This subtype is associated with familial adenomatous polyposis syndrome fap and, due to its intraabdominal location and involvement of vital organs, proves even more difficult to treat with surgical resection being the corner stone of current management. A cyst is an abnormal mass in the body thats filled with. Intra abdominal desmoplasticsmall round celltumor jaimie d. Kensuke adachi,1 yukari takada,2 and haruka okada3. The exact etiology of these tumors is unknown, but there are three known factors associated with desmoid tumors. Results neuroblastoma was the most common tumor constituting 46. Hcg tumor of the intraabdominal testis was, therefore, suspected. Intraabdominal desmoplastic small round cell tumor is a rare malignancy with a predilectionforyoungmales. Little is known about how to treat patients with recurrence.
A 21 year old male presented with abdominal pain of 3 months duration and recent complaints of vomiting, loose stools and weight loss of 5 kgs in 3 weeks. Abdominal wall desmoids may require abdominal wall resection and reconstruction while intraabdominal and mesenteric tumors may necessitate removal of a significant amount of bowel fig. Abdominal wall and intraabdominal desmoid tumors present unique surgical challenges due to their anatomic location. Preoperative assessment of intraabdominal disease spread in. An 11yearold girl with a malignant intraabdominal desmoplastic smallroundcell tumor dsrct and a high serum level of the tumor marker ca 125 is reported. The subject of this chapter is an abdominal tumour of special interest, not only on account of its fully successful treatment, but because the case was throughout watched and periodically examined by an allopathic surgeon of repute, who himself advised his patient to make the necessary long sea voyage from a distant hot country to london, to seek further skilled advice in regard to her. Pdf diagnosis and treatment approaches for intraabdominal. The most common symptom was abdominal pain in 26 57. By analyzing published papers, we discovered some common features of the disease. Differentiating between these two entities is important clinically because iaf is a benign tumor whereas gists frequently have malignant potential. The causes of pediatric abdominal masses are extensive, ranging from benign to neoplastic, and often originating from organs within the intraabdominal cavity table. Chowdhury d 2016 case report on a unique case of intra abdominal desmoid tumor clin case rep rev, 2016 doi.
Objective to compare the diagnostic performance of ultrasound and computed tomography ct for detecting pelvic and abdominal tumor spread in women with epithelial ovarian cancer. The article highlights an important point which has important therapeutic implications. Pdf bowel obstruction after treatment of intraabdominal. Adrenal myelolipoma is an uncommon, benign, and hormonally nonfunctioning tumor that is composed of mature adipose tissue and normal hematopoietic tissue. Any patient with an unexplained abdominal mass, such as a cyst, tumour or cancer, should seek treatment urgently. Intraabdominal testicular seminoma omics international. Gradually shrinking intra abdominal desmoid tumor derived from the stomach in a young boy. Tumor cells with collagen fibers were observed in histopathological examination, but heteromorphism and the nuclear fission image were not apparent. Desmoids do not have the ability to metastasize but can cause significant morbidity and mortality by local invasion. Cytogenetic abnormalities in an intraabdominal desmoplastic. Any information contained in this pdf file is automatically generated from.
Conclusion intraabdominal tumors are more common in males. A classification into abdominal wall tumours as well as extra and intra abdominal tumours is important with regard to further treatment and outcome. This type is more common in individuals with familial adenomatous polyposis fap, a genetic condition that leads to the development of colon cancer. Salem departmentofsurgery, yale universityschoolofmedicine, newhaven, connecticut background. Abdominal wall and intra abdominal desmoid tumors present unique surgical challenges due to their anatomic location.
Keywords desmoid tumor, aggressive fibromatosis, sonography, gardner syndrome, familial adenomatous polyposis. In rare instances, it occurs subsequent to gastrointestinal stromal tumor gist. Desmoid tumors, aggressive fibromatosis, are neoplasms that rarely become malignant and do not metastasize. Abdominal wall desmoids may require abdominal wall resection and reconstruction while intra abdominal and mesenteric tumors may necessitate removal of a significant amount of bowel fig. Cryptorchidism is the most common congenital malformation of the male genitourinary tract. This case highlights the need for careful consideration of a desmoid tumor when a rapidly growing spindle cell tumor is encountered in a post. Intraabdominal testicular seminoma in a woman with. An intraabdominal mass in a 56yearold woman gastroenterology. The outcomes are in the section titled followup figures. Intraabdominal desmoplastic small round cell tumor is a rare malignancy with a predilection for young males. The differential for an abdominal mass can be extensive and quite daunting, as it incorporates many systems including the gastrointestinal gi, genitourinary gu, and endocrine. About patients with gist in our institution from 1993 to 2010 were reevaluated based on their clinical and pathological data, the treatment strategies. A high serum ca 125 level may be a specific marker for dsrct, and thus may permit early diagnosis and.
Definisi tumor abdomen adalah suatu massa yang padat dengan ketebalan yang berbedabeda, yang disebabkan oleh sel tubuh yang yang mengalami transformasi dan tumbuh secara autonom lepas dari kendali pertumbuhan sel normal, sehingga sel tersebut berbeda dari sel normal dalam bentuk dan strukturnya. Intraabdominal fibromatosis iaf is an uncommon benign neoplasm that usually occurs in the mesentery or retroperitoneum and may, on occasion, mimic a gastrointestinal stromal tumor gist. The estimated incidence in the general population is two to four per million per year 8. Ambiguous presentation of an intraabdominal testicular. A yet rarer form of this rare pathology is the intraabdominal dt. An unusual intraabdominal tumor of the gastrointestinal.
Preoperative assessment of intraabdominal disease spread. A 57yearold male had undergone distal gastrectomy for advanced gastric cancer. Most cases to date are asymptomatic or have epigastric pain. Fiveyear survival of patients with stage i, ii, iii, and iv intraabdominal desmoid tumor were 95%, 100%, 89%, and 76% respectively p pdf download pdf. This special situation has clinical significance in imatinib era. The risk of malignancy in an undescended testis is 10% with the highest risk in an intraabdominal testis. Desmoid tumors are locally aggressive without potential for metastasis. Intraabdominal desmoid tumor mimicking gastric cancer. The clinical characteristics of imt are not clear and the treatment is controversial. These tumors may occur throughout the body, but are commonly found on the abdominal wall and within the intestinal mesentery. On examination, he had a huge epigastric intra abdominal mass. Laparoscopy is a valuable technique in the management of pediatric intraabdominal tumors. The patient was later diagnosed with testicular feminization syndrome, a form of male pseudohermaphroditism.
The intraperitoneal chemotherapy may be heated, which is intended to improve the tissue penetration, and this is. Desmoid tumors are monoclonal fibroblastic proliferations arising from soft tissue classified as intra. The estimated incidence in the general population is two to four per million per year. The cryptorchid testis is most often located in the inguinal canal. However, due to its large size, a mass may present as intraabdominal even though it. A case of intraabdominal desmoplastic smallroundcell tumor. Due to rarity, imaging findings in patients with testicular feminization syndrome and. Intra abdominal fibromatosis iaf is an uncommon benign neoplasm that usually occurs in the mesentery or retroperitoneum and may, on occasion, mimic a gastrointestinal stromal tumor gist. Intra abdominal desmoid tumor is also rare, except for familial adenomatosis coli. Surgery is the only curative treatment for intraabdominal and retroperitoneal sarcoma iars. The tumour weight was approximately one kilogram 920 grams figure 2. Mortality of intraabdominal desmoid tumors in patients.
Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. An intraabdominal tumor of unknown etiology was suspected, and an exploratory laparotomy was performed. Desmoplastic small round cell tumor dsrct is an aggressive, polyphenotypic, malignant tumor occurring predominantly in children and young adults, with a male predominance and an age range spanning the first through fifth decades. Jul 31, 20 intra abdominal fibromatosis iaf commonly develops in patients who had abdominal surgery. A 35yearold female presented elsewhere 3 years back with the complaints of diffuse abdominal pain, distention, on and off constipation for 10 months, for which she was managed on out patient department opd basis with laxatives. It affects mainly children and young adults and has a. Bowel obstruction after treatment of intraabdominal tumors article pdf available in european journal of pediatric surgery 204.
Intraabdominal desmoid tumor is a type of desmoid tumor that involves the abdominal organs. Under the light microscope, the tumor is composed of nests of small cells surrounded by an abundant desmoplastic stroma hg. This testicular tumor was histologically proven as seminoma. Intraabdominal desmoid tumors, particularly those derived from the stomach, are rare. The first case of intraabdominal desmoid tumor following a liver transplantation was described recently. Diagnostic challenges in incidental pediatric abdominal. Desmoidtype fibromatosis df, also known as desmoid tumor, is an extremely rare, benign, mesenchymal fibrous tumor with no potential for metastasis. It can arise from any part of the body, most commonly extraabdominally. Jun 20, 2019 abdominal masses are usually detected on physical examination. Desmoplastic smallroundcell tumor dsrct is an aggressive and rare cancer that primarily occurs as masses in the abdomen. As with this case, a sonogram is commonly the first diagnostic examination that is performed to determine the identity of the soft tissue tumor. Yang termasuk tumor intra abdomen antara lain, tumor hepar, tumor limpa lien, tumor lambungusus halus, tumor colon, tumor ginjal hipernefroma, tumor pankreas.
Gradually shrinking intraabdominal desmoid tumor derived. American college of surgeons division of education page 4 of 36. Despite the achieved grossly negative resection and the pathology that revealed microscopic disease at the margins, the patient received no adjunctive therapy. Intraabdominal and abdominal wall desmoid fibromatosis. Intraabdominal desmoplasticsmall round celltumor jaimie d. Rarely, an abdominal testicular tumor can cause abdominal pain, abdominal mass, and haematuria because of adjacent visceral infiltration 1. Pdf intraabdominal desmoplastic small round cell tumor. Giant intraabdominal desmoid tumor in a young male without. A 49yearold man married with five children, with no known previous complaint presented to the surgical unit at university charity teaching hospital with complaints of abdominal swelling for 4 months. Acsase medical student core curriculum intraabdominal and. He was referred to our hospital with no history of surgery, trauma, or familial adenomatous polyposis.
This subtype is associated with familial adenomatous polyposis syndrome fap and, due to its intra abdominal location and involvement of vital organs, proves even more difficult to treat with surgical resection being the corner stone of current management. Intra abdominal df can present sporadically, in sites of previous trauma, surgical scars and irradiation, or in association with familial adenomatous polyposis and gardner. Fiveyear survival of patients with stage i, ii, iii, and iv intra abdominal desmoid tumor were 95%, 100%, 89%, and 76% respectively p tumor size larger than 10 cm, and need for total parenteral nutrition were shown to further define survival within stages. Intraabdominal desmoid tumor is also rare, except for familial adenomatosis coli. Intraabdominal desmoid tumors mimicking gastrointestinal. Serum levels of carcinoembryonic antigen were found to be. The causes of pediatric abdominal masses are extensive, ranging from benign to neoplastic, and often originating from organs within the intra abdominal cavity table.
Tumor intra abdomen merupakan massa yang padat dengan ketebalan yang berbedabeda, yang disebabkan oleh sel tubuh yang mengalami transformasi dan tumbuh secara autonom lepas dari kendali pertumbuhan sel normal, sehingga sel tersebut berbeda dari sel normal dalam bentuk dan strukturnya. A desmoid tumor that developed after removal of a jejunal gist is reported. Abdominal wall desmoids have favorable outcomes when resected and it has. Hemorrhagic shock due to adrenal myelolipoma, to our. Desmoids in these areas may cause unique clinical problems for. Intra abdominal desmoid tumor should be considered as a differential diagnosis for a new single lesion in patients with gastrointestinal stromal tumor gist. Seminomas tumors are extremely sensitive to chemotherapy and radiotherapy 2. Cureus complex case of aggressive intraabdominal desmoid. Intraabdominal fibromatosis iaf commonly develops in patients who had abdominal surgery. Unique histological and immunocytochemical features distinguish the tumor from other members of the family of small round cell tumors of infancy and childhood.
Giant intraabdominal desmoid tumor in a young male. Immunostaining revealed betacatenin expression in the tumor cell nucleus. Laparoscopy in the management of pediatric intraabdominal tumors. Case report on a unique case of intraabdominal desmoid tumor. Abdominal masses can be the result of a number of factors, including an injury, cyst, benign tumor, cancer, or other disease. Abdominal wall desmoids have favorable outcomes when resected and it has been suggested. Intra abdominal desmoid tumors are generally observed in women at a higher incidence rate. Laparotomy was done through lower midline incision and total excision of the tumour was carried out. A yet rarer form of this rare pathology is the intra abdominal dt. An omental abscess mimicking an intraabdominal tumor.
One of the principal organs where intraabdominal masses occur is the liver. Diagnosis and management of the intraabdominal desmoid tumour. Intraabdominal mesenteric desmoid tumors dts after. Acute hemorrhage is the most dramatic manifestation of adrenal myelolipoma. By delivering chemotherapy intraperitoneally, drug exposure to the. Pdf serous ovarian cystadenomas usually present as bilateral small to medium size intraabdominal mass.
The objective of the present study was to observe the histopathological pattern of intra abdominal tumors in children less than 16 years. Intra abdominal desmoid tumor is a type of desmoid tumor that involves the abdominal organs. Intra abdominal desmoplastic small round cell tumor is a rare malignancy with a predilection for young males. The first case of intra abdominal desmoid tumor following a liver transplantation was described recently. Pdf bowel obstruction after treatment of intraabdominal tumors. Completely free and mobile intraabdominal tumor surgery. Intra abdominal desmoid tumors, particularly those derived from the stomach, are rare. We report a case of intraabdominal testicular tumor in a 36yearold married lady presenting with chief complaints of primary amenorrhea.
Intra abdominal desmoid tumor fibromatosis discussion. We here report the outcome in primary and recurrent sarcoma treated at the sarcoma center in aarhus, denmark. It is a rare tumor occurring in children and young adults, usually arising diffusely in the abdominal cavity with a predilection for the pelvic region. A case of intraabdominal desmoplastic smallroundcell. These tumors often infiltrate and destroy adjacent vital structures and organs. Intraabdominal and retroperitoneal softtissue sarcomas.
Methods an observational cohort study of 93 patients mean age 57. The risk of malignancy in an undescended testis is 10% with the highest risk in an intra abdominal testis. Intraabdominal desmoid tumors are rare and most often occur in patients with a history of familial adenomatous polyposis, surgery, or pregnancy. We report a case of an intraabdominal desmoid tumor mimicking the recurrence of gastric cancer. Here we describe a case of a 40yearold fertile man of fulbe origin who. A mass in the lower abdomen with a cryptorchidic testis strongly points towards the diagnosis of malignancy in abdominal testis. Painless enlargement of the testis is the common presentation of malignancy in an undescended testis. A high serum ca 125 level may be a specific marker for dsrct, and thus may permit early diagnosis and treatment of this fastgrowing tumor. An 11yearold girl with a malignant intra abdominal desmoplastic smallroundcell tumor dsrct and a high serum level of the tumor marker ca 125 is reported. Desmoplastic small round cell tumor is a tumor composed of small round cells associated with marked desmoplasia and multiphenotypic differentiation. Large intraabdominal desmoid tumour posing diagnostic and. Gradually shrinking intraabdominal desmoid tumor derived from the stomach in a young boy. Subsequent intraabdominal fibromatosis mimicking recurrent.
Chowdhury d 2016 case report on a unique case of intraabdominal desmoid tumor clin case rep rev, 2016 doi. The most common site is the abdominal serosa, although other sites have been described. Oct, 2018 intra abdominal desmoid tumor is a type of desmoid tumor that involves the abdominal organs. This article reports two rare cases involving the spontaneous regression of an unresectable intraabdominal inflammatory myofibroblastic tumor imt and summarizes the clinical characteristics of all published cases. Aug 17, 2011 an abdominal mass in a neonate, young child, or adolescent patient is something that every pediatrician needs to be wary of as these masses can indicate malignancy. It allows for tumor biopsy under direct vision, and adequate tissue is procured for all analyses.
Intra abdominal desmoplastic small round cell tumor. Desmoid tumors are rare and are most often located abdominally or intra abdominally. Acsase medical student core curriculum intraabdominal. Cysts intestinal tumors tumors of liver and spleen tumors of the ovaries abdominal tuberculosis intraabdominal tumors are frequently overlooked in children. Desmoid tumours are rare and are either found sporadically or more commonly associated with fap. Blended surgical education and training for life the value of laboratory testing is limited in the evaluation of simple hepatic cysts.
Abdominal masses are often incidentally discovered by a parent while bathing the child, palpated unexpectedly on routine physical examination, or detected on abdominal imaging. An estimated 7090% of hcc arises in the setting of cirrhosis, so patients with hepatitis b, hepatitis. Desmoid fibromatosis is a rare but locally aggressive tumor comprised of myofibroblasts. Gastrointestinal stromal tumor versus intraabdominal. Clear guidelines outlining possible therapy options including helpful algorithms are missing.
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