Primary cutaneous leiomyosarcoma (PCL) are soft-tissue sarcoma, arising in the dermis, with or without extension into the subcutis. They are thought to have an indolent course compared to their subcutaneous counterparts, they may recur but rarely metastases. We report the case of a patient with a PCL arising in the anterior trunk wall who developed pulmonary, bone and retroperitoneal metastases 6 years after wide surgical excision of the primary tumor. Leiomyosarcoma (LMS) are soft-tissue sarcoma which arise from smooth muscles. They can involve either peripheral soft tissues sites also called superficial LMS or deep soft tissues sites. Superficial LMS are rare mesenchymal neoplasms accounting for about 7% of soft-tissue neoplasms and 0.04% of all cancers. They have two subdivisions, cutaneous and subcutaneous forms basis of their histogenesis and different clinical and prognostic implications. Primary cutaneous leiomyosarcoma (PCL) are the rarest subtype, arising in the dermis, with or without extension into the subcutis. They are thought to have an indolent course compared to their subcutaneous counterparts; they may recur but rarely metastases. We report the case of a patient with a PCL arising in the anterior trunk wall who developed pulmonary, bone and retroperitoneal metastases 6 years after wide surgical excision of the primary tumor.A 30-year-old, otherwise healthy male presented to the outpatient department of dermatology in 2007 with a slow growing mass on his anterior chest wall. The swelling was present for more than 10 years without causing any complaint. During the year before presentation, the mass had progressively increased in size and became tender. No history of trauma, infection or radiation was present. The physical examination found an exophytic, reddish-brown swelling of 5cm with central ulceration of 2cm arising from the right pre-sternal region. A cutaneous biopsy was taken. It revealed a dermal fusocellular proliferation with pleomorphic and atypical nuclei but no evidence of mitosis. Hence, the diagnosis of atypical leiomyoma was rendered. Based on the above findings, the patient was referred to surgical department. Complete excision of the lesion was carried out through a periareolar incision. The distance of the tumor from the nearest resection margin was 1cm. Subcutaneous tissue and fascia of the pectoralis major was removed too.The excised skin nodule revealed a mass measuring around 4.5×4.5×2cm located in the dermis. Histopathological examination found a highly cellular mesenchymal proliferation especially in the deeper portions of the tumor. Numerous atypical mitotic figures were noted ranging from 4 per 10 high-power fields in the superficial portion to 16 per 10 high-power fields in the deeper portions. Eosinophilic cytoplasm with hyperchromatic and pleomorphic nuclei were seen too. The tumor showed large foci of tumoral necrosis (< 50%) and very limited extension to the hypodermis without vascular invasion . In immuAfter 3 years of regular follow-up, the patient was doing well with no clinical or radiological sign of recurrent or metastatic disease and then decided to abandon the routine follow up against medical advice. Six years postoperatively, he was admitted in our medical department of oncology with a six months history of a painless rapidly increasing mass of the left iliac fossa. There was no fever, transit disorder, vomiting or body weight loss. Physical examination discovered an 11cm mass of the left iliac fossa arising from the pelvis. The overlying skin was normal. No neurological deficits were detected. A keloid scar of 2cm was found on the right presternal region.Subsequent X-ray of the pelvis showed osteolytic lesions in the left iliac bone. Pelvis MRI depicted a tissular mass measuring 13cm x 13cm x 10 cm involving the psoas iliac and gluteal muscles until the iliac crest. A second tissular mass of 5 x 4 x 3cm was seen around the left ischio-pubic branch with hyperfixation in bone scintigraphy. Thoracoabdominopelvic computed tomography (TAP-CT) demonstrated bilateral lung metastases of 7mm to 45mm too. A biopsy of the pelvic mass was performed and led to diagnosis of leiomyosarcoma .th line therapy with metronomic oral cyclophosphamid.The case was discussed in a multidisciplinary consultation meeting indicating medical treatment be with doxorubicin and ifosfamide regimen. After 6 courses of chemotherapy, tumor response was compared according to the RECIST 1.1 criteria concluding to disease stabilization. A break from chemotherapy was allowed after discussion with the patient. During this period, he was followed up regularly and was evaluated by computed tomography scan every 3 months or when clinically indicated. The patient remained stable for almost 16 months until CT scans revealed significant disease progression. Then subsequent salvage second-line chemotherapy with high dose ifosfamide was given. Unfortunately, the patient showed rapid disease progression during the third course of treatment. Since performance status was maintained, he was eligible to receive third-line chemotherapy. Thus, disease stabilization in the following six months was achieved with gemcitabine monotherapy. Recently, patient’s performance status (PS) has deteriorated and further clinical progression was detected, so we began a 4th and 6th decade of life with a male preference [Primary Cutaneous Leiomyosarcomas (PCL) are highly-infrequent mesechymatal malignant neoplasm which are thought to arise from the arrector pili muscles in the dermis. They were described for the first time in 1959 by Montgomery and Winkleman , 3. PCL eference -6. Some eference . Our pateference -6. Localeference -10. Clineference . Overlyieference . Pain anAs clinical presentation may be non-specific, the diagnosis is on the basis of the histologic findings and immunohistochemical examination. It is usually made upon a skin biopsy. However, benign findings should not exclude the possibility of a cutaneous leiomyosarcoma . In our At present, there are no consensuses concerning the optimal treatment of PCL. Although there is evidence that wide local excision with negative lateral margins and depth including the subcutaneous tissue and fascia is the mainstay of treatment, the optimal excision margins for PCL remains controversial. As the recurrence rate and metastatic risk of PCL are strongly related to the adequacy of surgery, a large excision that includes 3-5 cm of uninvolved margins is strongly recommended , 14. OthAlthough our patient has shown numerous factors of bad prognosis including tumor size, subcutaneous involvement and numerous atypical mitotic figures, he didn’t undergo re-excision to obtain wider margins or receive adjuvant therapy. That may be partly due to the presumed benign course of PCL and no standardized treatment regimens. To date, effectiveness of radiotherapy in this setting has not been determined especially that it has been considered as a causative factor itself. Reports have shown that both radiotherapy and chemotherapy should be reserved for cases of recurrence or metastasis , 5. As tAlthough local recurrence is common occurring in up to 66.7% of the patients, its potential for distant metastases is negligent (0 - 14.3%). According to the very few cases being referred to the literature, lung was the most common site followed by skin , 18. TheBecause of its scarcity, there are no series to outline the management of metastatic PCL. Indeed, treatment options in metastatic setting are not dictated by the localization to the skin but by the histological subtype . ClassicPrimary CLM are a diagnostic and therapeutic challenge for clinicians. The present case displayed interesting clinical features of a rare case with an uncommon outcome of CLM and highlights the need for more proper standardized treatments.The authors declare no competing interests.


Myeloid sarcoma (MS) is an extremely rare disease, closely correlated to Acute Myeloid Leukamia (AML) and presenting as a tumoral lesion in potentially any anatomic location. It is seen either concomitant with AML, during remission, or more seldom, prior to any detectable haematological abnormality. While MS remains a difficult diagnosis, this rare tumor must be included in the differential diagnosis of atypical, local obstructive abdominal processes, especially when coinciding with a myeloproliferative disorder. We present a case report of an otherwise healthy young patient with small bowel obstruction due to an invasive ileal mass, histologically diagnosed as a myeloid sarcoma. Myeloid Sarcoma (MS) is an extremely rare tumoral lesion, with an infaust prognosis. It presents as an extra-medullary manifestation of a myeloproliferative disorder, strongly correlated with acute myeloid leukemia (AML). MS can occur at all ages, in any possible part of the body. Presentation and symptoms are therefore linked to its anatomical site. In our case, a patient, without previous haematological medical history, presented with an obstructive small bowel lesion, after resection histologically diagnosed as a myeloid sarcoma.A 46-year-old man with no relevant medical history presented at the emergency department with nausea and a vague epigastric abdominal pain. An initial ultrasound examination demonstrated an ileus of the small intestine with small bowel wall distention mainly in the peri-umbilical region.Computed tomography (CT) confirmed a large mesenteric tumoral mass extending towards the ileum, where circumferential small bowel wall invasion caused intestinal obstruction Figures  and 2. TThe patient was subsequently referred for surgery, revealing an obstructive tumoral lesion in the ileum and a mass in the adjacent mesentery Figure . There wThe pathology examination confirmed an ileum tumor five centimeters in length, invading all layers of the bowel wall and a second, mesenteric mass six centimeters in length. Two out of nine lymph nodes were positive. On histology, the resected mass consisted of atypical cells with a high mitotic activity and an increased nuclear-cytoplasmatic ratio. Immunohistologic staining showed a high Ki-67 expression and highly positive myeloid markers such as MPO, CD-43, CD-117 and Lysozyme  followed by adenocarcinoma’s (32.6%), lymphoma’s (14.7%), GIST’s (7.2%) and sarcoma’s (1.2%) [In contrast, a myeloid sarcoma, also referred to as granulocytic sarcoma or chloroma, is not a primary small bowel malignancy, but rather an extra-medullary manifestation of a myeloproliferative disorder. It is mainly associated with AML and to a lesser extent with chronic myeloid leukaemia, myeloid proliferative neoplasm and myelodysplatic disorder.MS occurs at all ages, yet 60% are found in pediatric patients , in cont34Imaging features on CT and MR are fairly non-specific, including variable morphology and enhancement, possibly mimicking abscess formation, meningioma or lymphoma 7. MultipMS is associated with a poor prognosis. Without chemotherapy all cases of isolated MS progress to AML within one year . A retroIn conclusion, a myeloid sarcoma is a rare extra-medullary manifestation of a myeloproliferative disorder and can cause abdominal obstruction when presenting as a mesenteric and/or small bowel mass. While it can precede an AML, it must be considered in the differential diagnosis of all patients presenting with a non-specific mass and a history of haematological malignancy.


Esthesioneuroblastoma (ENB) is a rare malignant tumor that commonly develops in the upper nasal cavity. Standard treatment is not established, especially in locally advanced disease which portends the worse prognosis. Hereby, we report a case of a 27-year-old, 23-week pregnant woman, with a 2-month history of progressively growing right cervical lymphadenopathy, nasal obstruction, anosmia, frequent episodes of epistaxis, and right frontal headache. Imagiological evaluation revealed a lesion with 7×5,2×3,2 cm in the nasal fossae with extension to the ethmoidal complex and right olfactive fend and invasion of the endocranial compartment associated with lymphadenopathy. The biopsy revealed a high-grade EBN. Neoadjuvant chemotherapy with cisplatin and etoposide was administrated during pregnancy and continued after delivery up to 6 cycles of treatment with partial response. Radiotherapy followed, with complete response. This case report is intended to highlight that a high grade of suspicion should be kept in the presence of nonspecific symptoms of nasal obstruction, anosmia, facial pain, and/or headache and focus that chemotherapy is an important component of a combined-treatment modality for locally advanced ENB that can be used during pregnancy in a lifesaving situation. Esthesioneuroblastoma (ENB), also referred to as olfactory neuroblastoma (ONB), is a malignant neuroectodermal tumor thought to originate from the olfactory membrane of the sinonasal tract . ENB is A 27-year-old 8-week pregnant woman, with a history of allergic rhinitis and atopic eczema, presented to a general hospital with right nasal obstruction, right cervical lymphadenopathy, and pain in the right superior dental arcade. A nonsteroidal anti-inflammatory drug was started with resolution of the dental pain after one week of treatment. Two months later, while maintaining a progressively growing right cervical lymphadenopathy and right nasal obstruction, the patient developed anosmia, frequent episodes of epistaxis, and right frontal headache. A fine needle biopsy of the lymphadenopathy was performed with an inconclusive result, revealing only the presence of inflammatory cells. A core biopsy was then performed which revealed lymph node metastasis from a poorly differentiated malignant neoplasm. At the 23rd week of pregnancy, the patient was referred to our hospital. On physical examination, the patient had a voluminous right cervical lymphadenopathy with 15 cm from levels Ib to V associated with cutaneous erythema as well as right ocular oedema . A veget2 on day 1 and etoposide 75 mg/m2 on days 1 to 3, cycles every 28 days, was started after an appropriate discussion with the patient's obstetrician. The following premedication before each treatment cycle was prescribed: hydrocortisone 100 mg, metoclopramide 10 mg, and ondansetron 8 mg. Additional treatment with daily folic acid, oral iron, iodine supplementation, and prophylactic enoxaparin was made as recommended by the obstetrician.The case was evaluated by a multidisciplinary team of head and neck surgeons, medical oncologists, and radiation oncologists. The multidisciplinary tumor board determined that there was no indication to perform surgery due to local extent of the disease. The patient was proposed to do systemic treatment with chemotherapy followed by reevaluation by the multidisciplinary tumor board. Treatment with cisplatin 75 mg/mAfter the first cycle of treatment, a clinical reduction of the lesion was noted . Concern2 on day 1 and etoposide 100 mg/m2 on days 1 to 3, cycles every 21 days. After 4 cycles of treatment, a positron emission tomography-computed tomography (PET-CT) and MRI were performed, revealing a partial response. The patient completed 6 cycles of treatment with good tolerance. The main toxicities reported during treatment were grade 1 anemia, grade 1 nausea, and emesis treated with oral iron, folic acid, and metoclopramide as needed.Fourteen days after the delivery, chemotherapy was resumed at a full dose with cisplatin 100 mg/mThe case was again discussed in a multidisciplinary tumor board, and treatment with radiotherapy (RT) was proposed. The patient performed 33 fractions of treatment with volumetric modulated arc therapy (VMAT) at a dose of 70 Gy to the neoplastic lesion, right retropharyngeal area, and right cervical Ib to V levels and 50 Gy to the left retropharyngeal area, left cervical Ib to V levels, and left perinasal area. The treatment had a duration of 45 days with good tolerance. The main toxicities were grade 2 dysphagia, grade 2 odynophagia, grade 2 xerostomia, grade 2 oral mucositis, and grade 2 cervical dermatitis.A period of clinical vigilance was started, and 12 weeks after the last treatment of radiotherapy, a PET-CT was performed revealing no radiopharmaceutical uptake. At the 12th month of follow-up, the baby is healthy and presents a normal development. The patient is clinically well presenting as treatment sequels a grade 1 diminution of visual acuity in the right eye, xerostomia grade 1, and cervical fibrosis grade 1.This clinical case demonstrates the challenge of diagnosis and management of a rare and aggressive type of esthesioneuroblastoma in a pregnant woman. ENB is an uncommon tumor that frequently presents with nonspecific symptoms such as nasal obstruction and facial pain. The diagnosis is a challenge for the clinicians due to the rarity and unspecificity of this entity. Moreover, while the histologic diagnosis of a well-differentiated tumor is quite evident, a less differentiated form can be more laborious requiring additional markers for the differential diagnosis among the group of small round cell malignant neoplasms that can affect the sinonasal tract. Olfactory neuroblastomas frequently express NSE and synaptophysin and are negative for the expression of leucocyte common antigen and CD99 . The entThe standard treatment for ENB has not been established. The lack of prospective data due to its rarity and the absence of molecular therapeutic targets are contributing factors , 11. Sur2 every 3 weeks [Exposure to some chemotherapeutic agents during the second and third trimesters has not been associated with increased risk of foetal malformations or major problems in the short and long terms –19, cont 3 weeks . There i 3 weeks  that the 3 weeks .Considering that it was a lifesaving situation for both the pregnant patient and her baby, the decision was to start treatment with chemotherapy with a regimen of cisplatin and etoposide that was administered during the 24th to 31st week of pregnancy. Furthermore, due to the high emetogenic potential of cisplatin-based regimens, it was essential to decide the pretreatment associated with each cycle of chemotherapy for prevention of chemotherapy-induced nausea and vomiting in a pregnant patient. Both the dopamine D2 antagonist metoclopramide and the serotonin 5-hydroxytryptamine type (5-HT3) receptor antagonist ondansetron can be used during pregnancy as there is no report of an increased risk of pregnancy-related events . In relaThere were no complications during the neonatal period and the 12th month period of follow-up for both the baby and the mother, although a longer period of follow-up is necessary to ascertain this good outcome. Due to the aggressive nature of the esthesioneuroblastoma, clinical evaluation is made on a monthly basis.This case highlights that chemotherapy is an important component of the multimodal treatment strategy of locally advanced esthesioneuroblastoma and shows that in a lifesaving situation, chemotherapy with cisplatin and etoposide can be administered in the 2nd and 3rd trimesters of pregnancy. Moreover, a prompt diagnosis is critical to achieve complete remission of the disease especially in the case of a biologically aggressive neoplasm. Prognosis of patients diagnosed with cancer during pregnancy is usually comparable with nonpregnant patients ; howeverThis clinical case should be looked as a contribution to future similar cases as it is a unique illustration of a rare and aggressive esthesioneuroblastoma in a pregnant woman.


Heterotopic pancreas, also known as ectopic pancreas, is pancreatic tissue located outside the pancreatic parenchyma without vascular or ductal communication with the gland. Ectopic pancreas is rarely symptomatic, typically detected incidentally at surgery or autopsy. Eighty-five to 90% are in the upper GI tract, especially the gastric antrum. We report a 54-year-old man with symptomatic gastric heterotopic pancreas presenting as recurrent, initially undiagnosed, abdominal pain. Surgery revealed heterotopic pancreas including excretory ducts, acini, and islet cells. Evidence of acute pancreatitis was present, marked by inflammation and abscess formation. Chronic pancreatitis was diagnosed by fibrosis and dilated ducts containing proteinaceous material. Submucosal location with normal overlying mucosa on endoscopy increases risks of delayed or missed diagnosis. Complications include GI bleeding, acute or chronic pancreatitis, pancreatic necrosis, pseudocyst, gastric outlet obstruction, perforation, and, rarely, pancreatic carcinoma. This rare disorder mimics more common diseases. Low suspicion, nondiagnostic imaging or endoscopy contribute to frequent diagnostic delay. Ectopic pancreas is rarely symptomatic. The vast majority of lesions are detected incidentally at surgery or autopsy. Barbosa and colleagues reported approximately 1 incidental case detected per 500 upper abdominal operations; the incidence for all autopsies was 1.7% . Other sWe report a rare case of symptomatic gastric heterotopic pancreas presenting as nonspecific, recurrent abdominal pain with a characteristic submucosal gastric mass on endoscopy. This case is exceptional because the ectopic tissue contained evidence of acute and chronic pancreatitis and abscess formation.A 54-year-old man was admitted with 4-5 days of abdominal pain that began in the left upper quadrant and then migrated subumbilically. The pain was sharp, steady, and of moderate severity. He also described nausea and a low-grade fever. Past medical history included kidney stones and a sigmoid colectomy for diverticulitis 15 years previously. He denied a history of excess alcohol use. His only medication was atenolol 50 mg daily.On admission, physical examination revealed normal sinus rhythm, normal pulse and blood pressure without postural change, normal temperature, moderate tenderness to palpation in the left supraumbilical and subxiphoid areas without peritonitis, organomegaly, or mass lesion. Stool was negative for occult blood.3/μL, Hgb: 16g/dL, Chemistry 7, liver enzymes, and serum amylase and lipase were within normal limits. Upper GI endoscopy was interpreted as a 5 mm sessile gastric antral polyp with normal gastric mucosa on biopsy. Abdominal CT scan showed mesenteric inflammation surrounding the distal body and proximal antrum of the stomach and adjacent low-density thickening of the stomach wall, measuring up to 18 mm in thickness. He was treated with IV fluids and pain medication. His condition improved and he was discharged home without a definitive diagnosis.Lab results: WBC: 10 x 10He felt well for four months and then developed recurrent mild, diffuse, sharp, steady abdominal pain. He denied weight loss, nausea, vomiting, or bowel complaints. Physical examination showed normal vital signs, afebrile. His abdomen was soft, nontender, and otherwise unremarkable. Basic laboratory studies, serum amylase, and lipase were normal. Repeat upper GI endoscopy showed a firm submucosal mass with intact overlying mucosa and a central umbilication. Biopsy of the lesion revealed normal gastric mucosa . AbdominFindings were nondiagnostic, but inconsistent with leiomyoma or leiomyosarcoma.Open gastric antrectomy with a Billroth I technique was performed for a preoperative diagnosis of gastric adenocarcinoma. Histology of the resected specimen revealed ectopic pancreatic tissue, including excretory ducts, acini, and islet cells within the gastric muscularis layer . EvidencHeterotopic pancreas, also known as ectopic, accessory, or aberrant pancreas, or pancreatic rest, is defined as pancreatic tissue outside the normal pancreatic parenchyma in an aberrant location with a vascular and nerve supply separate from the pancreas itself . It is tAmong patients with symptomatic gastric heterotopic pancreas, the most commonly reported symptom is mid-abdominal pain, occurring in as many as three-quarters of cases , 8. NausReported complications of gastric ectopic pancreas include acute or chronic pancreatitis , 5, 7, pDelayed or missed diagnosis is frequent , 10. FacUpper GI endoscopy and CT scanning are the most useful techniques to evaluate the majority of patients. The diagnosis of a submucosal mass lesion in the upper gastrointestinal tract with normal overlying mucosa on endoscopy can be divided into benign and malignant etiologies. Benign lesions include leiomyoma, lipoma, varices, neural tumors , granular cell tumor, inflammatory fibroid polyp, duplication cyst, lymphangioma, Brunner's gland hyperplasia, benign GIST, and heterotopic pancreas. Malignant lesions that should be considered are malignant GIST, carcinoid tumor, lymphoma, glomus tumor, and gastric or metastatic carcinoma  , 9.Endoscopic ultrasound (EUS) is the most accurate study to differentiate submucosal lesions not diagnosed by conventional endoscopy. Typical findings in heterotopic pancreas are a hypoechoic lesion with indistinct borders . UncommoHeterotopic pancreas may contain any of the components of normal pancreatic tissue, which consists of acini, ducts, and islets of Langerhans. In the case series of 32 patients reported by Ormarsson and colleagues, all cases contained ducts, 97% contained acini, and 41% contained islets . ClinicaThe diagnosis of symptomatic gastric heterotopic pancreatitis is challenging. This disorder, rarely detected ante-mortem, should be considered in selected patients with submucosal gastric mass lesions. We describe a patient with histologic confirmation of gastric ectopic pancreatic tissue including excretory ducts, acini, and islet cells within the gastric muscularis layer. Histologic acute and chronic pancreatitis with fibrosis, dilated ducts containing proteinaceous material and abscess formation, as in our patient, is exceptional. This disorder commonly presents with nonspecific symptoms, mimicking more common diseases. Nondiagnostic imaging and endoscopic findings are common, contributing to delay in diagnosis.


Hepatic artery aneurysm (HAA) is the common visceral aneurysms with the highest reported rate of rupture. The clinical manifestations depending on the size of the aneurysm include epigastric pain, obstruction of biliary tract, rupture and death. Imaging modalities like computed tomography (CT) scan and CT-angiography have a valuable role in the early detection of HHAs. Complications and selecting appropriate treatments depending on the size and location of the aneurysms. This article aimed to report clinical presentation, imaging finding and treatment of some patients presenting with HAAs to emergency department. Hepatic artery aneurysm (HAA) is a rare disease 0.002%–0.4%) but is a clinically important phenomenon . HAAs ar%–0.4% buCase 1The first case was a 50-year-old man with acute-onset epigastric and right upper abdominal pain. Abdominal ultrasonography demonstrated free abdominal fluid with internal clots. CT angiography was performed, which revealed a ruptured proper HAA with hemo-peritoneum in perihepatic space, para-colic gutters, and massive abdominopelvic hematoma. A simultaneous visceral aneurysm was also detected at the origin of the left gastric artery. The exploratory laparotomy revealed 2.5 liters of hemo-peritoneum, a ruptured aneurysmal sac in proximal of the left branch of proper hepatic artery with surrounding clots and intact liver parenchyma. Ligation in the proximal and distal parts of artery was done .Case 2The second patient was an 11-year-old boy with history of falling and blunt trauma to the flank and formation of liver hematoma. Three weeks later, he was referred to emergency department with the chief complaint of abdominal pain. After proving pseudo-HAA in contrast-enhanced computed tomography (CECT) scan, the patient was referred for catheter base angiography and treated with coiled embolization .Case 3th post-operative day hospitalization, the patient referred to the hospital a week later with severe abdominal pain and vomiting. In CT scan, there was evidence of acute necrotizing pancreatitis, along with collection in liver. The patient was successfully managed with conservative treatment and discharged after two weeks without major complication  showed HAA. The patient was treated successfully with coiled embolization .Case 5The patient was a 24-year-old male with history of gunshot traumatization and surgery due to hepatic artery aneurysm and liver hematoma. He was referred due to re-bleeding and large liver-infected hematoma. The patient underwent successful coil embolization similar to case 2.True HAAs are mostly due to degenerative or dysplastic change of the extrahepatic vessels. Their main cause is atherosclerosis , althougThe clinical manifestations tend to be non-specific, depending on the size of the aneurysm. Although small HAAs are often asymptomatic, the natural history is for progressive enlargement with increasing risk of rupture and death. HAAs may cause right upper quadrant and epigastric pain or obstruction of biliary tract. The classic triad of Quinke's, i.e., obstructive jaundice, abdominal pain and hemobilia, is seen in 30% of the patients . Inflammation associated with septic emboli may erode the arterial wall and then prompt HAA rupture, causing hemobilia . The preMultiple diagnosis tests are used, such as abdominal ultrasound, CT, CT angiography, MRI, endoscopy and angiography. Angiography is a therapeutic modality of choice in splanchnic aneurysms through embolization . The senThe size and location of the aneurysm, patient age and comorbidities have critical role in determining the specific approach . Trans-aVisceral aneurysms such as hepatic artery aneurysm should be considered as a rare but important differential diagnosis of acute abdominal pain. All emergency physicians and surgeons should kept this diagnosis n their mind to can prevent from its life threatening complications.


Teaching point: In the context of malignancy with lytic bone lesions, strongly hypoechoic liver nodules should alert for the possible diagnosis of extramedullary multiple myeloma. An 81-year-old man was found to have multiple bone lesions on a thoracic computed tomography (CT) in the context of pneumonia. The patient only reported a mild weight loss and ordinary low back pain. His physical examination was unremarkable. Routine laboratory tests results were normal.Abdominal contrast-enhanced computed tomography (CECT) showed disseminated osteolytic bone lesions and two barely visible hypoattenuating liver nodules  of the hepato-biliary tract confirmed two homogeneous strongly hypoechoic liver nodules, measuring 20 and 12 mm in diameter. The lesions’ margins were well-defined, with neither hypoechoic halo sign, nor posterior acoustic enhancement or shadowing . The immunochemistry staining of kappa light chain was positive Figure .To illustrate the imaging characteristics of the bone lesions, a magnetic resonance imaging (MRI) was realized and confirmed malignant marrow-replacing lesions, especially on the spine  is the most common primary bone malignancy among adults. Soft tissue involvement of MM is uncommon upon diagnosis and is referred to as extramedullary myeloma (EM) . There aDiffuse plasma cell infiltration of the liver, usually reported as autopsy findings in 40% of patients dying with MM and radiologically silent.Nodular liver presentation is a rare condition with an incidence of 0.35%. Only 27 cases have been reported so far in the scientific literature. The lesions are often described as very hypoechoic on US and hypoattenuating on CT. This was the case for our patient. This presentation usually indicates advanced stage of the disease with aggressive clinical course and poor prognosis.The approach to a patient with strongly hypoechoic liver nodules remains challenging. The differential diagnosis is broad and includes: benign focal liver lesions, hepatocellular or cholangiocellular carcinomas, metastatic carcinomas, metastatic endocrine tumors, and lymphoma. Although uncommon, EM should be included in the differential diagnosis, especially in the context of associated lytic bone lesions.


Post-traumatic diaphragmatic hernias are not commonly diagnosed immediately after the initial trauma. There is a greater prevalence of left hernias due to the fragility or injury in diaphragm muscle and the lack of solid and fixed structures on the left side.A male patient, 30 years old, he was admitted to the emergency department presenting diffuse abdominal pain, vomiting, dyspnea, pain in the left hemithorax with worsening during forced inspiration. After performing X-ray and computed tomography (CT), the presence of organs in the abdominal cavity outside the usual position was evidenced and with discrete deviation. Immediate surgery was performed with posterolateral thoracic access through the sixth left intercostal space combined with left subcostal access. Initially, it was found jejunum, ileum and left colonic flexure and accessory spleen filling the hernial sac.This report is the first case to report two accessory spleens in manual reduction of herniation between thoracic and abdominal cavities after trauma and percutaneous perforation. The splenectomy performed in both organs occurred due to their advanced ischemia that was due to reduced vascularity.The presence of the reported accessory spleen inside the thoracic cavity is only a possible variation within the possibilities in cases of diaphragmatic hernias, which does not modify the surgical procedure in a relevant way. Acute symptoms are uncommon, and they usually appear over time. It happens because of the negative pressure in the thoracic cavity that increases the initial injury on the diaphragm and the volume of visceral content in the thoracic cavity. This initial difficulty in the diagnosis generates serious complications that can occur through indeterminate and variable time ,2. TheseThere is a greater prevalence of left hernias due to the fragility or injury in diaphragm muscle and the lack of solid and fixed structures on the left side. Both perforating and blunt trauma can cause injury. However, there is a tendency of having greater caution in cases of blunt trauma of high impact due to the kind of the trauma (mainly traffic accidents) and the initial difficulty of diagnosing diaphragmatic hernia ,5. The mComputed tomography in 3D, scintigraphy, and magnetic resonance help to identify specific organs with more accuracy. However, they should not delay the conduct in cases of urgency and are not available at all health centers. In addition of all of these factors, there are the presence of several abdominal visceral organs that may be the contents of the hernial sac, depending on the location of the trauma, such as spleen, splenic flexure, jejunum, ileum, liver (right hernia) which can lead to various complications such as pneumonia, enteric ischemia, twisting of the hilum  and it's perforation. Laceration of organs is mostly from perforating cause . This wo2A 30 years old male patient, born and raised in the countryside of Amazonas State, was admitted to the emergency department in Manaus city. The distance between the emergency department and its location was 40 km. He had constipation for two days, diffuse abdominal pain, vomiting, dyspnea, pain in the left hemithorax with worsening during forced inspiration. He denied other clinical complaints and associated clinical comorbidities or the use of daily medications. He just reported a prior emergency surgery from nine years ago due to a stab injury in his left flank. This first trauma occurred due to perforation by steel rebar at left thoracoabdominal region, evolving with hemopneumothorax. Only chest drainage was performed at the time, as there were no signs of abdominal cavity injury or other complaints, according to the patient, and presented discharge with improvement five days later. He did not present social and demographic data worth mentioning. At the examination, the patient presented good general condition, lucid and oriented in time and space, pulmonary auscultation with reduction of vesicular murmur in the middle and basis of left hemithorax, with tympanic percussion, respiratory rate 15irpm.Abdomen was globose, distended, diminished breath sounds, pain to superficial and deep palpation diffusely, with higher intensity in deep palpation in left hypochondrium, percussion without alterations. After pain control with venous analgesia, chest X-ray and thorax/abdomen computed tomography were performed with evidence of abdominal material filling the left thoracic cavity on its middle portion, elevation of gastric bulge height with a contralateral deviation of the mediastinum and without evidence of pneumothorax or atelectasis. The tomography showed the hernia content, with the presence of splenic flexure of the colon, stomach, omentum, spleen and organs in the abdominal cavity outside the usual position with discrete deviation .Fig. 1CTImmediate surgery was performed with posterolateral thoracic access on the sixth intercostal space on the left side. It was initially found jejunum and ileum filling the chest cavity, bowel with adequate blood flow, good viability, no perforations or adhesions in pleura , Fig. 3.Lysis of adhesion herniary sac was realized and continued to decrease through the same thoracoabdominal wound. However, it was unsuccessful after consecutive attempts because of the large volume of the contents. It was chosen a concomitant left subcostal laparotomy incision to reduce the bowel to obtain a total success in reducing all hernia contents to the abdominal cavity , Fig. 5.3Within the natural course of hernia, the presented case was in chronic phase with association of intestinal obstruction and ischemic symptoms. They are classic signs of progression in the presence of a hidden lesion in the diaphragm. Because of the extent of the injury, the symptoms themselves were not cleared. The presented case is the first to report two accessory spleens in manual reduction of herniation between thoracic and abdominal cavities after trauma and percutaneous perforation.In the literature, there is only one case accessory spleen in Bochdalek Syndrome and other incidental findings in asymptomatic diaphragmatic hernia unrelated to previous trauma ,10. The In the reported case, the reason for using thoracotomy to undergo laparotomy was the initial difficulty in reducing the visceral organs to the original cavity. Using two surgical approaches was better because of control of the positioning of the organs and intraoperative visualization. Some literature considers the use of one incision the ideal due to the minor trauma caused by the surgery and for the shorter hospitalization time . HoweverDiaphragm closure can also be associated with fenestrated polypropylene mesh to avoid relapses although this method of repair is referred to be the best result of techniques for correction of abdominal wall hernias, not diaphragmatic lesions ,15. Ther4The presence of the reported accessory spleen is only a possible variation within the possibilities in cases of diaphragmatic hernias, which does not modify the surgical procedure in a relevant way. Regarding the difficulty in reducing content only with thoracotomy, studies should be conducted to compare the possible techniques, indications and complications. We currently have only the consensus that thoracotomy is indicated when the hernia is late and laparotomy is most appropriate immediately after trauma, due to intraoperative characteristics and their technical difficulties. The studies should formalize systematic care and easy access to other professionals, especially to newly trained or resident surgeons.We do not have any funding source, this manuscript is just a case report, not a research.As the manuscript is not a research study, we only have the patient consent for writing and others forms of publication. Also, the ethical approval for this case reports has been exempted by our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Marineide Melo, Jade Saldanha and Juan Rodriguez made contributions to conception and design. collected the patient details and wrote the paper. Ketlen Sousa, Rafaelle Silva made contributions to patient management. Izabela Medeiros and Renato Souza critically revised the article. All authors read and approved the final manuscript.The manuscript is a case report, not considered a formal research involving participants.Dra. Ketlen Gomes.Not commissioned, externally peer-reviewed.We do not have any conflicts of interests.


Mature teratoma of the mediastinum poses a significant surgical challenge due to close vicinity to vital structures causing respiratory insufficiency or hemodynamic compromise. While the malignant variety of germ cell tumors (GCT) generally present with florid symptoms, benign teratomas are detected incidentally on imaging. Large teratomas presenting as mediastinal mass syndrome have additional difficulty in airway access. Herein, we report a case of a 40-year-old-female with no significant comorbidities presenting with rapidly progressing symptoms of chest pain, dyspnea, and superior vena cava (SVC) compression. Computed tomography (CT) scan of the neck and chest confirmed a large cystic lesion with marked compression of the great veins in the neck, arch of aorta, trachea, and proximal bronchial divisions. Airway access intraoperatively was done by awake fiberoptic bronchoscopy. However, sudden hypoxia and hemodynamic deterioration warranted emergency sternotomy. Adequate preoperative preparation, as well as standby extracorporeal circulatory support, led to successful and complete excision of the tumor. The patient had an uneventful recovery and extubated in the intensive care unit (ICU) the next day. Histopathology of the mass confirmed to be mature benign cystic teratoma. At six-month follow-up, the patient was completely asymptomatic without any complications. The impact of intraoperative catastrophe on the healthcare team can be immense. Inability to achieve a secure airway and the resultant hypoxia can result in permanent neurological damage. A multidisciplinary approach leading to adequate preoperative assessment, intraoperative preparedness for an emergency sternotomy with standby extracorporeal circulatory support due to the risk of mediastinal mass syndrome, were key features in the successful management of the patient. Approximately 5% to 10% of all germ cell tumors (GCT) arise in non-gonadal sites, particularly in the mediastinum and retroperitoneum -4. Rare A 40-year-old female, previously healthy, presented to the outpatient clinic with intermittent cough without expectoration, breathlessness, difficulty in swallowing and facial puffiness for the last two to three months with gradual worsening over the past one month. The symptoms aggravated on lying in a supine position. Her past medical history was significant for hypertension and was on atenolol. On physical examination, her vitals were stable in the sitting position; it also revealed facial puffiness and distended neck veins. All her labs were within normal limit. Chest X-ray showed mediastinal widening. On contrast-enhanced computed tomography (CECT) of the chest, a large cystic lesion of size 14.4 x 10 x 8.6 cm was seen in the anterior mediastinum with marked compression of the brachiocephalic confluence, SVC, arch of aorta, proximal vessels of the neck and trachea, and proximal bronchial divisions Figures -1C.CT guided fine needle aspiration biopsy (FNAB) showed cheesy fluid aspirate with no atypical cells. A decision for tumor excision via a median sternotomy approach was planned. A thorough preoperative planning with regards to surgical access, use of intraoperative bronchoscopy, and patient positioning were done. Anticipating cardiorespiratory compromise as an intraoperative complication, preparation for extracorporeal circulation using femoral cannulation was kept ready. Large bore venous access was placed in the lower extremity, spontaneous ventilation was maintained, and fiberoptic bronchoscopy was performed to visualize the airway for any compression and facilitate intubation. However, after induction, the saturation suddenly dropped to low 50s. The cyst was aspirated from the left second intercostal space as an emergent measure, the neck was flexed, and head end elevated 30 degrees to facilitate ventilation. Emergency sternotomy was done and the tumor was totally resected thus relieving the compression Figures -2B.Mediastinal and pleural chest tubes were placed for drainage. The patient was extubated in the operating room after the surgery and observed in the intensive care unit (ICU). The post-operative stay was uncomplicated. The mediastinal and left pleural drains were removed on post-operative day six and seven, respectively; the patient was discharged on day eight. Final histopathology report revealed a benign cystic teratoma, grossly showing multiple solid and cystic areas with bone cartilage and skin with hair follicle. Microscopic examination showed mature epidermis, adnexal elements, bone and cartilage Figures -3B. At sGCT is typically derived from ectoderm, endoderm, and mesoderm germ cells ,3,6. TheAs seen in Figure Preoperative management includes bed rest, elevation of the head end and oxygen administration . IntraveIndividuals with SVC syndrome may not be able to lie comfortably in a supine position for an extended period because of increased intracerebral venous pressure. As with all thoracic surgeries, proper positioning of the patient is of paramount importance. Tumors or cysts located in the anterior mediastinum are generally approached through median sternotomy approach via standard single-lumen endotracheal intubation. Double-lumen endotracheal tube for single-lung ventilation is reserved for thoracotomy incision and for all procedures performed using video-assisted thoracoscopic surgery (VATS). Additional exposure includes a hemiclamshell thoracotomy, which may be preferred for tumors in the anterior mediastinum with extensive involvement of the hemi-thorax -18. A neTumors that extend to adjacent structures necessitate resection of the thymus, pericardium, part of the lung, phrenic nerve, innominate vein as also the reconstruction of SVC. Post-operative care is like that for any routine thoracic surgery. Extubation can be performed after the procedure or in the post-anesthesia recovery area. Patients requiring prolonged ventilatory support should have pulmonary toilet to prevent atelectasis and clear any bronchial secretions. Patient-controlled analgesia (PCA) is one of the widely used methods of pain management. Thoracic epidural analgesia can provide good pain relief and reduce the need for postoperative analgesia .Sternal wound complications although rare, are significantly elevated in emergency cases. Heilmann et al., in their multivariate analysis, revealed emergency sternotomy as an independent prognostic factor for all sternal wound complications (odds ratio (OR) 3.5, 95% confidence interval (CI), 2.0-6.3; p<0.001) . When drPrognosis after resection of a mediastinal tumor varies widely depending on the type of lesion resected. After resection of mediastinal cysts and benign tumors, prognosis generally is excellent .Complete surgical removal of a mediastinal teratoma is the treatment of choice, as it establishes the diagnosis, besides preventing life-threatening complications in many patients. As most mediastinal teratomas are benign, even a subtotal resection conserving adherent vital structures provides excellent long-term results. In the present era of modern surgical practices, excellent outcomes have become the rule. The interesting feature of our patient was an atypical presentation with rapidly developing symptoms despite the benign nature of the tumor. The anticipation of cardiorespiratory compromise during anesthesia induction, appropriate preparedness, and prompt responsiveness in case of any compromise as performed in this case, is helpful to prevent catastrophic events.


Uterine fibroids are the commonest benign gynecological tumors. Laparoscopic myomectomy is becoming increasingly popular as one of the surgical treatment options for symptomatic cases. Large tissues such as leiomyomas or even the uterus need to be morcellated in order to be retrieved from the abdominal cavity. Some of the morcellated fragments or small fibroids may be accidentally left in the abdominal cavity during the retrieval process. These may subsequently become implanted in the abdominal cavity, develop blood supply from the surrounding structures, and grow to form parasitic myomas with varied clinical presentation, depending on the location and size.A 47-year-old African woman presented to our hospital 6 years after laparoscopic myomectomy with a lower abdominal mass. Her work-up revealed an anterior abdominal wall mass consistent with uterine leiomyoma. She was scheduled for excision of the mass, which was subsequently histologically confirmed to be a uterine fibroid.Parasitic leiomyomas are a rare late complication of power morcellation following laparoscopic myomectomy or hysterectomy. Most patients present with an abdominal/pelvic mass and may need surgical excision to relieve the symptoms. Care should be taken during power morcellation to prevent excessive fragmentation of the tissues, some of which may become implanted and persist to form parasitic myomas. Moreover, effort should be made to retrieve all myoma fragments by carefully checking the abdominal cavity. Whenever possible, the morcellation should be done in a containment bag. Uterine fibroids are the most common benign gynecological tumors of the female genital tract. Despite their relatively high prevalence, clinically apparent in up to 40% of women over 40 years of age, the majority of the patients with uterine fibroids are asymptomatic , 2. VariThe increasing availability of power morcellators has made it possible for large leiomyomas to be removed laparoscopically with the benefit of decreased blood loss, shorter hospital stay, and faster recovery time , 4, 5. OA 47-year-old, para 3 + 0, of African descent presented with a lower abdominal mass that had been progressively increasing over a 3-year period. The mass was on her left lower abdomen and was not painful. Six years earlier, she had undergone laparoscopic myomectomy with power morcellation for myoma retrieval.She also reported persistent menorrhagia and dysmenorrhea since the previous surgery, for which she was mainly receiving medical therapies. These included tranexamic acid and mefenamic acid with the onset of menses, which provided good symptom relief. She reported no symptoms of anemia, such as easy fatigability, palpitations, or dyspnea, and had not used any hematinics prior to the current presentation. Her Papanicolaou smears for cervical cancer screening were up-to-date with normal cytology.All her three deliveries were through cesarean section, two before and one after the initial surgery. She had no urinary symptoms such as frequency of micturition, dysuria, or incomplete voiding. In addition, she had no bowel symptoms such as constipation.The examination revealed an anterior abdominal wall mass in the left iliac fossa. The mass was firm, mobile, and nontender overlying the previous left laparoscopy port site Fig. . This wa9/L, and platelet count of 261,000 × 109/L.Her preoperative work-up included a pelvic ultrasound examination, which revealed multiple intramural fibroids with a superficial left hypoechoic mass in the left iliac fossa region measuring 4 cm by 3 cm. Abdominopelvic magnetic resonance imaging (MRI) revealed an anterior abdominal mass measuring 7.2 cm by 5.1 cm by 3.4 cm on the anterior abdominal wall in the left iliac fossa (Fig. Management options were discussed, and the patient opted to have a total abdominal hysterectomy and excision of the abdominal mass. The hysterectomy was done through the previous cesarean section scar site. A separate 5-cm incision was made over the left iliac fossa via the previous 12-mm port site scar, and dissection was done to retrieve a 7.5-cm by 5.5-cm by 3.5-cm parasitic myoma Figs. . The parLaparoscopic myomectomy and hysterectomy are increasingly becoming more available. To aid in tissue removal, large tissues such as the uterus and leiomyomas are often fragmented with the use of power morcellators. Some of the resultant small fragments may inadvertently remain in the abdominal cavity and become implanted with development of blood supply, resulting in subsequent growth –9. TheseThe clinical presentation is quite nonspecific and depends on the site of recurrence. The common sites include the pelvic cavity/wall, small intestines, rectum, cecum, vaginal/cervical stump, and laparoscopic port site , 7, 10. Given the nonspecific presentation, there is no specific investigation that is ideal in the diagnosis of parasitic myomas. The definitive diagnosis requires confirmation at histology, given the tendency to find the lesions distant from the uterus. However, histology of a benign smooth muscle tumor distant from the uterus in a patient with a history of laparoscopic myomectomy with morcellation should increase the suspicion of a parasitic leiomyoma . This isThe initial investigation, just as in cases of a patient presenting with a pelvic mass, should therefore include a pelvic ultrasound. MRI may further help in distinguishing benign leiomyomas from other solid pelvic and abdominal tumors . The nonMeasurement of tumor markers such as CA 125, α-fetoprotein, or CA19-9 may be done, though they may be falsely elevated with occasional ascites . Our patTreatment involves excision of the parasitic myoma. This would be achieved through laparoscopy with good outcomes or through laparotomy, especially in cases of large myomas or suspected malignancy. In our patient, excision was performed through a laparotomy. Whenever possible, a multidisciplinary approach should be used, especially when the parasitic myoma is near other organs . This waTo reduce the risk of developing parasitic leiomyomas following power morcellation, various alternatives have been suggested , 9. The Parasitic leiomyomas are a rare late complication of power morcellation following laparoscopic myomectomy or hysterectomy. Most patients present with an abdominal/pelvic mass and may need surgical excision to relieve the symptoms. Care should be taken during power morcellation to prevent excessive fragmentation of the tissues, some of which may become implanted and persist to form parasitic myomas. Moreover, effort should be made to retrieve all myoma fragments by carefully checking the abdominal cavity. Whenever possible, the morcellation should be done in a containment bag.


Renal angiomyolipoma  is a benign Tumor, composed of adipose tissue, smooth muscle tissue, and blood vessels. Tumors more than 10 cm (referred to as “giant” AMLs) are infrequent and a very small number of reported cases were measured more than 20 cm. Epithelioid angiomyolipoma (EAML), is a rare variant of angiomyolipoma with malignant potential. There are less than 10 cases of huge EAML reported in English literature, that were all associated with recurrence and complications.Here we report a rare case of renal EAML in a 48-year-old male patient that was not associated with tuberous sclerosis. He was admitted to our hospital with a complaint of severe abdominal pain. Computer Tomography Scan (CT-scan), revealed two masses attached to the left kidney with a size of 32 × 22 × 8 cm and 20 × 10 × 3 cm. The patient underwent a radical left nephrectomy. Postoperative histopathological examination confirmed the diagnosis of an EAML. The two-year follow-up CT-scan showed no recurrence.Malignant EAML, although rare, does exist. Thus, besides evaluations required for ruling out the diagnosis of renal sarcomas, surgeons must follow patients after total nephrectomy for possible recurrence. Benign cases can be completely cured by surgery and do not show recurrence at the end of the follow-up period. Epidemiological studies revealed that AML accounts for about 1 % of all resectable renal masses [The incidence rate of AML is about 0.3 %–3.0 %. Tumors more than 10 cm (referred to as “giant” AMLs) are rare. Only a few reported cases, had giant tumors larger than 20 cm.Angiomyolipoma is either find sporadically  or in association with other conditions (such as Tuberous Sclerosis Complex Associated AML or TSCAML) . The assImmunohistochemically, AML is characterized by co-expression of melanocytic markers (HMB-45 and Mart-1/Melan A) and myoid markers (SMA and muscle-specific actin). Despite its benign behavior, it can invade the renal vascular system and involve the lymph node [World Health Organization (WHO) has classified renal AML into two categories; Classical AML and Epithelioid Angiomyolipoma (EAML). The Classical AML is a benign mass composed of variable amounts of triphasic histology, a variable amount of adipose tissue, spindle and epithelioid smooth muscle tissue originating from perivascular epithelial cells and abnormal thick-walled blood vessels, despite the presence of focal nuclear pleomorphism and mitotic activity in few cases .AML was clinically considered a benign lesion since recurrence and metastasis barely occurred in patients after surgical treatment. Nevertheless, EAML is a rare variant of AML, composed of epithelioid cells and scarce adipose tissue. EAML has been reported to pursue a more aggressive clinical course .EAML is a subtype of perivascular epithelioid cell tumors (PEComas) with malignant potential. Around 160 cases of EAML are reported in the English literature until 2018 [In contrast to classic AML, EAML is a potentially malignant mesenchymal neoplasm withpossible lymph node metastasis and distant metastasis .EAMLs are mostly found in the kidney. They can also involve other organs including the liver, lungs, pancreas, bladder, prostate, uterus, ovary, vulva, vagina, and bone with lower incidence .EAMLs are mainly composed of the human melanoma black (HMB)- 45 epithelioid cells arranged in sheets and lack adipocytes . Based oSince the ﬁrst description of EAML, many case reports and small series of this entity have been published. Although it is now a well-known entity, morphological criteria to differentiate EAML from renal epithelial neoplasms are not well deﬁned. EAML has no specific clinical or radiological feature, therefore the diagnosis of EAML is mostly based on pathological findings. Thus performing a biopsy and surgical resection is considered as the gold standard of diagnosis and treatment ,11.Surgical resection is the treatment of choice for the management of EAML. However, advances in minimally-invasive therapies and novel targeted chemotherapeutics have increased the options for the management of AML .Here we report a rare case of giant renal EAML in a male Iranian patient who was diagnosed at an academic institution in Tehran. This case was benign and not associated with TSC. This project has been reported in line with the SCARE criteria .2Here we report a 48-year-old male case of EAML of the kidney which is a relatively newly described renal neoplasm and is closely related to the more common classic AML of the kidney and perivascular epithelioid cell neoplasm of extra-renal sites .The ethical committee of our institute gave us permission for the current publication. The patient gave informed consent and is aware of this case report.The patient arrived at our institute, complaining of severe pleuritic chest pain for approximately three months. He was not a smoker and did not consume any illicit drug or alcohol. He had never experienced urinary problems. His past medical history was unremarkable. He had no history of previous surgery.On physical examination, a huge abdominal mass was palpated in the left flank. Incredibly, he did not feel this enormous mass earlier which may suggest the rapid growth of the lesion.There were no abnormal findings in laboratory studies  except for a slightly elevated Erythrocyte Sedimentation Rate (ESR). Renal and liver function tests were within normal limits. He was admitted for further diagnostic evaluations. The chest Computed Tomography scan (CT-scan) revealed a massive pleural effusion in the left hemithorax. Abdominopelvic CT-scan showed two infrarenal heterogeneous masses arising from the medial pole of the left kidney with significantly increased vascularity. Both masses contained adipose tissue, which was suggestive of either AML or a well-differentiated liposarcoma .Fig. 1CoPurified Protein Derivatives (PPD) skin test and cytopathologic analysis of pleural fluid were done in order to rule out tuberculosis and malignancy respectively. The aspirated fluid only contained inflammatory materials.Then, he underwent a radical left nephrectomy which is the treatment of choice for huge AML of the kidney based on the literature. Surgery was performed by an experienced urologist, under general anesthesia, and via a midline incision. After exploring the abdominal cavity, two huge masses attached to the middle pole of the left kidney were observed . MassiveThe specimen was sent for histopathologic evaluations. The larger mass contained necrotic tissue and atypical epithelioid cells with the mitotic rate of 0–1 IN 10 HPF. The atypical mitotic figure was not identified. These findings completed the gross characteristics to confirm the diagnosis of EAML, possibly associated with tuberous sclerosis. Fig. 3 which presents with multi-system progressive tumors, intractable epilepsy, and mental retardation, was ruled out. He had no skin lesion suggestive of sclerotic changes or a history of neurological manifestations of TSCs.No specific concurrent treatment was applied for him except antibiotic (1 g of Cefazolin every 6 h) and analgesia (when required) for two weeks postoperatively. He was under our close observation during his stay at the hospital. Renal Function Test and Complete blood count were evaluated every other month. His abdominal pain was completely relieved after the surgery. At a two-year follow-up by CT-scan. There has been neither metastasis nor recurrence during the 24-month period since resection .Fig. 4Fo3EAML in contrast to the classic AML (which is benign), may be associated with distant metastasis and local recurrence. Malignant behavior and metastasis to lymph nodes, liver, lungs or spine has been reported in approximately one-third of cases of EAML .Over a decade, the diagnosis and management of renal AMLs have been discussed in the literature. The deﬁnitive diagnosis of primary renal EAML needs both histochemical and pathological evaluations since there are no definite clinicopathological criteria .Radiologic characteristics of EAML cannot help in making a definite diagnosis. Some of the imaging manifestations of EAML are similar to renal carcinomas. However, the use of immunohistochemical examinations has to be performed to achieve the exact diagnosis.On CT-scan, soft tissue lesion with higher density  and enhancement pattern of “Rapid wash-in and slow wash-out” are suggestive of EAML.On Magnetic Resonance Imaging (MRI), EAML has a low-to-high T1WI signal (due to acute hemorrhage), low T2WI signal (with diffusion restriction due to hypercellularity of mass), distinct margins, and noticeable heterogeneous enhancement but lacks adipose tissue.On Ultrasonography, EAML is seen as a hypoechoic lesion with heterogeneous peak enhancement that may have a pseudo capsule .In our current case, the imaging studies revealed two infra-renal heterogeneous masses arising from the medial pole of the left kidney with significantly increased vascularity. Both lesions had characteristics of adipose tissue which suggest the diagnosis of either AML or a well-differentiated liposarcoma.Despite the benign behavior of most AMLs, few researchers reported that some lesions may be malignant when first observed or may later undergo malignant change and need immediate supervision. The issue was that later histopathologic evaluations of three previously reported cases of malignant AML without association with tuberous sclerosis suspected the primary diagnosis. Thus, some pathologists, have questioned the existence of a malignant EAML, when the diagnosis is not confirmed by immunohistochemical results .Recently, several Cases of malignant EAML with metastasis to the liver, lung, and bone have been reported. The similarity in the morphology of epithelioid cells and renal cell carcinoma (RCC) has made the diagnosis of EAML challenging .EAML is the malignant variation of renal AML, that can be distinguished by containing a large number of human melanoma black (HMB)- 45 epithelioid cells and lacking adipocytes .We believe a malignant EAML, although rare, does exist. Many of the previously reported cases of malignant AML lack the fat cells and abnormal blood vessels that are seen in the classic AML. Some of them were primarily misdiagnosed with renal cell carcinomas before the immunohistochemical evaluations confirm the diagnosis of AML.The small number of detected AMLs with malignant behavior makes it impossible to define a criterion for distinguishing benign from malignant EAMLs. Even though the significance of necrosis is under question since two of the researches designed by Delgado et al.  and EbleIn another study performed by Lee et al.  587 caseOur case had 2 SAMLs with a size of 2*1*0.5 cm and 32*22*8 cm. The greater mass was notably larger than the previously reported cases. Interestingly that huge mass remained asymptomatic and did not develop any complication till enlarged to reach that size .De Bree et al. reported a case of a huge renal EAML with a maximum diameter of 13 cm in the area of the resected 17 cm kidney, but their case in contrast to ours had hepatic and peritoneal metastases at the time of diagnosis [Guan et al. also reported a co-existence of AML and angiosarcoma in a 64-year-old woman who was presented with a progressively enlarging mass in the left abdomen. The tumor CT-scan revealed an 18 cm × 11 cm mass in the left posterior renal fascia. Immunohistochemical examinations confirmed the diagnosis. Their reported tumor was comparable to our case, in terms of size, while, that primary renal angiosarcoma was possibly arisen in a pre-existing AML of the kidney and showed a malignant behavior [Similarly, Espinosa et al. reported a case with recurrence of sporadic renal EAML in a 34-year-old patient, who underwent radical nephrectomy after diagnosis of a renal mass of 10 × 12 cm. This lesion had characteristics suggesting a poor prognosis . After nephrectomy, the patient did not receive adjuvant chemotherapy. Seven months after the surgery their patient developed 3 liver metastases. Their finding supports the rare, but possible malignant behavior of renal EAML. Thus, they recommended a long term follow-up after surgical resection of EAMLs .4Three recent studies designed by Brimo et al. , Nese etThe indicated unanimous factors associated with poor prognosis are namely, severe cytological, extent of nuclear atypia , larger epithelioid cell component, presence of more than 2 mitoses per 10 high power fields, association with TSC or concurrent AML, tumor size of larger than 7 cm, lymphovascular invasion, extra-renal extension, renal vein involvement, and carcinoma-like growth patterns.5Folpe, A.L. et al.  have beeDue to the malignant potential of renal EAML, the treatment of choice is complete surgical resection. Based on our review of the literature, all cases have remained asymptomatic after surgical management and there were no reports of recurrence after sparing nephrectomy or embolization .Sometimes the surgery should be performed in combination with target therapy (m-TOR inhibitors). however, the definite effects of this therapy have not been confirmed yet ,23. SeleThe tumor was excised and the margins were free of malignancy. The patient was doing well postoperatively. We did not recommend advanced chemotherapy after the surgery. Two years later, no local recurrence and distant metastasis were found in the follow-up CT-scan.The limitation of our study was that our patient did not accompany a further follow-up visit as he was satisfied with the outcome.6We believe, EAML has the potential for aggressive behavior and metastasis, so the diagnosis must be confirmed by pathological and immunohistochemical examinations when we find characteristics of this rare mesenchymal mass on the CT-scan. Considering the malignant potential of renal EAML, a long-term follow-up is required after the surgery. Our case was unique in that this patient had a sporadic giant Angimyolipoma with epithelioid content which was not associated with the TSC at the time of diagnosis. However, because of the radiologic features that EAML and malignant renal lesions have in common, renal carcinoma should be considered as an important differential diagnosis. Our patient had an uneventful follow-up period without recurrence.This article has no conflict of interest.We had no source of fundingThe ethical committee of Urology research centre at Iran University of Medical Sciences is aware of current publication and gave permission.Our patient gave us informed consent after making sure his information is published anonymously-Mohammad Ali Ghaed – Diagnosis and surgery – supervising this report-Maziar Daniali – Review of articles and writing manuscript-Paniz Motaghi – Review of articles and writing manuscript-Hamid Jalali Solhi – performing surgery and diagnosisThis study is not a clinical trial, thus has not been registered.Dr. Mohammad Ali Ghaed, the first author.Not commissioned, externally peer-reviewed


Gastrointestinal stromal tumor (GIST) is an uncommon tumor of the GI tract usually seen in elderly patients, often difficult to diagnose because of the unspecific symptoms such as abdominal pain and fullness. Recurrent GIST may have an even more obscure clinical presentation.  A 44-year-old female with a history of surgically treated GIST presented to the emergency room complaining of acute onset sharp RLQ pain, nausea, and vomiting. Clinically, she had RLQ tenderness with rebound, her WBC was elevated, and CT scan showed signs of appendicitis and also soft tissue masses suspicious for malignancy. After contemplating treatment options including antibiotics and further workup, it was recommended to proceed with surgery. Laparoscopy showed a thickened appendix with nodular infiltration and multiple mass-like lesions in the RLQ not amendable to minimal invasive resection. From a lower midline incision, an open appendectomy and excision of multiple masses in the terminal ileum and in the abdominal wall were done. Narrow-based Meckel's diverticulum with multiple nodular lesions was also removed. Pathology identified appendicitis and serosal involvement of GIST in all specimens staining positive for CD68, CD117, and vimentin. The patient was started on imatinib and remained recurrence-free after 6 months.  This case illustrates a rare presentation of acute symptomatic recurrent metastatic GIST. Our patient was unusually young, and GIST recurrence presented with acute RLQ pain suggestive for acute appendicitis and also involved Meckel's diverticulum. Surgical debulking followed by imatinib seems to be a reasonable approach in such cases. Gastrointestinal stromal tumor (GIST) comprises less than 1% of all GI tumors , 3. The Acute appendicitis may be treated with antibiotics alone; however, most surgeons still consider laparoscopic appendectomy the treatment of choice for most individuals . CT scanWe herein report the rare case of recurrent metastatic GIST to both the appendix and Meckel's diverticulum in a patient presenting with RLQ pain and appendicitis on CT scan.A 44-year-old female presented to the emergency room with sudden onset sharp right lower quadrant pain, nausea, and vomiting. On physical exam, she was positive for McBurney's, Rovsing's, psoas, and obturator signs. Her white blood cell count was elevated at 16.5. CT scan showed signs of appendicitis as well as suspicious intraabdominal soft tissue masses . Five yeSecondary to the CT findings suggestive for appendicitis, nonoperative management and further workup were contemplated. The RLQ masses were most concerning for recurrent GIST, and after discussion with the patient, indication for surgery was made. On diagnostic laparoscopy, the appendix wall was found thickened and acutely inflamed with nodular lesions. In addition, multiple up to 2 cm in diameter nodules on the terminal ileum and the parietal peritoneum of the anterior and lateral abdominal walls were visualized . One larThe patient had an uneventful recovery and was discharged from the hospital on postoperative day five. The patient was started on imatinib, and at the 6-month interval, she was doing well without any complaints; CT scan showed no evidence for recurrent GIST. She was then lost to follow-up when she moved.This is a rare presentation of GIST, in an unusually young patient with simultaneous GIST lesions to the appendix and a Meckel diverticulum amongst other intraabdominal sites. The patient presented with RLQ pain, elevated WBC, and CT scan findings suggesting acute appendicitis. At time of presentation to our ER, the patient was not aware of possible recurrent GIST; however, such pathology was immediately considered.Operative management remains the primary treatment modality for low-grade GIST tumors. For high-risk tumors, the recurrence rate after surgical resection alone remains high  with recIn patients with a history of GIST who present with abdominal symptoms, recurrent disease should be considered. Metastatic and primary GISTs may present similar to acute appendicitis , 3; even


Tumoral calcinosis (TC) is an exceedingly rare disease, significantly so when located in the spine. Here, we present a 4-month-old patient with decreased head control and range of motion of the neck for several weeks. CT and MRI demonstrated a calcified mass in the retropharyngeal area and surrounding C1/C2, and TC was suspected. The patient underwent surgical biopsy and aspiration, which confirmed TC. The purpose of this case report is to document a rare disease, significantly so when taking into account both the location of the lesion and the patient's age, and to detail the treatment and response. Tumoral calcinosis (TC) is a rare condition characterized by calcium deposits in periarticular soft tissues. This term can be used to describe both primary occurrences and those that arise from a secondary disorder, most frequently from kidney disease. Primary TC is usually further separated into two classifications based on the metabolic phosphorous status, either hyperphosphatemic or normophosphatemic. TC usually manifests in the first two decades of life as a painless mass around a joint. The most commonly affected joints are the hip, elbow, and shoulder. Here, we present a case of a TC in the cervical spinal canal because of the rarity of its location and the lack of literature on these occurrences.A 4-month-old Caucasian female with a medical history of mild torticollis presented to the emergency department at Children's Hospital New Orleans on May 30, 2017, with loss of head control milestone and hypotonia of the neck for 2 weeks. She also had experienced decreased range of motion of the neck and poor feeding during this time. On examination, the patient was found to have poor head control and could not hold her head up when placed in either the upright position or the prone position. Range of motion and strength was normal in all extremities. Neck CT (Figure After imaging and discussion among the interdisciplinary team, it was agreed upon to take the patient for surgery using a biopsy procedure to confirm an accurate diagnosis of TC. After induction of anesthesia and orotracheal intubation, the patient was positioned in the operating room bed and mouth was opened with a Crowe-Davis mouth gag. A bulge was seen in the left retropharyngeal space, and an incision was made in the retropharyngeal space. A stat was then inserted and opened, and fluid with chalky white deposits was aspirated and sent for microbiology and pathology. A curet was then taken and the anterior portion of the lesion was curettaged, and about 2 mL of chalky white fluid mixed with serosanguinous fluid was aspirated, only taking the portions of the mass that were easily accessible. Afrin-soaked pledgets were packed into the incision to close the gap, and the patient was handed back to anesthesia for recovery.Pathology Figure  demonstrThe patient recovered well postoperatively and saw improvements with apraxia and head control during the hospital stay, as well recovered feeding capabilities, and was discharged on postoperative day 7. The patient was then followed up with serial CT scans and with neurology over the next 5 months. The patient returned to the clinic for follow-up at 5 months postoperatively and was found to have no neurological deficits. Full strength and range of motion was seen in the neck and all four extremities, and no sensory deficits were noted. Repeat CT showed no residual retropharyngeal calcifications, some persistent calcification surrounding dens and extradural space at C1 and C2 levels, and no new calcium deposits. A subsequent follow-up at 11 months postoperatively continued to show no physical complaints, and a normal examination and repeat CT Figure  showed n1 after reviewing cases of TC; the pathogenesis-based classification system was proposed as follows: primary normophosphatemic TC, primary hyperphosphatemic TC, and secondary TC, most commonly from renal failure. Primary normophosphatemic TC is the most common form of the disease, and patients have no known disorders of calcium or phosphate metabolism, which was also demonstrated in this case of primary normophosphatemic TC. Trauma has been hypothesized to contribute to the development of the TC lesion because injury has been frequently reported preceding the development of TC, especially in the primary normophosphatemic form, and minimal repetitive trauma or chronic pressure has also been implicated.2 In this case, however, no preceding injuries or trauma were noted before the development of TC, which was further evidenced by the histopathologic evaluation of the biopsy specimen that revealed predominately calcified, acellular material with a notable absence of granulomatous tissue or bone marrow elements such as leukocytes. These inflammatory changes would normally be seen on pathology after muscle trauma3 or traumatic brain injury4 if they had occurred, thus the absence of such material provides further support to the atraumatic development of TC in this case.TC is a rare disease with an unclear cause. As previously discussed, a classification system was developed by Smack et al5 However, although not widely prevalent, TC involving the spine has been reported6 in the literature, along with cases specifically involving the cervical spine789 which was seen in this case. Regarding TC of the spine, a literature search revealed 15 cases of TC involving the cervical spine, not including this case, in addition to three involving the thoracic spine and 14 involving the lumbar spine.9 Of note, the youngest reported case of TC involving the spine was found to be 1.4 years of age,10 making this case possibly the youngest instance of a spinal primary TC reported in literature at 4 months of age.TC most commonly involves the periarticular soft tissues of the large joints of the extremities, including the hip, elbow, and shoulder, as well as the foot and wrist less commonly.11 Here, the patient presented with loss of the head control milestone and decreased tone and range of motion of the neck.TC most commonly manifests as a painless and asymptotic mass around joints, but it may cause compression symptoms, including pain, weakness, and sensory deficits, or limited range of motion of the joint involved.11 Furthermore, surgical resection has been shown to be curative in cases of primary TC seen specifically in infants, with some cases additionally reporting evidence of spontaneous regression of lesions in this age group.12 A curative result was also seen in this case although resection was performed only of the accessible area for biopsy, and it successfully resulted in a return to normal functioning of head control and neck range of motion. The intraspinal area was then monitored and treated conservatively with serial imaging and a clinic follow-up. As of this writing, no occurrence of neurological or musculoskeletal deficits has been observed. In addition, serial imaging has demonstrated a near complete resolution of the TC with only residual punctate calcifications surrounding the dens, suggesting spontaneous regression of the nonresected portion of the lesion, and no incidence of new calcifications or deposits have been seen.Treatment for TC most frequently involves surgical resection, especially for symptomatic cases causing neurologic dysfunction or joint function limitations. With the primary forms of TC, surgical resection is the primary treatment and has been shown to be successful with low recurrence rates.TC is a rare disease, especially when located within the cervical spine, with an unclear etiology. However, it should be considered on the differential diagnosis when considering spinal lesions. Common presenting symptoms can include pain, weakness, sensory deficits, and limited range of motion in the involved joint. Surgical resection is the primary method of treatment. However, in this case, observation was the primary method of treatment after surgical biopsy, which should be considered in the rare instance of intraspinal lesions in the neonate or infant with a nondeteriorating examination.


Hepatic angiomyolipoma is a rare primary liver tumor, with a radiographic appearance very similar to hepatocellular carcinoma. We present the case of a noncirrhotic patient with a liver tumor suspicious for HCC by imaging features. Liver biopsy demonstrated angiomyolipoma, and the patient successfully underwent a laparoscopic liver resection. Hepatic angiomyolipoma (AML) is a rare primary liver tumor, described only by small case series. Most cases occur in middle-aged women, who either are asymptomatic or describe vague abdominal pain. AMLs are tumors of mixed mesenchymal origin and predominantly occur in the kidney. Radiographically, hepatic AML can be difficult to differentiate from other liver tumors, particularly hepatocellular carcinoma (HCC), as they commonly appear as well-defined lesions with CT arterial phase enhancement and “washout” in the portal and delayed phases. Imaging features are not specific and can lead to a misdiagnosis of HCC if pathologic confirmation is not obtained . The golWe present a case of epithelioid hepatic AML diagnosed preoperatively by biopsy and successfully laparoscopically resected.A 54-year-old Caucasian female with a history of cholecystectomy, obesity, and hypertension presented to the Emergency Department with acute onset right upper abdominal pain. Basic laboratories including liver function tests, complete blood count, and basic metabolic panel were unrevealing.Outpatient workup revealed normal AFP, CA19-9, and CEA levels and negative hepatitis serologies. Ultrasound demonstrated a 2.3 cm, low-density mass in the left liver lobe. MRI showed a 3.4 cm mass with arterial phase enhancement and contrast washout in delayed sequences . By imagThe patient underwent laparoscopic hand-assisted left lateral hepatic segmentectomy. Our surgical approach included three trocar sites and a small midline hand port. Resection was completed using a combination of Ligasure and Cavitron Ultrasonic Surgical Aspirator (CUSA). One Jackson-Pratt drain was left at the cut edge of the liver.Pathologic gross examination revealed a 3.0 x 2.5 x 2.0 cm mass, well-demarcated from adjacent hepatic parenchyma . On micrThe patient was seen in follow-up one year after her tumor resection. She was doing well and had fully returned to her presurgical level of function. 4-phase liver CT done at that time demonstrated no tumor recurrence or evidence of abdominal metastasis. LFTs were all within normal range.Hepatic AML typically presents as an incidentally found liver mass or in patients with vague abdominal discomfort, as in our case. Rarely are these tumors large enough to lead to presentation from mass effect, palpation, or rupture, though this has all been reported . There iOn contrast-enhanced imaging, both hepatic AML and HCC appear hyperenhanced in the arterial phase with early washout times . SeveralMisdiagnosis as HCC has serious clinical and psychological implications for patients. Biopsies are not routinely performed once a radiographic diagnosis of HCC is made. Traditional treatment for HCC in a noncirrhotic patient is liver resection. However, liver transplantation is evolving as the standard of care for small HCCs due to simultaneous survival benefit and reduction in disease recurrence . The diaAccurate preoperative diagnosis as AML allowed the patient and surgeon to come to an informed decision and to undergo the appropriate treatment of minimally invasive resection. Damaskos et al. reported the first laparoscopic resection of hepatic AML in 2017, and our case further demonstrates the safety and utility of this approach . In the We encourage clinicians to have a high index of suspicion of misdiagnosis of HCC in noncirrhotic patients without other HCC risk factors and to employ preoperative biopsy to confirm diagnosis in these cases. A correct diagnosis is crucial to tailor appropriate tumor management. HCC warrants excision in noncirrhotic patients and, increasingly, referral for transplant evaluation; hepatic AML can be treated with a laparoscopic resection as a safe, novel alternative to open surgery or clinical observation.


Hemangioendotheliomas are vascular tumors characterized by the presence of an “epithelioid” or “histiocytoid” endothelial cell. They consist of blood filled spaces mixed with cellular areas that mostly occur in the liver, but other organs can be affected. These lesions cause a wide spectrum of clinical presentations depending on the organ involved. Complete surgical excision is the primary form of treatment; they tend to recur after surgery in some cases.A-45-year old lady presented with 2 attacks of massive hematemesis. Upper GIT endoscopy was done which showed a vascular tumor arising from the duodenal ampulla, biopsy was not taken because of the risk of bleeding.Laparotomy and duodenotomy were performed, there was a soft irregular 3 cm mass with areas of hemorrhage arising from the duodenum, complete excision was done and frozen section examination showed a benign lesion.Two months later the patient presented with melena and anemia. CT-scan of the abdomen showed a mass at the head of pancreas. Second surgery was done and during surgery a firm mass was found in the region of the head of pancreas, pancreaticoduodenectomy was done and the sample was sent for histopathological examination which showed an epithelioid hemangioendothelioma of the head of the pancreas.The treatment strategy should be tailored to the individual patient, the clinical presentation, and the rate of progression of the tumor. The natural history of the disease is still poorly understood and there is no uniform treatment form for such tumors. The term HE was first described as a distinct tumor by Weiss and Enzinger in 1982, when they defined this tumor as soft tissue vascular lesions originating from the endothelium and they have a clinical behavior in-between benign hemangiomas and angiosarcomas .These types of tumors tend to affect females mostly with female: male ration of 3:2, and mostly affect those more than 40 years, although their occurrence at younger age groups had been reported [The lesions consist of blood filled spaces mixed with cellular areas. These vascular tumors can occur in various organs in the body such as the liver, skin, spleen, salivary glands, and some other anatomical sites. The tumors stain positively with one of the vascular markers on immunohistochemistry such as factor VIII related antigen, CD31 and/or CD34 ,4,5.These lesions cause a wide spectrum of clinical presentations depending on the organ involved, cases have been reported presenting with abdomen pain, jaundice, and duodenal obstruction ,7.The work of this case report has been reported in line with the SCARE criteria .1.1A-45-year old lady presented with melena for 20 days, she had two attacks of massive hematemesis before admission. The patient had no history of any medication intake before this presentation. The patient had history of renal cell carcinoma which was operated by right nephrectomy before 10 years.1.1.1At admission the pulse rate was 120 beats/minutes and the blood pressure was 95/60 mm Hg. Resuscitation was done in the form of 2 peripheral intravenous lines and 2 liter of Ringer’s lactate solution, the patient received 9 units of fresh whole blood and 15 units of fresh frozen plasma.1.1.2Upper GIT endoscopy was done, biopsy was not taken because of the risk of bleeding, 1.1.3Decision for surgery was done, during laparotomy and duodenotomy, there was a soft irregular 3*5 cm mass with areas of hemorrhage arising from the wall of the duodenum, complete excision was done and sent for frozen section examination which showed a benign lesion, th postoperative day with no complications.The duodenum was closed transversely and the patient was discharged in the 5The histopathology report after the first surgery revealed a benign inflammatory lesion or a possibility of polyp.Two months later she presented with melena and anemia. She received 6 units of blood.Upper gastro-intestinal tract (GIT) endoscopy was done which showed recurrence of the mass, the patient was sent for CT-scan of the abdomen which showed a 7*4 cm mass with extension to the head of the pancreas, Decision for pancreaticoduodenectomy was done. During surgery a firm mass was found in the region of the head of pancreas found, pancreaticoduodenectomy was done and the sample was sent for histopathological examination which revealed a vascular mass lined by endothelial cells with intervening epithelioid spindle cells, there was no evidence of nuclear atypia, the tissue stained positively for CD 34, FL1, and pancreatin and stained negatively for CD117, S100, desmin and chromogranin A. These features were consistent of epithelioid hemangioendothelioma of the head of the pancreas, all the resection margins were free from the tumor, 1.1.4The patient was discharged 7 days after surgery with no complications. Follow up was done for 2 years after the surgery with no recurrence of the tumor.2The majority of these tumors affect the liver which occur in around 70% of the cases, with right lobe involvement more than the left one and tend to be multifocal. Extrahepatic involvement is observed in the rest of the patients, the lung is the second most common organ that is involved, other organs also may be involved such as the lymph nodes, the bones, the peritoneum, and the diaphragm in order of decreasing frequency .The tumor histological examination such as nuclear pleomorphism and mitotic rate don’t predict the biological behavior of the tumor .Local recurrence is common which may be due to contiguous spread or multifocality of the disease, metastatic disease is extremely rare .The treatment of such tumors depend on the size of the lesion, the location, and the presentation. Some lesions are treated with enucleation, others are treated with local or wide excision, systemic use of steroids and interferon have been used with different responses, in our case we didn’t use these form of treatment because the patient had emergency presentation, we performed complete surgical excision as a primary form of treatment ,10.The tumor mostly recurs locally, but in some rare occasions these tumors metastasize to other organs, the mediastinum, the retroperitoneal space, the myocardium, the pericardial membrane, the brain, the local lymph nodes draining the anatomical area of the primary tumor, the pancreas, and the uterus are some sites of distant metastatic disease that were reported by some authors .Currently there is no uniform modality for treatment of these tumors, and all the therapeutic trials are directed for individual cases, local and international guidelines are required for the management of these tumors depending on the organ involved and the long term follow up of the registered cases.3I thought that the mass in the pancreas is recurrence of my renal tumor, I am happy with the results of surgery and I will keep regular check up in the future.None.Ethical approval has been exempted by my institution for reporting this case.Written informed consent was obtained from the patient for publication of this case report and accompanying images.Dr Ayad Ahmad Mohammed and Dr Sardar Hassan Arif are the surgeons who performed the operation.The concept of reporting the case, data recording, and drafting the work done by Dr Ayad Ahmad Mohammed and Dr Sardar Hassan Arif.Dr Sardar Hassan Arif took the consent from the patient for publishing the case.Final approval of the work to be published was done by Dr Ayad Ahmad Mohammed.This work is case report and there is no need of registration.Dr Ayad Ahmad Mohammed is guarantor for the work.Not commissioned, externally peer-reviewed.The author has no conflicts of interest to declare.


An 83-year-old patient with a recent history of constipation was referred to the emergency room with diagnosis of acute abdomen. The patient had experienced a very acute periombilical pain a few hours before admission. At arrival the pain was continuous and diffuse. Diffuse tenderness with rebound was present especially in the left iliac fossa. The patient was afebrile. Except a mild hyperleucocytosis there was no inflammatory syndrome in blood tests.Unenhanced Abdominal CT was immediately performed . Diffuse pneumoperitoneum was found with countless small gas bubbles in all peritoneal recesses (black arrow). There was virtually no peritoneal fluid but diffuse infiltration of the peritoneal folds was already present evocating diffuse peritonitis.Solid feces were still found in the middle portion of the sigmoid colon (black star) and in the rectum but the major part of the entire colon appeared nearly almost flat and empty.The major CT finding was a very large accumulation of solid feces within the free peritoneal cavity (white stars) extending from the right paraumbilical area to the Douglas’ pouch and that all along the antimesenteric border of the thin and stretched distal sigmoid (white arrows). The level of perforation was not clearly visible. The patient underwent emergency laparotomy. A large ischemic ulcer was found at the level of the rectosigmoid junction and an enormous amount of feces were evacuated. A Hartmann’s procedure was performed and extensive peritoneal lavage was performed with 15 liters of hydrosaline solution.Colon perforation is a rather uncommon event usually caused by malignancy, amoebic colitis, diverticular disease, spontaneous perforation, foreign body perforation, steroid therapy, trauma, and ulcerative colitis. Stercoral ulcer with perforation is a rather very rare event. Fewer than 150 cases have been reported in the literature through 2011 but the incidence is probably underestimated. Severe chronic constipation is considered to be the main causative factor of stercoral colonic perforation because it may induce the formation of stone-hard fecalomas and maintain a persistent pressure over the bowel wall leading to pressure necrosis of the mucosa. Fortunately stercoral ulceration of colonic mucosa does not always occur in the vast majority of constipated patients and not every stercoral ulceration results in colon perforation. A great majority of patients remain at the intermediate state of stercoral colitis . The CT findings of stercoral colitis include focal colonic or rectal wall thickening involving the dilated sigmoid colon and rectum, which demonstrate fecalomas. Pericolonic and/or perirectal fat stranding is usually seen due to colonic ischemia or wall edema. The presence of intramural or extraluminal bubble of gas or of an abscess suggests that perforation has already occurred. The three most common locations for stercoral ulceration are the anterior rectum just proximal to the peritoneal reflection, the antimesenteric border of the rectosigmoid junction (the most common site) and the apex of the sigmoid colon. There are several combined reasons why the perforation sites are mainly located in the antimesenteric border of the distal sigmoid colon. First the sigmoid is the narrowest region of the entire colon, and the passage of stools with a more solid consistency at this distal level can be difficult. In such circumstances, fecaloma may exert localized pressure on the walls of the sigmoid colon, particularly in the area with the most precarious vascular supply known as the Sudeck’s critical point . Finally prolonged localized pressure and ischemia can give rise to pressure ulceration eventually complicated by stercoral perforation. The mortality related to colon stercoral perforation has been reported to be high. A more favorable outcome in the treatment of stercoral perforation depends upon an immediate treatment of any underlying sepsis, the removal of all stercoral ulcerated diseased colonic tissue, extensive peritoneal lavage, aggressive therapy to counteract colonic perforation peritonitis and appropriate treatment of any co-morbid medical conditions.The authors declare that they have no competing interests.