Two Cases of Plexiform Angiomyxoid Myofibroblastic Tumor (PAMT) of Gastrointestinal (GI) Tract: One With 8-Year Follow-up, One in Distal Jejunum

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S56-S56
Author(s):  
Mustafa Erdem Arslan ◽  
Timothy Jennings ◽  
Hua Li ◽  
Edward Lee ◽  
Ankesh Nigam ◽  
...  
Keyword(s):  
Small Bowel ◽  
Imaging Finding ◽  
Disease Patient ◽  
Outlet Obstruction ◽  
Muscularis Propria ◽  
Gastric Antrum ◽  
Proximal Jejunum ◽  
Spindle Cell Neoplasm ◽  
Distal Jejunum

Abstract Objectives PAMT is an exceedingly rare mesenchymal tumor of the gastric antrum that was first described in 2007. Although likely benign, long-term follow-up is limited due to its recent description and rarity. In small bowel, only four cases of PAMT have been reported, two in duodenum and two in proximal jejunum. Methods (A) A 16-year-old female presented with GI bleeding and gastric outlet obstruction. Imaging studies suggested a gastrointestinal stromal tumor (GIST) arising in duodenum. An open biopsy revealed a bland spindle cell neoplasm. Resection of the tumor was performed. (B) A 68-year-old male presented with an incidental mesenteric mass on imaging during hematuria workup. He underwent surgical resection of the mass to exclude a GIST. Results Case A was a 15.0-cm mass arising in gastric antrum, and case B was an 8.0-cm mass arising in antimesenteric side of distal jejunum. Both tumors showed multinodular plexiform growth pattern and foci of cystic changes, with a proliferation of bland spindle cells in a prominent myxoid matrix. Numerous vascular structures were noted without tumor necrosis, nuclear atypia, or mitotic activity. The lesional cells were positive for actin and negative for desmin. The lesional cells were negative for other markers including cytokeratin, CD34, CD117, DOG-1, S-100, and PDGFRA (case B). Both masses were based in the subserosa with focal extension to the muscularis propria in case A. Patient A was followed up for 8 years with no evidence of disease. Patient B was lost to follow-up. Conclusion While clinical presentation and imaging finding of PAMT overlap with GIST, PAMT appears to be more indolent. PAMT of distal jejunum has not been previously reported. Small bowel PAMT is much less common than gastric PAMT but can involve variable segments. PAMT may be underdiagnosed.

scholarly journals Brown Bowel Syndrome Is a Rare and Commonly Missed Disease: A Case Report and Literature Review

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Joaquin Ponce-Zepeda ◽  
Wenchang Guo ◽  
Giorgioni Carmen ◽  
Daniel Moon Kim ◽  
Gregory C. Albers ◽  
...  
Keyword(s):  
Vitamin E ◽  
Small Bowel ◽  
Chronic Diarrhea ◽  
Muscularis Propria ◽  
Mitochondrial Damage ◽  
Vitamin E Deficiency ◽  
Case Presentation ◽  
History Of ◽  
Resected Small Bowel

Background. Brown bowel syndrome (BBS) is a rare gastrointestinal condition, and vitamin E deficiency has been considered to be a main contributor. However, vitamin E deficiency has been found in only a few patients throughout the published literature studies and its cutoff lab value for diagnosis is not entirely clarified. Case Presentation. A 56-year-old female patient with a history of congenital bowel obstruction (repaired at birth) presented with bloating, abdominal pain, and chronic diarrhea. Endoscopy identified unremarkable gastrointestinal mucosa except a few small polyps in the colon. A partial obstruction was detected by a small bowel follow-through series and then confirmed by CT scan. The resected small bowel was significantly dilated with a thickened brown wall and extensive serosal adhesion. Microscopic examination revealed unremarkable mucosa, but dense granular brown pigments were identified in the cytoplasm of the smooth muscle cells in the muscularis propria. These deposits resulted to be lipofuscin, and BBS was diagnosed. The patient was asymptomatic at 9-month follow-up after surgery without vitamin E supplement. Conclusion. Mitochondrial damage with lipofuscin deposition is at the root of BBS pathogenesis. Any etiology associated with mitochondrial damage can cause this disease, and vitamin E deficiency is just one of them. Dysmotility from extensive serosal adhesion could be a possible etiology for this patient. Due to overlapping symptoms, lipofuscin deposition primarily in the muscularis propria, and unclear serum value of vitamin E, this syndrome is often missed in routine clinical practice from the superficial biopsy. A transmural biopsy is necessary for a definite diagnosis.

scholarly journals Solitary fibrous tumour of caecum wall: an unlikely cause of low gastrointestinal haemorrhage

2018 ◽  
pp. bcr-2018-227238
Author(s):  
Mariana Nogueira Santos ◽  
Amelia Brandao Tavares ◽  
Fernando Arruda Viveiros ◽  
Helena Baldaia
Keyword(s):  
Clinical Presentation ◽  
Spindle Cell ◽  
Muscularis Propria ◽  
Gastrointestinal Haemorrhage ◽  
Right Hemicolectomy ◽  
Solitary Fibrous Tumour ◽  
Radical Right ◽  
Spindle Cell Neoplasm ◽  
Cell Neoplasm

Solitary fibrous tumour (SFT), previously denominated as haemangiopericytoma, is a rare, spindle cell neoplasm that was first described in the thoracic pleura. It is now known that this tumour may develop from almost any anatomic location. We report a case of SFT, in a 65-year-old man, which was located in the muscularis propria layer of the caecum with involvement of the serosa and the ileocecal appendix, location never described in the literature, and with an uncommon clinical presentation of hematochezia. A radical right hemicolectomy was performed, and the patient was asymptomatic without evidence of metastasis or relapse after 6 months of follow-up.

scholarly journals Gastric Ganglioneuroblastoma in an Elder Man: A Case Report and Literature Review

2021 ◽  
Author(s):  
xuetong jiang ◽  
Jianqiang Wu ◽  
Feng Su ◽  
Hailong Huang ◽  
Yang Ding ◽  
...  
Keyword(s):  
Treatment Guidelines ◽  
Submucosal Tumor ◽  
Gastrointestinal Surgery ◽  
Muscularis Propria ◽  
Laparoscopic Approach ◽  
Gastric Antrum ◽  
Arterial Phase ◽  
Tissue Mass ◽  
Abdomen Ct

Abstract Background: GNB is a moderate tumor of sympathetic origin, which is considered a disease of children with the majority of cases less than five years old and it rarely occurs in adults. There are no treatment guidelines for adults GNB. Here, we reported a rare case of adult gastric GNB, and was resected completely by laparoscopic approach.Case presentation:A 73-year-old man presented with dull pain in the upper abdomen along with abdominal distension for one month. Gastroscopy examination revealed chronic gastritis and submucosal tumor of gastric antrum. Endoscopic ultrasonography showed a hypoechoic mass in gastric antrum arising from the muscularis propria. Combined with the characteristics of gastroscope and ultrasound , the mass was suspected as gastrointestinal stromal tumor. Abdomen CT scan of revealed irregular soft tissue mass in gastric antrum with heterogeneous enhancement in arterial phase, and gastric cancer was suspected.The patient was addressed to the department of gastrointestinal surgery. The mass was completely resected in laparoscopic surgery. Histopathology reported that the mass contained differentiation neuroblasts, mature ganglion cells and ganglioneuromatisis component. Immunohistochemical staining revealed tumor cells were immunoreactive for CD56, Syn, S100 , while negative for CK, CD117, DOG-1, CgA. The pathological diagnosis was GNBi and the patient was staged as stage I. The patient received no adjuvant chemotherapy or radiotherapy.Until his one-year follow-up, the patient was doing well and without any signs of recurrence.Conclusion:Although the rarity of gastric GNB as a primary site of origin, it should be considered in the differential diagnosis of gastric masses in adult. Radical surgery is sufficient for the treatment of GNBi and long-term follow-up should be performed.

scholarly journals Clinical Efficacy of Various Diagnostic Tests for Small Bowel Tumors and Clinical Features of Tumors Missed by Capsule Endoscopy

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Jung Wan Han ◽  
Sung Noh Hong ◽  
Hyun Joo Jang ◽  
Seong Ran Jeon ◽  
Jae Myung Cha ◽  
...  
Keyword(s):  
Small Bowel ◽  
Clinical Features ◽  
Capsule Endoscopy ◽  
Diagnostic Tools ◽  
Proximal Jejunum ◽  
Inflammatory Fibroid Polyp ◽  
Small Bowel Tumors ◽  
Negative Findings ◽  
Fibroid Polyp ◽  
Distal Jejunum

Background. We aimed to evaluate the efficacy of various diagnostic tools such as computerized tomography (CT), small bowel follow-through (SBFT), and capsule endoscopy (CE) in diagnosing small bowel tumors (SBTs). Additionally, we aimed to evaluate the clinical features of SBTs missed by CE. Methods. We retrospectively studied 79 patients with histologically proven SBT. Clinical data were analyzed with particular attention to the efficacy of CT, SBFT, and CE in detecting SBT preoperatively. We also analyzed the clinical features of SBTs missed by CE. Results. The most common symptoms of SBT were bleeding (43%) and abdominal pain (13.9%). Diagnostic yields were as follows: CT detected 55.8% of proven SBTs; SBFT, 46.1%; and CE, 83.3%. The sensitivity for detecting SBTs was 40.4% for CT, 43.9% for SBFT, and 79.6% for CE. Two patients with nondiagnostic but suspicious findings on CE and seven patients with negative findings on CE were eventually found to have SBT. These nine patients were eventually diagnosed with gastrointestinal stromal tumor (4), small polyps (3), inflammatory fibroid polyp (1), and adenocarcinoma (1). These tumors were located in the proximal jejunum (5), middle jejunum (1), distal jejunum (1), and proximal ileum (1). Conclusion. CE is more efficacious than CT or SBFT for detecting SBTs. However, significant tumors may go undetected with CE, particularly when located in the proximal jejunum.

Long-term stability of fusiform dilatation of the internal carotid artery following surgery adjacent to the circle of Willis: report of 2 cases

2020 ◽  
pp. 1-4
Author(s):  
Madeline B. Karsten ◽  
R. Michael Scott
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Arachnoid Cyst ◽  
Pediatric Patients ◽  
Internal Carotid ◽  
Imaging Finding ◽  
Neurological Status ◽  
Suprasellar Region

Fusiform dilatation of the internal carotid artery (FDCA) is a known postoperative imaging finding after craniopharyngioma resection. FDCA has also been reported following surgery for other lesions in the suprasellar region in pediatric patients and is thought to be due to trauma to the internal carotid artery (ICA) wall during tumor dissection. Here, the authors report 2 cases of pediatric patients with FDCA. Case 1 is a patient in whom FDCA was visualized on follow-up scans after total resection of a craniopharyngioma; this patient’s subsequent scans and neurological status remained stable throughout a 20-year follow-up period. In case 2, FDCA appeared after resection and fenestration of a giant arachnoid cyst in a 3-year-old child, with 6 years of stable subsequent follow-up, an imaging finding that to the authors’ knowledge has not previously been reported following surgery for arachnoid cyst fenestration. These cases demonstrate that surgery involving dissection adjacent to the carotid artery wall in pediatric patients may lead to the development of FDCA. On very long-term follow-up, this imaging finding rarely changes and virtually all patients remain asymptomatic. Neurointerventional treatment of FDCA in the absence of symptoms or significant late enlargement of the arterial ectasia does not appear to be indicated.

Fourth Ventricle Outlet Obstruction and Diverticular Enlargement of Luschka Foramina in a Child with Down Syndrome

2020 ◽  
pp. 1-4
Author(s):  
Valentina Orlando ◽  
Pietro Spennato ◽  
Maria De Liso ◽  
Vincenzo Trischitta ◽  
Alessia Imperato ◽  
...  
Keyword(s):  
Down Syndrome ◽  
Fourth Ventricle ◽  
Endoscopic Third Ventriculostomy ◽  
Obstructive Hydrocephalus ◽  
Radiological Finding ◽  
Outlet Obstruction ◽  
Third Ventriculostomy ◽  
Viable Option ◽  
Case Presentation

<b><i>Introduction:</i></b> Hydrocephalus is not usually part of Down syndrome (DS). Fourth ventricle outlet obstruction is a rare cause of obstructive hydrocephalus, difficult to diagnose, because tetraventricular dilatation may suggest a communicant/nonobstructive hydrocephalus. <b><i>Case Presentation:</i></b> We describe the case of a 6-year-old boy with obstructive tetraventricular hydrocephalus, caused by Luschka and Magen­die foramina obstruction and diverticular enlargement of Luschka foramina (the so-called fourth ventricle outlet obstruction) associated with DS. He was treated with endoscopic third ventriculostomy (ETV) without complications, and a follow-up MRI revealed reduction of the ventricles, disappearance of the diverticula, and patency of the ventriculostomy. <b><i>Conclusion:</i></b> Diverticular enlargement of Luschka foramina is an important radiological finding for obstructive tetraventricular hydrocephalus. ETV is a viable option in tetraventricular obstructive hydrocephalus in DS.

scholarly journals Unusual Tissue – Unusual Issue: Pancreatic Heterotopia Presenting as Gastric Outlet Obstruction

2021 ◽  
pp. 338-343
Author(s):  
Thu L. Nguyen ◽  
Shivani Kapur ◽  
Stephen C. Schlack-Haerer ◽  
Grzegorz T. Gurda ◽  
Milan E. Folkers
Keyword(s):  
Gastric Outlet Obstruction ◽  
Epigastric Pain ◽  
Outlet Obstruction ◽  
Needle Aspiration ◽  
Pancreatic Tissue ◽  
Cystic Mass ◽  
Pancreatic Heterotopia ◽  
History Of

Pancreatic heterotopia (PH) is a common, but typically small (<1 cm), incidental and asymptomatic finding; however, PH should be considered even for large and symptomatic upper gastrointestinal masses. A 27-year-old white woman presented with a 3-week history of burning epigastric pain, nausea, early satiety, and constipation. Physical examination revealed epigastric and right upper quadrant tenderness with normal laboratory workup, but imaging revealed a 5-cm, partly cystic mass arising from the gastric antrum with resulting pyloric stenosis and partial gastric outlet obstruction. Endoscopic ultrasound-guided fine needle aspiration revealed PH – an anomalous pancreatic tissue lying in a nonphysiological site. The patient ultimately underwent a resection and recovered uneventfully, with a complete pathologic examination revealing normal exocrine pancreatic tissue (PH type 2) without malignant transformation. We report a case of heterotopic pancreas manifesting as severe gastric outlet obstruction, in addition to a thorough diagnostic workup and surgical follow-up, in a young adult. Differential diagnoses and features that speak to benignity of a large, symptomatic mass lesion (PH in particular) are discussed.

An elderly male with lower urinary tract symptoms during COVID-19 pandemic: Extraperitoneal perforation of bladder diverticulum

2021 ◽  
pp. 039156032110229
Author(s):  
Amit Sharma ◽  
Deepak Biswal ◽  
Satyadeo Sharma ◽  
Siddhant Roy
Keyword(s):  
Bladder Outlet Obstruction ◽  
Outlet Obstruction ◽  
Transurethral Resection Of Prostate ◽  
Bladder Diverticulum ◽  
Elderly Male ◽  
Bladder Drainage ◽  
Intravenous Antibiotics ◽  
Urinary Tract Symptoms ◽  
Lower Urinary

Case: We present a case of spontaneous extra-peritoneal rupture of an acquired diverticulum an elderly male with symptoms of bladder outlet obstruction who presented in emergency with acute abdomen. Outcome: The acute phase was managed conservatively with bladder drainage and intravenous antibiotics. He recently underwent Transurethral Resection of Prostate. He is asymptomatic on follow-up. Conclusions: Acquired bladder diverticulum are rare in adults and are mostly seen in patients with high pressure bladder due to bladder outlet obstruction. Atraumatic extraperitoneal ruptures of diverticulum are uncommonly reported.

scholarly journals Comparative short-term and long-term outcomes between internal and external intestinal plication in the management of small bowel obstruction

BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Song Liu ◽  
Qiongyuan Hu ◽  
Lihua Shao ◽  
Xiaofeng Lu ◽  
Xiaofei Shen ◽  
...  
Keyword(s):  
Small Bowel ◽  
Small Bowel Obstruction ◽  
Bowel Obstruction ◽  
Short Term ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Gut Dysfunction ◽  
Gut Function

Abstract Background Small bowel obstruction (SBO) is common and usually requires surgical intervention. Intestinal plication is a traditional but critical strategy for SBO in certain scenarios. This study is to compare the short-term and long-term outcome between internal and external plications in the management of SBO. Methods All patients receiving intestinal plication in our hospital were retrospectively collected. Short-term outcome including postoperative complications, reoperation, postoperative ICU stay, starting day of liquid diet and postoperative hospitalization, as well as long-term outcome including recurrence of obstruction, readmission, reoperation and death were compared between groups. Gut function at annual follow-up visits was evaluated as well. Results Nine internal and 11 external candidates were recruited into each group. The major causes of plication were adhesive obstruction, abdominal cocoon, volvulus and intussusception. Lower incidence of postoperative complication (p = 0.043) and shorter postoperative hospitalization (p = 0.049) was observed in internal group. One patient receiving external plication died from anastomosis leakage. During the 5-year follow-up period, the readmission rate was low in both groups (22.2 % vs. 9.1 %), and none of patients required reoperation or deceased. None of patients exhibited gut dysfunction, and all patients restored normal gut function after 4 years. Patients in external group demonstrated accelerated recovery of gut function after surgery. Conclusions This study compares short-term and long-term outcome of patients receiving internal or external intestinal plication. We suggest a conservative attitude toward external plication strategy. Surgical indication for intestinal plication is critical and awaits future investigations.

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