia with hyperchlorhydria. His secretin stimulation test was negative so were his tumor localization studies. However, a CT scan showed a 4-cm left lower lobe (LLL) mass with mediastinal adenopathy. Fine needle aspiration of the lung mass revealed atypical cells that stained positive for chromogranin A (Cg A) and synaptophysin suggesting neuroendocrine tumor. Apart from an elevated serum gastrin and serum Cg A all other hormonal assays were normal including serum gastrin-releasing peptide (GRP). Results: The patient underwent a thoracotomy with lobectomy of the LLL and resection of 21 lymph nodes (LN) of which 18 had tumor invasion. The histologic diagnosis was metastatic neuroendocrine carcinoma of the lung (large cell type). Immunohistochemical studies showed tumor cells to stain positive for gastrin, synaptophysin and CgA. Fasting serum gastrin level decreased immediately post-operatively from 1000 to 100 pg/ml (normal ⬍200 pg/ml). Also serum CgA level decrease postoperatively to normal range. The patient subsequently received adjuvant radiation treatment. Conclusions: Until recently all gastrinomas producing Zollinger-Ellison syndrome were intra-abdominal. Recently two cardiac gastrinomas were described and this case is the second extra-abdominal location of a tumor causing ZES. Many lung cancer types are known to produce peptides with hormonal activity including GRP, vasopressin and occasionally gastrin. This led some authors to suggest using these peptides as a tumor marker and as a tool to follow-up treatment response after surgery. Large cell neuroendocrine lung cancer is a member of the neuroendocrine lung cancer family that also include carcinoid (typical and atypical) and small cell lung cancer. In some cohort studies, there is an association of peptic ulcer disease with lung cancer, smoking was thought to be the common predisposing factor. However, gastric acid hypersecretion and peptic ulcer disease as a result of a peptide produced by lung cancer has not been reported.
Abstracts
2557
This case raises the possibility. This phenomenon may be more common than previously reported.
500 Unilateral retroperitoneal fibrosis presenting as an abdominal mass Aboyoussef MD M, Bahl MD V, Stept MD L, Evans MD S. UPMC Shadyside, Pittsburgh, PA. A 47-year-old black male previously in good health presented with three weeks of left flank pain associated with constipation. His past medical history is significant for diabetes mellitus type 2 for five years. His only medication is metformin. He has never had any abdominal surgeries. Preliminary work up revealed increase in creatinine to 1.6. Later, a CAT scan of the abdomen and pelvis revealed left hydroureter and hydronephrosis, with soft tissue fullness, 4 cm in the largest diameter, surrounding the left ureter. A stent was placed in the left ureter, which led to the relief of his pain. A laparotomy was performed, with lysis of adhesions around the ureter. Final pathology of an abdominal lymph node and tissue from the mass revealed retroperitoneal fibrosis. After ten days of hospital admission, his creatinine decreased to 0.9 and his bowel habits returned to normal. He was discharged with outpatient follow-up. Six months later he remains asymptomatic and follow-up CT scan shows no evidence of recurrence. Discussion: An interesting case of unilateral retroperitoneal fibrosis presenting as a retroperitoneal mass responding well to surgical lysis of adhesions and no pharmacotherapy.
501 A case of PNH and intestinal ischemia Tonya L. Adams, MD, David E. Fleischer, MD, Gustavo Marino, MD, Eileen Rusnock, MD, Lee Li, MD. Georgetown University Medical Center, Washington, D.C., USA. Thrombotic complications, particularly microthrombi involving intra-abdominal veins leading to intestinal ischemia, have remained a major cause of morbidity in patients with paroxysmal nocturnal hemoglobinuria (PNH). While intestinal ischemia has been postulated to be the cause of recurrent bouts of abdominal pain in this population, direct antemortem evidence for this complication is scarcely documented in the literature. We describe a case of PNH in a patient who presented with abdominal distress 3 years after the initial diagnosis was established. Clinical features and a combination of diagnostic modalities, including radiography, serial endoscopies and histology were used to make the prompt diagnosis of intestinal ischemia. This is the first case in which the electronic microscopy of the gastrointestinal lesion is described. Our patient was successfully treated with conservative measures and anticoagulation.
502 Intraductal papillary mucin tumor (IPMT): Clinical characteristics in a cohort of young patients Adler MD Douglas G, Chari MD Suresh T, Dahl RN Tamela, Pearson MD FACG Randall K. Mayo Clinic, Rochester, MN. Introduction: IPMT is a neoplastic disorder of the pancreatic ducts characterized by an ectatic ductal system due to a mucin secreting, papillary mucosa. Typically, patients present late in life with symptoms compatible with chronic pancreatitis or malignancy. The diagnosis is often delayed due to confusion of early clinical symptoms with other disorders. Invasive carcinoma is often present at diagnosis and benign IPMT is considered pre-malignant. We report the clinical characteristics of 5 patients under the age of 50 with histologically proven IPMT. Methods: Patients with IPMT were identified through the Mayo Clinic Pancreas Clinic and Surgical Pathology Database from 1982 to the present. Information abstracted from charts of consenting patients is reported.