INTRABDOMINAL RUPTURE AND BLEEDING OF MESENTERIC-EPIPLOIC EWING’S SARCOMA:A RARE CASE REPORT
Abstract
Ewing sarcoma is a tumor of undifferentiated small round cells, which occurs most commonly in the bones of children and adolescents. Exceptionally, this tumor can have an extra skeletal origin arising from soft tissue. The most frequent sites of occurrence are the chest wall, lower extremities an paraverteblral regions. Less frequently, the tumor occurs in the pelvis and hip region, the retroperitoneum and the upper extremities(1). The age at the time of diagnosis, unlike its osseous counterpart, has a wide range. Clinical or imaging findings are nonspecific and diagnosis is based on histology. We present a rare case of intraabdominal Ewing sarcoma in a 32-year-old female, who presented to us with acute abdomen and a hemorrhagic palpable abdominal mass. Surgical biopsy of the mass showed histological features compatible with extra skeletal Ewing sarcoma.
Introduction
Ewing sarcoma is a malignant small, round, blue- cell tumor. It is a rare disease in which cancer cells are found in the bone or seldom in soft tissue. When compared Ewing sarcoma of bones and extra skeletal Ewing sarcoma(eES) displays several differences. First, eES has a more equal distribution between sexes instead of the male predilection of usual Ewing sarcoma. A second important difference is the age of occurrence, as in eES the average age is 20 years of age.
Extra skeletal Ewing sarcoma is an extremely rare condition, where the imaging and laboratory tests cannot give any diagnostic clew. The diagnosis is being confirmed by histochemical examination. Below it is described one of the three intra-abdominal eES which presented with acute abdominal symptoms and intra-abdominal bleeding.
Case report
The patient was a 32-year old woman who was admitted to our hospital for sudden abdominal pain, acute abdomen signs and presence of free fluid in the peritoneal cavity. After clinical examination, a palpable abdominal mass between hypogastrium and right iliac fossa was detected. From the past history, the patient used contraceptive pills. The patient was firstly admitted to the gynecological clinic with suspicious intraabdominal hemorrhage. Apart from blood tests, ultrasound imaging and blood cross-match, the patent could not complete the diagnostic follow.
During the diagnostic procedures, the health condition of the woman was worsened with signs of hemorrhagic shock and hemodynamic instability. Therefore, she was driven in the surgery room immediately. The most important element of biochemical control was the value of Ca125=250mg/dl(116,7U/ml), which became known after the surgery.
Surgical findings:
Figure 1
After a Caesarian incision from the gynecologists a gross amount of blood was extracted which fulfilled the whole lower abdomen. A bleeding mass with cystic degenerations was palpated with partial a=engagement of mesentery, jejunum and omentum. Macroscopic was not confirmed visible infiltration of the small intestine. After that, the surgeons performed a capable jejunal resection which included the ruptured and bleeding part of the mass, the companion mesentery and omental segment. Upon review of the rest of the abdomen, a small cystic tissue at the opposite site of the ovarian tube which was also removed. All excited species were sent for biopsy and pathological examination. The patient recovered well after the surgery.
Pathological findings:
Figure 2
Extensive and repeated inspections were required by virtue of the rarity of the diagnosis. The tumor departs from the gut wall but interferes serosa surface as it micro filtrates the lumen of small vessels’ mucosa. Massive bleeding filtration of the submucosal tunic was detected. The tumor cells were found with capable quantity of glycogen and expression of vimentin, c-kit, CD99, NSE and chromogranin in a moderate number of cells. The proliferative index Ki67 was 10%. The two sent nodes were free of tumor infiltration. The presence of abundant glycogen, the most limited expression of epithelial markers and strong diffuse expression of CD99 in combination with the new age of the patient and the absence of tumor in another organ, conduce to diagnosis of Ewing sarcoma. The diagnosis reconfirmed by a United States’ pathological department.
The patent recovered well and was referred to our oncologic clinic. She underwent chemotherapy over a period of six months after the surgery where there was not recurrence or metastasis of the disease. The follow-up continued for one year to our hospital. The patient deceased 26 months later because of disease recurrence and multi-organ metastasis.
Discussion
The extra-skeletal and intra-peritoneal Ewing sarcoma is a rare diagnosis, which can be done only with the increased use of histochemical and molecular examinations. Making an accurate diagnosis is critical for optimal patient management and prognostication. Imaging with Computed Scanning and Magnetic Resonance Imaging is able to provide information regarding the size of the tumor, the involvement of adjacent structures and the presence of
metastasis but the histopathological features are those who confirm the diagnosis(12). This may lead to a revision of traditional classification of intra-abdominal and retro-peritoneal tumors, previously based on morphological characteristics.
The case of extra skeletal intra-abdominal Ewing sarcoma with acute abdominal onset and intraperitoneal bleeding is a very rare event, where our case is the forth in the known till today international literature (3,11,12)
The age and the surgical treatment are the most important prognostic factors in management and treatment of eES. Young patients have significantly better survival compared to elderly patients (10). Further research needs to take place in order to determine the role of chemotherapy and/or radiotherapy before or after the surgery, as done in osseous Ewing sarcoma (3). Nevertheless, the increased frequency of intraabdominal and retroperitoneal primitive neuroectodermal tumors shows that Ewing sarcoma should be included in the differential diagnosis of cystic lesions in these anatomical structures.
References
1. “Extraskeletal Ewing’s sarcoma in the abdominal wall: a case report”, Aydinli, Ozturk, ActaOncologica, 2006
2. “Extraskeletal Ewing sarcoma”, Weerakkody and Pearson et al., radiopaedia.org
3. “Extra-skeletal Ewing’s sarcoma resembling acute abdomen. Report case”, Valdivia-Gòmez, Soto-Guerrero et al., Cir Cir 2010
4. “Extraskeletal Ewing’s sarcoma of small bowel:case report and literature review”. Adair A, Harris SA et al. JR CollSurgEdinb, 2001
5. “Image of the month-quiz case. Retroperitoneal extraskeletal Ewing sarcoma”, Rosa F, Tortorelli AP et al., Arch Surg, 2008
6. “Extraskeletal Ewing sarcoma of the abdominal wall”, Askri, Ben Farhalet al., Cancer Imaging, 2008
7. “Perigastricextraskeletal Ewing’s sarcoma: A case report”, Colocic, Grubor et al, World j Gastrenterol, 2009
8. “Huge peripheral primitive neuroextodermal tumor of the small bowel mesentery at nonage: A case report and review of the literature”, Liu, Xu et al, World J Clin Cases, 2016
9. “Blue Cell Tumor at unusual site: Retroperitoneal Ewing’s sarcoma”, Javagli, Karigoudar et al., Journal of Clinical and Diagnostic Research, 2016
10. “Primary primitive neuroectodermal tumor arising in the mesentery and ileocecum: A report of three cases and review of literature”, Peng, Yang, Wu et al, Experimental and Therapeutic Medicine, 2015
11. “Ewing’s sarcoma/peripheral primitive neuroextodermal tumor(pPNET) arising in the omentum as a multilocular cyst with intracystic hemorrhage”, Tanida, Tanioka, Inukaiet al., J Gastrenterol, 2000
12. “Intra-abdominal Ewing’s sarcoma”, Royal Australasian college of surgeons, J of sugery, 2011
Abstract
Ewing sarcoma is a tumor of undifferentiated small round cells, which occurs most commonly in the bones of children and adolescents. Exceptionally, this tumor can have an extra skeletal origin arising from soft tissue. The most frequent sites of occurrence are the chest wall, lower extremities an paraverteblral regions. Less frequently, the tumor occurs in the pelvis and hip region, the retroperitoneum and the upper extremities(1). The age at the time of diagnosis, unlike its osseous counterpart, has a wide range. Clinical or imaging findings are nonspecific and diagnosis is based on histology. We present a rare case of intraabdominal Ewing sarcoma in a 32-year-old female, who presented to us with acute abdomen and a hemorrhagic palpable abdominal mass. Surgical biopsy of the mass showed histological features compatible with extra skeletal Ewing sarcoma.
Introduction
Ewing sarcoma is a malignant small, round, blue- cell tumor. It is a rare disease in which cancer cells are found in the bone or seldom in soft tissue. When compared Ewing sarcoma of bones and extra skeletal Ewing sarcoma(eES) displays several differences. First, eES has a more equal distribution between sexes instead of the male predilection of usual Ewing sarcoma. A second important difference is the age of occurrence, as in eES the average age is 20 years of age.
Extra skeletal Ewing sarcoma is an extremely rare condition, where the imaging and laboratory tests cannot give any diagnostic clew. The diagnosis is being confirmed by histochemical examination. Below it is described one of the three intra-abdominal eES which presented with acute abdominal symptoms and intra-abdominal bleeding.
Case report
The patient was a 32-year old woman who was admitted to our hospital for sudden abdominal pain, acute abdomen signs and presence of free fluid in the peritoneal cavity. After clinical examination, a palpable abdominal mass between hypogastrium and right iliac fossa was detected. From the past history, the patient used contraceptive pills. The patient was firstly admitted to the gynecological clinic with suspicious intraabdominal hemorrhage. Apart from blood tests, ultrasound imaging and blood cross-match, the patent could not complete the diagnostic follow.
During the diagnostic procedures, the health condition of the woman was worsened with signs of hemorrhagic shock and hemodynamic instability. Therefore, she was driven in the surgery room immediately. The most important element of biochemical control was the value of Ca125=250mg/dl(116,7U/ml), which became known after the surgery.
Surgical findings:
Figure 1
After a Caesarian incision from the gynecologists a gross amount of blood was extracted which fulfilled the whole lower abdomen. A bleeding mass with cystic degenerations was palpated with partial a=engagement of mesentery, jejunum and omentum. Macroscopic was not confirmed visible infiltration of the small intestine. After that, the surgeons performed a capable jejunal resection which included the ruptured and bleeding part of the mass, the companion mesentery and omental segment. Upon review of the rest of the abdomen, a small cystic tissue at the opposite site of the ovarian tube which was also removed. All excited species were sent for biopsy and pathological examination. The patient recovered well after the surgery.
Pathological findings:
Figure 2
Extensive and repeated inspections were required by virtue of the rarity of the diagnosis. The tumor departs from the gut wall but interferes serosa surface as it micro filtrates the lumen of small vessels’ mucosa. Massive bleeding filtration of the submucosal tunic was detected. The tumor cells were found with capable quantity of glycogen and expression of vimentin, c-kit, CD99, NSE and chromogranin in a moderate number of cells. The proliferative index Ki67 was 10%. The two sent nodes were free of tumor infiltration. The presence of abundant glycogen, the most limited expression of epithelial markers and strong diffuse expression of CD99 in combination with the new age of the patient and the absence of tumor in another organ, conduce to diagnosis of Ewing sarcoma. The diagnosis reconfirmed by a United States’ pathological department.
The patent recovered well and was referred to our oncologic clinic. She underwent chemotherapy over a period of six months after the surgery where there was not recurrence or metastasis of the disease. The follow-up continued for one year to our hospital. The patient deceased 26 months later because of disease recurrence and multi-organ metastasis.
Discussion
The extra-skeletal and intra-peritoneal Ewing sarcoma is a rare diagnosis, which can be done only with the increased use of histochemical and molecular examinations. Making an accurate diagnosis is critical for optimal patient management and prognostication. Imaging with Computed Scanning and Magnetic Resonance Imaging is able to provide information regarding the size of the tumor, the involvement of adjacent structures and the presence of
metastasis but the histopathological features are those who confirm the diagnosis(12). This may lead to a revision of traditional classification of intra-abdominal and retro-peritoneal tumors, previously based on morphological characteristics.
The case of extra skeletal intra-abdominal Ewing sarcoma with acute abdominal onset and intraperitoneal bleeding is a very rare event, where our case is the forth in the known till today international literature (3,11,12)
The age and the surgical treatment are the most important prognostic factors in management and treatment of eES. Young patients have significantly better survival compared to elderly patients (10). Further research needs to take place in order to determine the role of chemotherapy and/or radiotherapy before or after the surgery, as done in osseous Ewing sarcoma (3). Nevertheless, the increased frequency of intraabdominal and retroperitoneal primitive neuroectodermal tumors shows that Ewing sarcoma should be included in the differential diagnosis of cystic lesions in these anatomical structures.
References
1. “Extraskeletal Ewing’s sarcoma in the abdominal wall: a case report”, Aydinli, Ozturk, ActaOncologica, 2006
2. “Extraskeletal Ewing sarcoma”, Weerakkody and Pearson et al., radiopaedia.org
3. “Extra-skeletal Ewing’s sarcoma resembling acute abdomen. Report case”, Valdivia-Gòmez, Soto-Guerrero et al., Cir Cir 2010
4. “Extraskeletal Ewing’s sarcoma of small bowel:case report and literature review”. Adair A, Harris SA et al. JR CollSurgEdinb, 2001
5. “Image of the month-quiz case. Retroperitoneal extraskeletal Ewing sarcoma”, Rosa F, Tortorelli AP et al., Arch Surg, 2008
6. “Extraskeletal Ewing sarcoma of the abdominal wall”, Askri, Ben Farhalet al., Cancer Imaging, 2008
7. “Perigastricextraskeletal Ewing’s sarcoma: A case report”, Colocic, Grubor et al, World j Gastrenterol, 2009
8. “Huge peripheral primitive neuroextodermal tumor of the small bowel mesentery at nonage: A case report and review of the literature”, Liu, Xu et al, World J Clin Cases, 2016
9. “Blue Cell Tumor at unusual site: Retroperitoneal Ewing’s sarcoma”, Javagli, Karigoudar et al., Journal of Clinical and Diagnostic Research, 2016
10. “Primary primitive neuroectodermal tumor arising in the mesentery and ileocecum: A report of three cases and review of literature”, Peng, Yang, Wu et al, Experimental and Therapeutic Medicine, 2015
11. “Ewing’s sarcoma/peripheral primitive neuroextodermal tumor(pPNET) arising in the omentum as a multilocular cyst with intracystic hemorrhage”, Tanida, Tanioka, Inukaiet al., J Gastrenterol, 2000
12. “Intra-abdominal Ewing’s sarcoma”, Royal Australasian college of surgeons, J of sugery, 2011
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