scholarly journals Kaposiform Hemangioendothelioma of the GI Tract: An Exception to Occam’s Principle in an Adult with SBO

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Luis E. Aguirre ◽  
Robert A. Ali ◽  
Darcy A. Kerr ◽  
Mahsa Khanlari ◽  
Gilberto Lopes

Kaposiform hemangioendothelioma (KHE) is a rare and locally aggressive vascular tumor with histological features resembling Kaposi sarcoma and capillary hemangioma mainly occurring in children and adolescents. Approximately 200 cases have been reported since its original description in 1993, with the vast majority presenting at an early age as raised ill-defined lesions with a red-blue hue mainly involving the skin and soft tissues in the extremities. Cases in adults remain extremely rare. Herein, we describe the case of a 29 year-old man who presented with progressive abdominal pain for 4 months and signs of obstipation found to be consistent with small bowel volvulus. The patient underwent exploratory laparotomy and resection of 55 cm of necrotic small bowel followed by enteroenterostomy and anastomosis. Microscopic examination revealed KHE involving small intestinal mesentery, muscularis propria, and submucosa. His recovery was uneventful and he was discharged after stabilization, opting to manage him expectantly with abdominopelvic imaging and to monitor for development of Kasabach-Merritt phenomenon. To our knowledge, this represents the first reported case of this entity presenting as intestinal obstruction in an adult for which we also present a review of the existing literature and possible treatment options.

2000 ◽  
Vol 124 (10) ◽  
pp. 1542-1544 ◽  
Author(s):  
Jennifer J. Wilken ◽  
Frederick A. Meier ◽  
Michael J. Kornstein

Abstract Kaposiform hemangioendothelioma is a rare pediatric neoplasm that presents most commonly in the soft tissues. We report the case of a 1-month-old infant who presented with stridor and was found to have a diffusely infiltrating tumor in the thymus that extended into the pericardium and up the carotid sheaths. Histologic examination revealed a vascular tumor infiltrating among the lobules of the lymphocyte-depleted thymus. The lesion had features of both a capillary hemangioma and Kaposi sarcoma. Immunoperoxidase studies on formalin-fixed, paraffin-embedded tissue demonstrated the neoplastic endothelial cells to be positive for vascular markers CD31 and CD34. Antibody to factor VIII-related antigen labeled feeding vessels, but failed to stain the lobules of tumor. Although these tumors have been treated in a fashion similar to capillary hemangiomas in the past, it may be important to differentiate Kaposiform hemangioendotheliomas because of their association with Kasabach-Merritt syndrome and recent success with more aggressive chemotherapy regimens.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Qurratulain Chundriger ◽  
Muhammad Usman Tariq ◽  
Jamshid Abdul-Ghafar ◽  
Arsalan Ahmed ◽  
Nasir Ud Din

Abstract Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. Methods We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. Results Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. Conclusions KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.


2021 ◽  
Vol 20 (3) ◽  
pp. 74-91
Author(s):  
L. A. Khachatryan ◽  
I. S. Kletskaya ◽  
A. N. Remizov ◽  
G. A. Novichkova ◽  
A. A. Maschan

Kaposiform hemangioendothelioma (KHE) is a rare, usually congenital vascular tumor. It resembles Kaposi sarcoma histologically, but etiologically it is not associated with herpes simplex virus type 8. KHE refers to tumors of intermediate malignancy degree. The most severe complication is the addition of thrombocytopenia and consumption coagulopathy, i.e. development of the Kasabach–Merritt syndrome/phenomenon (KMS), which determines the high mortality rate (up to 30%) in this histological variant. The frequency of occurrence of KMS is unknown. Over Patients with KHE/KMS have clear clinical and laboratory characteristics, which in most cases allow make to diagnose without histological confirmation. Over 7-year follow-up period 32 patients with KHE were registered in our center; in 90.6% of cases it was complicated by the development of KMS. The study was approved by the Independent Ethics Committee and Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. In the most of patients the tumor was detected from birth (84%), in half of the cases (52%) hematological complications were diagnosed simultaneously with the detection of the tumor. Сommon local complications include joint contractures, destruction of bone tissue, and invasion of neighboring organs. The half of the patients had changes in the heart function: from minor cardiac pathology to congenital defects. In addition, there were clinical and instrumental changes associated with volume overload: an increase in liver size, myocardial hypertrophy. Despite the presence of clear clinical and laboratory characteristics of KMS, some cases require differential diagnosis with other vascular anomalies accompanied by thrombocytopenia and consumption coagulopathy – with congenital hemangiomas (rapidly involuting congenital hemangioma), multifocal lymphangioendotheliomatosis with thrombocytopenia, kaposiform lymphangiomatosis, venous malformations. The parents of the patients agreed to use the information, including photos of children, in scientific research and publications.


2018 ◽  
Vol 14 (4) ◽  
pp. 322-329 ◽  
Author(s):  
Irene Schmid ◽  
Anne K. Klenk ◽  
Monika Sparber-Sauer ◽  
Ewa Koscielniak ◽  
Rebecca Maxwell ◽  
...  

2021 ◽  
Vol 8 (5) ◽  
pp. 83
Author(s):  
Jae-Eun Hyun ◽  
Hyun-Jung Han

A 7-month-old neutered male poodle dog presented with general deterioration and gastrointestinal symptoms after two separate operations: a jejunotomy for small-intestinal foreign body removal and an exploratory laparotomy for diagnosis and treatment of the gastrointestinal symptoms that occurred 1 month after the first surgery. The dog was diagnosed as having small-bowel obstruction (SBO) due to intra-abdominal adhesions and small-bowel fecal material (SBFM) by using abdominal radiography, ultrasonography, computed tomography, and laparotomy. We removed the obstructive adhesive lesion and SBFM through enterotomies and applied an autologous peritoneal graft to the released jejunum to prevent re-adhesion. After the surgical intervention, the dog recovered quickly and was healthy at 1 year after the surgery without gastrointestinal signs. To our knowledge, this study is the first report of a successful treatment of SBO induced by postoperative intra-abdominal adhesions and SBFM after laparotomies in a dog.


2021 ◽  
Vol 108 (Supplement_3) ◽  
Author(s):  
M Soto Dopazo ◽  
E Pérez Prudencio ◽  
A Arango Bravo ◽  
C Nuño Iglesias ◽  
C Mateos Palacios ◽  
...  

Abstract INTRODUCTION Internal hernias caused by broad ligament defects are an infrequent cause of bowel obstruction. These defects may be congenital or acquired mainly by gynecological antecedents. Small bowel is the most common affected and the diagnosis is difficult due to nonspecific symptoms and absences of characteristic radiological signs. MATERIAL AND METHODS We report the cases of three women aged from 43 to 56 years old, who came to the emergency with abdominal pain, vomiting and bloating of hours duration. One patient has a history of laparoscopic appendectomy, the rest of them with no surgical history. In all of the cases, x-rays showed dilatation of small bowel loops and air-fluid levels and the abdominal TC revealed a generalized distention of bowel loops with transition point in the terminal ileum with no identifiable cause compatible with small bowel obstruction. RESULTS We decided to perform an urgent surgery with an exploratory laparotomy in one case and the rest by laparoscopic approach, finding an internal hernia occasioned by incarceration of small bowel through a broad ligament defect. In all cases, the hernia content was liberated without evidence of ischemia with no need for intestinal resection, and the defect was closed. All patients had a favourable postoperative course without complications. DISCUSSION Broad ligament defects are a rare cause of internal hernias. These are difficult to diagnose clinically as well as radiologically for an absence of characteristic signs. A high level of clinical suspicion allows early diagnosis and the treatment should be performed as soon as possible to reduce the chances of intestinal necrosis.


2021 ◽  
pp. 000313482199867
Author(s):  
Nikolaos G Symeonidis ◽  
Kalliopi E Stavrati ◽  
Efstathios T Pavlidis ◽  
Kyriakos K Psarras ◽  
Eirini Martzivanou ◽  
...  

B-lymphoblastic lymphoma is a neoplasm of immature B cells and is characterized by aggressive behavior and disease progression. Common sites of involvement are skin, lymph nodes, bone, soft tissues, breast, and the mediastinum. Gastrointestinal lesions are rarely encountered and therefore not fully described. We herein report the case of a 28-year-old male, who presented with abdominal pain and CT scan showed a tumor involving the small bowel and its mesentery. He underwent emergency laparotomy and enterectomy. Histopathology report revealed B-lymphoblastic lymphoma affecting the small bowel and the adjacent mesentery. This is the first documented case of a small bowel tumor diagnosed as B-lymphoblastic lymphoma in published literature.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Kodai Nagakari ◽  
Akikazu Yago ◽  
Yu Ohkura ◽  
Daisuke Tomita ◽  
Shusuke Haruta ◽  
...  

Abstract Background Pyogenic granuloma is a benign vascular tumor, usually occurring on the skin or in the oral cavity. Small intestinal pyogenic granuloma is extremely rare, but intestinal intussusception due to the tumor is even rarer. Only 3 cases have been reported in the English literature at this writing. Case presentation An 86-year-old woman presented with abdominal pain and vomiting. Laboratory data discovered anemia. Contrast-enhanced computed tomography revealed small bowel obstruction due to intestinal intussusception. After decompression by long tube for 1 week, the obstruction did not improve and the anemia got worse. Therefore, laparoscopic assisted small bowel resection was performed as a diagnostic therapy. Pathology confirmed the diagnosis of pyogenic granuloma. The postoperative course was uneventful and the patient was discharged 10 days after surgery. Conclusions We experienced a case of intestinal intussusception and progressive anemia due to pyogenic granuloma of the ileum. Although the condition is extremely rare, surgeons must take into consideration the tumor in similar cases, and complete surgical resection is required.


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