A unique presentation of the rare Nora's lesion, symptomatic bizarre parosteal osteochondromatous proliferation: A case report.

Bizarre parosteal osteochondromatous proliferations, also known as Nora's lesions, are rare benign tumors with a high recurrence rate. They are often difficult to identify because of their similar appearance to other tumors. We describe a 25-year-old healthy female patient with bizarre parosteal osteochondromatous proliferations in an uncommon location on the pelvic ilium, presenting with unique clinical findings of abdominal pain and femoral paresthesia and showing atypical radiographic findings. To the best of our knowledge, this is one of the very few cases ever reported in the literature of Nora's lesion in this particular location and possibly the first case ever with this specific presentation. The lesions' radiographic images, combined orthopedic and general surgery procedures, and histological analysis are detailed. The patient's continued 4-year follow-up has demonstrated no symptoms or evidence of recurrence.

Splenectomy in Gaucher Disease: A Call for Minimally Invasive Surgery.

OBJECTIVE: The objective of this article was to demonstrate that Gaucher disease (GD) patients with refractory hypersplenism and massive splenomegaly may successfully undergo hand-assisted laparoscopic splenectomy (HALS). METHODS: This was a retrospective audit conducted at the Gaucher clinic at a national referral center over a 10-year period. PATIENT POPULATION: This study included 8 GD patients who underwent hand-assisted or conventional laparoscopic splenectomy for massive or complicated splenomegaly between the years 2007 and 2017. RESULTS: Seven patients underwent an elective HALS procedure because of refractory hypersplenism, whereas 1 patient underwent an urgent conventional laparoscopic splenectomy because of torsion of a wandering spleen. Only 1 patient required conversion to open surgery because of multiple adhesions from a previous partial splenectomy. The mean weight of the removed spleens was 2373 g (range, 480 to 4900 g), mean craniocaudal length of the removed spleens was 25 cm (range, 20 to 33.5 cm), and mean operating time was 150 minutes (range, 96 to 280 min). Postoperative complications were limited to 2 patients and included thrombosis of the splenic vein stump in 1 patient, and propagation of a preoperative splenic vein thrombus to the portal system, as well as an accumulation of an intra-abdominal hematoma in another patient. There was no mortality. Mean length of hospital stay was 5 days (range, 2 to 11 d). CONCLUSION: HALS for GD patients with refractory hypersplenism and massive splenomegaly is safe and feasible in experienced hands.

Leiomyosarcoma of the inferior vena cava: radical surgery without vascular reconstruction.

Leiomyosarcoma of the inferior vena cava (IVC) is a rare tumor of mesenchymal origin, occurring most frequently in middle-aged women. Insidious complaints delay diagnosis, prognosis is poor, and the only curative modality remains an aggressive surgical resection yielding clear margins of disease. Commonly, radical tumor excision mandates caval repair or reconstruction, with significant related morbidity and mortality. We present a case of a 50-year-old woman with a leiomyosarcoma arising from the lower segment of the IVC, managed by surgical en-bloc resection of the tumor and IVC segment without further caval repair or reconstruction. During 14 months of follow-up the patient is well, had not had any complications, and is disease free.

Abdominal cocoon: a potential pitfall in patients with ovarian carcinoma.

Background. Abdominal cocoon, or sclerosing encapsulating peritonitis, is a rare condition characterized by partial or total encasement of small bowel and mesentery by a thick fibrocollagenous sack that looks like a cocoon. Within the sack, bowel loops are drawn together causing intestinal obstruction.Case presentation. We report on a 57-year-old female patient who developed a very unusual complication of ovarian cancer: abdominal cocoon formation.Conclusions. This report highlights the need for a timely diagnosis of sclerosing encapsulating peritonitis in cancer patients.

Lupus-associated pancreatitis.

OBJECTIVES: Involvement of the pancreas in systemic lupus erythematosus is rare. The purpose of this article is to provide a detailed review of lupus-associated pancreatitis. METHODS: We describe 3 patients with lupus-associated pancreatitis and review the English literature of the last 30 years, including the demographic, clinical, therapeutic, and prognostic aspects of this disorder. RESULTS: There were detailed descriptions of 77 patients, 88% were females. Median age was 27 years. In 44% of the patients pancreatitis developed within 1 year of the diagnosis of lupus, and 84% had active lupus at the time of pancreatitis. Abdominal pain was the most frequent pancreatitis-related symptom (88%), followed by nausea or vomiting (67%). In 97% the diagnosis of pancreatitis was based on laboratory evidence of elevated serum amylase or lipase. Abdominal computerized tomography and ultrasonography did not show signs of pancreatic involvement in 24 and 45%, respectively. The mortality rate was 27%; active lupus and some biochemical abnormalities were significantly associated with increased mortality. Treatment with corticosteroids or azathioprine was not associated with increased mortality. On the contrary, mortality was decreased in patients who were treated with these agents after the onset of pancreatitis (20% mortality, compared with 61% among those who were not treated with steroids for their pancreatitis, P = 0.005). CONCLUSIONS: Pancreatitis should be suspected in any SLE patient with abdominal pain. Mortality rate is related to both active lupus and some biochemical markers. In most cases, the onset of pancreatitis appears unrelated to previous treatment with steroids or azathioprine. Moreover, treatment with these medications improves prognosis.

Symptomatic diaphragmatic endometriosis ten years after total abdominal hysterectomy.

BACKGROUND: Endometriosis is a disease that affects women, mostly in the age range of 25-35 years, and in most cases pelvic organs are involved. Involvement of the diaphragm after hysterectomy is extremely uncommon. CASE: A 50-year-old woman presented to our department with right upper-quadrant abdominal pain. Ten years before her admission, she underwent total hysterectomy and right salpingo-oophorectomy for a large leiomyomatous uterus. On evaluation, a right diaphragmatic lesion was identified by computed tomography. An explorative laparotomy was then performed, which revealed a 4-cm diaphragmatic cyst compressing the liver surface and containing thick chocolate-colored material. The lesion was totally excised. Pathological examination confirmed the diagnosis of endometriotic cyst. CONCLUSION: The diagnosis of endometriosis involving the diaphragm with no evidence of disease in the pelvis 10 years after hysterectomy, although a rare situation, should be considered in the differential diagnosis of a symptomatic diaphragmatic lesion in a woman with a single functioning ovary.