Schistosoma mansoni or Schistosoma japonica. Sudan is considered one of the endemic areas of schistosomiasis and many studies were done as regard to the prevalence and impact of the disease. Schistosomal appendicitis is rare, particularly in developed countries compared to endemic areas.Intestinal Schistosomiasis is a parasitic disease caused by This is a case of 36 –year- old man with past history of schistosomiasis presented with features of intestinal obstruction to Ibrahim Malik teaching hospital in Khartoum-Sudan. Exploratory laparotomy revealed gangrenous appendix. Histopathology results came back as extensive necrosis of the appendix with multiple viable Schistosomal ova. Stool analysis revealed no ova. The patient received praziquantel therapy and now he is in a good condition.Clinicians and pathologist should be aware of schistosomal appendicitis, particularly in endemic areas like Sudan. •Schistomiasis can have variable ways of clinical presentations especially in endemic countries.•Acute appendicitis can be one of the clinical presentations of schistosoma.•Clinicians and pathologists should be aware of this rare presentation of schistosoma. However, there is few reported cases of intestinal Schistosomiasis presenting as acute appendicitis in Sudan. In the literature, Schistosoma haematobium was reported as the inducer of a granulomatous inflammatory response in addition to eosinophilia and fibrosis of the appendix. Oviposition was associated with frank acute appendicitis, and serosal involvement resulting in peritoneal adhesions and ileoileal intussusception [Intestinal Schistosomiasis is a parasitic disease caused by  sources . Schisto sources . A revie sources . This is sources . Sudan i sources ,4. Howevsception . An Afrisception . Many sisception . In one sception . The rolsception .2CT) scan of the abdomen revealed dilated bowel loops (This is a case of 36-year-old man who presented with features of intestinal obstruction to our institution. He was diagnosed in Egypt as a case of intestinal Schistosomiasis one year prior to admission and received treatment. However, follow up investigation to confirm eradication of the disease was not done. The patient presented to our surgical department complaining of absolute constipation, abdominal pain, abdominal distention and vomiting. On examination the abdomen was distended and bowel sounds were absent. There was tenderness in the lower part of the abdomen and digital rectal examination revealed collapsed rectum. Computed tomography (el loops . The patel loops  followedel loops . Subsequ3Schistosomiasis is a parasitic disease transmitted through contact with contaminated water sources. It is related to bad personal hygiene and environmental sanitation. This explains the dominance of this disease in the endemic areas within the developing countries. The disease is endemic in Sudan ,4. SchisIn fact, the predominance of features of intestinal obstruction in the present case is considered to be an atypical presentation for acute appendicitis. Such way of presentation has been rarely reported and it might be linked to paralytic ileus.The diagnosis of Schistosomal appendicitis in this case was confirmed using reliable histopathological methods. Despite the negativity of stool analysis for Schistosomal ova, there is an evidence of past history of Shistosomiasis which strongly support the current diagnosis.It is now agreed that appendiceal schistosomiasis may be the atiology of acute appendicitis. This may be due to ischemic changes caused by intravascular egg impaction. Moreover, this may diminish mucosal immunity, increasing the risk of bacterial infection .In endemic areas like Nigeria, Badmos et al.  reported4Clinicians and pathologist should be aware of schistosomal appendicitis, particularly in endemic areas like Sudan.The research was ethically approved.The case report was not funded.The author Mugahid A Salih IS a general surgeon who met the case and he is the one who wrote the manuscript.No conflict of interest.None.The guarantor is the author Mugahid A Salih.The patient signed informed consent.Not commissioned, externally peer reviewed.


Plasmacytoma as the 1st presentation of skull tumors is a rare disorder. When it is combined with brain trauma or dramatic changes in intracranial pressure, patients are more prone to misdiagnosis.A 67-year-old woman complaining of a headache presented with a history of head trauma for the past 1 hour. Emergency head computed tomography initially suggested an epidural hematoma.Emergency surgery was performed to remove the intracranial hematoma, but a tumor-like mass was found during surgery, and pathologic assessment confirmed plasmacytoma. Surgery was difficult because of bleeding. The tumor was radically removed.The patient underwent whole-brain radiotherapy and chemotherapy. She died 40 months after the surgery.Epidural lesions found after a head injury may be assumed to be an epidural hematoma, leading to unnecessary surgery. Diseases such as hematomas, meningiomas, eosinophilic granulomas, bone metastases, and osteosarcomas must be considered. However, skull plasmacytomas initially showing mass-like tumors are rare in clinical practice and are easily misdiagnosed as meningioma or metastatic tumors.–3 We report a patient with a skull plasmacytoma and discuss the preoperative clinical diagnosis of this patient. There was no systemic involvement in this case.Multiple myeloma is a neoplastic lesion that usually originates in bone marrow, including that from the pelvis, skull, vertebrae, and ribs. Multiple myeloma constitutes approximately 1% of all malignant neoplasms. The disease may also have very rare extraosseous manifestations, which can arise in any tissue, but are found in <5% of patients with multiple myeloma.2In November 2014, a 67-year-old woman was admitted to our hospital because of head trauma. She had a history of a headache for 2 weeks before the trauma. On physical examination, a firm, nontender, subcutaneous nodule 2 cm in diameter was observed in the parietal region. Her Glasgow coma scale (GCS) score was 14. Computed tomography (CT) of the head revealed a 6 × 3 × 6.5-cm, epidural, slightly dense mass in the left temporoparietal region and 2 smaller epidural masses in other regions. The left mass was compressing part of the brain but had well demarcated to the dura. There was no evidence of a midline shift, but the left ventricle was smaller than the right ventricle . The intracranial manifestations of plasmacytomas as mass lesions are rare and easily misdiagnosed. Cranial tumors may also be the only manifestation of plasmacytomas. Moulopoulos et al found that pure central nervous system involvement without any bone lesions is very rare, occurring in <1% of patients with multiple myeloma.,7 or hearing disturbances, while some patients might have no symptoms at all. Nguyen and Patel noted that a plasmacytoma in one patient was found by her hairdresser. However, in our case, the patient had a short history of a headache, and mixed with head trauma, these circumstances confused the doctor's judgment. CT showed that a destructive or well-demarcated soft-tissue mass was compressing part of the brain. On magnetic resonance imaging, the tumor was isointense relative to the brain and enhanced on T1-weighted imaging.,7,9 Unlike multiple myeloma, solitary skull plasmacytomas may not have serum M protein or Bence Jones light chains in the urine.,5,7,9 The diagnosis is usually made after pathologic tissue is obtained after surgery. Examination of a bone-marrow biopsy specimen is needed.,7,9,10Patients with solitary skull plasmacytomas often present with several months of slight headaches The dura is involved in plasmacytoma infiltration in some patients with these diseases. Solitary intracranial plasmacytomas without any other signs of multiple myeloma have been associated with a good prognosis after surgical or radiation therapy,7,9; on the contrary, the development of extraosseous manifestations in multiple myeloma are characterized by rapid progression and resistance to treatment. Some authors consider that it is best to administer radiotherapy after histologic diagnosis of the tumor, as this tumor is highly radiosensitive.,8 Mäntylä et al and Yang et al have argued that gross total removal of the tumor followed by chemotherapy is successful.,13 Nguyen and Patel stated that a patient had undergone autologous bone marrow transplantation after the mass was excised. Alessandro et al suggested that combined therapy (chemotherapy followed by radiotherapy after surgery) can be very efficacious.The tumor may exhibit invasion from the subcutaneous tissue to the brain parenchyma or from the dura mater to the brain.Plasmacytomas originating from the calvarium are usually rich in vasculature, and pathologic vessels and a tumor stain may be observed on angiography. In this case, the assessment was insufficient before surgery; the patient was not considered to have a plasmacytoma, and bleeding during the surgery lead to increased patient risk. When the preoperative diagnosis is a skull plasmacytoma, the neurosurgeon should pay attention to special control of the skull bleeding and choose different methods of craniotomy. The process of opening the skull should include the process of blocking the blood supply: drilling the skull out of the edge of the tumor, then biting but not milling the bone from the edge of the tumor in the normal skull, and sealing the edge of the bone with bone wax quickly to stop bleeding. This method can reduce the bleeding during a surgery effectively. The tumor must be completely removed; otherwise, it is easy to cause bleeding again. Intracranial plasmacytomas have some clinical features that can contribute to identifying other tumors, such as hematomas, meningiomas, eosinophilic granulomas, bone metastases, and osteosarcomas; these include the following: Age: Plasmacytoma usually occurs in patients >40 years; the median age of onset is 62 years, and only 2% to 3% of patients are younger than the age of 30 years. Eosinophilic granulomas are always observed in children and young individuals, and they mostly present as a single mass (85%). Radiologic signs: CT features of skull plasmacytomas include a mass of slightly high density and containing multiple osteolytic damage in the cranial diploë, which breaks through the cortical bone and erodes the surrounding soft tissue. Calcification can be observed in the mass; the minor bone involvement could be explained by pressure erosion; because of the pressure phenomenon, meningiomas may cause osteolysis, but this is usually associated with areas of sclerosis and thickening. Osteosarcomas and bone metastases also have osteolytic types, but the edge of the bone is blurred. Laboratory examination including bone marrow, serum protein electrophoresis, serum immunoglobulin, blood routine, Bence Jones protein, and renal function assessments. A systemic examination is necessary to evaluate the state of the body in some patients who have a solitary skull plasmacytoma. Solitary plasmacytomas are circumscribed neoplastic lesions with no clinical or radiologic signs of systemic involvement, accounting for about 7% of myelomas. In this way, usually nonspecific proteinuria and serum immunoglobulin develops, although renal function remains unimpaired.Singleton and Koerner demonstrated the diagnostic challenge associated with one of the most common pathologic entities in neurosurgical practice: extradural mass lesions.Conceptualization: Zu Peng Chen.Data curation: Zu Peng Chen.Software: Xu Li.Writing – original draft: Zu Peng Chen, Xu Li.Writing – review & editing: Zu Peng Chen.


Spontaneously ruptured large splenic cyst is a rare in children, and traditionally total or partial splenectomy has been performed for treating an emergent case. We herein present a first case with spontaneously ruptured pediatric splenic cyst treated with emergent laparoscopic dome resection with omental suturing to the parenchymal edge of the spleen., and the greater omentum was then sutured to the anterior edge of the splenic parenchyma under a laparoscopic view. No complications were observed during the operation. A histological examination revealed a congenital splenic cyst lined by epithelial cells. The postoperative course was uneventful, and an ultrasound scan showed no evidence of cyst recurrence at 3 years after the operation.A 12-year-old girl with a spontaneously ruptured large non-parasitic splenic cyst (SC) was successfully treated by emergent laparoscopic dome resection with omental suturing to the edge of the splenic parenchyma. The patient presented with acute abdominal pain and was diagnosed with a ruptured non-parasitic SC and peritonitis by contrast-enhanced computed tomography (CT). Emergent laparoscopic dome resection of the SC and omental suturing to the splenic parenchymal edge were then performed. The protruding part of the cyst wall was completely resected using an ultrasonically activated device (USAD)This minimally invasive laparoscopic procedure was feasible and effective for treating a ruptured large splenic cyst in an emergent pediatric patient. Congenital splenic cysts (SCs) are uncommon , and spo3. The serum levels of carbohydrate antigen (CA19-9) and CA125 were 8 U/ml  and 12 U/ml , respectively. She had no history of abdominal trauma, and cystic Echinococcosis was excluded because she denied any history of traveling abroad. Malignant potential was also excluded, as her tumor makers were negative, and contrast-enhanced CT did not reveal any malignant features, such as heterogeneous, multifocal, irregular nodules [A 12-year-old girl presented to a clinic with acute abdominal pain. Her symptoms occurred suddenly, without abdominal trauma, during a school mathematical education class. She was first diagnosed with acute appendicitis by a pediatrician and transferred to our hospital at 5 h after the onset of symptoms. A physical examination showed abdominal distension, with rebound tenderness in the right lower quadrant. Her heart rate was 88 beats/min; blood pressure was 105/66 mmHg. Contrast-enhanced CT demonstrated a collapsed large simple SC  without solid or multifocal component at the upper pole of the spleen , a 30° laparoscope was inserted through a 12-mm umbilical port, followed by two 5-mm working ports, . Laparoscopic abdominal investigation revealed a lot of brownish ascites and a ruptured large cyst in the upper pole of the spleen. The wall of the ruptured cyst showed loose wrinkles. The hilum of the spleen was dissected initially to facilitate the control of bleeding in the event of an emergency, as a branch of the splenic vessels had already been detected in the parenchyma near the border of the collapsed cyst on preoperative enhanced CT (Fig.  L/min, aThe postoperative course was uneventful at 3 years after surgery, with ultrasound scans showing no evidence of recurrence of the SC.The management of non-parasitic SCs remains controversial. Small and asymptomatic cysts are often conservatively treated. In contrast, large or symptomatic SCs are usually surgically treated , 4. DespLaparoscopic complete resection of the protruding part of the wall of SC using a USAD and omental suturing to the edge of the splenic parenchyma may be a feasible and effective procedure for managing ruptured large non-parasitic SCs in pediatric patients.


Fibroids are a condition commonly seen as part of routine gynaecological practice and generally present with menorrhagia, infertility, or pressure symptoms depending on their exact location. We report the case of a postmenopausal 57-year-old lady presenting with left-sided pain and frequency with a complex 15 cm mass mid pelvis and an adjacent additional 7 cm cyst but a risk of malignancy index suggesting benign pathology. She underwent total abdominal hysterectomy (TAH) and right-sided salpingo-oophrectomy (RSO) on recommendation of the multidisciplinary team (MDT) and histology confirmed leiomyomatosis peritonealis disseminata. This rare but important condition is often confused with peritoneal carcinomatosis on imaging. The mainstay of treatment involves lesion and omental excision followed by removal of hormonal stimuli although this approach must be personalised as many younger women may wish to retain their uteri. Long-term follow-up is essential due to the potential for malignant transformation. Fibroids are one of the commonest benign gynaecological conditions, occurring in almost 1 in 3 women over the age of 30 [A 57-year-old postmenopausal lady, para 0 with an unremarkable smear history, presented to the gynaecology department with a 3-month history of left-sided abdominal pain and frequency of micturition. She previously had a laparoscopic left-sided salpingo-oophrectomy for a benign mucinous cystoadenoma 3 years earlier. Clinical examination confirmed a pelvic mass arising from the pelvis. There were no features suggestive of an acute abdomen. An ultrasound scan demonstrated a large complex thick-walled cyst mid pelvis measuring 15 × 13 × 12 cm displacing the uterus to the right. There was an additional 7 × 6 cm complex cyst seen adjacent to this mass. Neither ovary was subsequently identified. The Ca125 was 8, giving a risk of malignancy index (RMI) of 24. A subsequent MRI pelvis was consistent with right ovarian cystic adenoma/cyst adenocarcinoma, and a bulky postmenopausal fibroid uterus containing multiple fibroids displaced to the right of the midline. The patient was referred to the MDT and total abdominal hysterectomy, right salpingo-oophrectomy, and omental biopsy were recommended in view of the potential for malignant diagnosis. This was completed uneventfully. At operation, the findings confirmed a multi-fibroid uterus with a large right-sided cystic mass. She made a good recovery postoperatively and a follow-up CT undertaken 6 months after surgery did not show any evidence of disease recurrence. She was commenced on letrozole 2.5mg daily in view of the histology results. Oestrogen results were not measured pre- or postoperatively.Histology confirmed leiomyomatosis peritonealis disseminata–oestrogen receptor positive but no obvious malignant cells.Psathas et al.  define lThe pathogenesis is currently uncertain, although multiple theories have been postulated by Yang et al. (2009) including peritoneal metaplasia, alterations in oestrogen and progesterone levels, genetics or iatrogenic factors, and most notably the dissemination of fragments of tissue through the peritoneal cavity secondary to fibroid morcellation at laparoscopic myomectomy.Its appearance on imaging may be most commonly confused with peritoneal carcinomatosis, although other primary peritoneal malignancies, liposarcoma, lymphoproliferation, and disseminated endometriosis are also amongst the differentials . Yang etPsathas et al.  propose Although the majority of cases of LPD are benign, there remains potential for malignant transformation and, in view of the young age of many of these patients, long-term follow-up is essential . DebulkiLPD remains a rare but important differential for disseminated peritoneal carcinomatosis, particularly when seen in women of reproductive age. Although conservative management may be offered after surgery, long-term surveillance is essential to reduce the impact of progressive disease or malignant transformation.


Introduction The Littre hernia is a rare complication of Meckel's diverticulum. Meckel's diverticulum is vestigial remnant of the omphalomesenteric duct occurring in approximately 2% of the general population with an estimated 4 to 16% risk of complications. Usual sites of the Littre hernia include inguinal (50%), umbilical (20%), and femoral (20%). We report a case of an acquired transthoracic Littre's hernia occurring through the left part of the diaphragm triggered by a history of traumatic rib fractures associated with alcohol abuse.Case Report A 71-year-old man presented with 4-day history of worsening shortness of breath, colicky lower abdominal pain, and inability to open bowels despite passing flatus, without nausea or vomiting. His past medical history was remarkable for multiple traumatic rib fractures caused by falls which were associated with excessive alcohol consumption. A noncontrast computed tomography (CT) scan of the abdomen and pelvis showed distended jejunal loops containing air/fluid levels likely resulting from herniated jejunum between the left chest wall and left diaphragm. An urgent laparotomy was performed which revealed small bowel and omentum herniating through a small defect in the left posterior hemidiaphragm. The contents of the sac were reduced and a Meckel's diverticulum was found inside the sac, characteristic of Littre's hernia. The diaphragmatic defect was closed and the Meckel diverticulum stapled and excised.Discussion Herniation of Meckel's diverticulum through the diaphragm most commonly occurs in the pediatric population. Acquired transthoracic Littre's hernia is rare and may arise following thoracobdominal trauma caused by surgery, motor vehicle accidents, and falls from height. Left-diaphragmatic tears are characteristically more clinically apparent and symptomatic than the right since the liver often has a protective effect on the right part of the diaphragm. Herniation of abdominal contents in the chest cavity causes respiratory distress and requires urgent surgical correction. Diagnosis is often delayed since diaphragmatic hernia tends to present very late after the initial trauma, subjecting the patient to possible life-threatening complications. While it is easier to reduce the herniated contents and repair the diaphragm via a thoracic approach, laparotomy is often preferred in cases of acute trauma associated with intra-abdominal injuries. Repair of Littre's hernia then consists of resection of the diverticulum and herniorraphy.Conclusion Internal Littre's hernia is usually of congenital origin. This is the first case of a transthoracic Littre's hernia caused by traumatic rib fractures. Hence, it is of utter importance that a clinician is aware of such uncommon pathology. Meckel's diverticulum is the most common congenital malformation of the gastrointestinal tract and is a vestigial remnant of the omphalomesenteric duct. During fetal development in-utero, this structure connects the primitive gut with the yolk sac of the embryo and usually regresses between the sixth and seventh week of life. It is a true diverticulum involving all three layers of the intestinal wall; however, it may also contain ectopic gastric, pancreatic mucosa, and less commonly duodenal and colonic mucosa.As far as the characteristics of Meckel's are concerned, literature often quotes the rule of two's. The prevalence of Meckel's in the general population is reported to be approximately 2%, although it is difficult to obtain a true estimate since the condition is typically silent. It reaches 2 inches length (5 cm) and occurs within 2 feet (60 cm) proximal to the ileoceacal valve on the antimesenteric border of the ileum.Littre's hernia is the protrusion of Meckel's through a potential abdominal opening. Two kinds are recognized as follows: (1) a combined Littre's hernia (when a Meckel's diverticulum is accompanied by another viscus in the hernia sac) and (2) true Littre's hernia (a solitary Meckel's diverticulum).A 71-year-old man presented to the emergency department at Mater Dei Hospital  with 4-day history of colicky lower abdominal pain, without nausea or vomiting, and an inability to open bowels despite passing flatus. He also complained of a cough productive of greenish sputum and worsening shortness of breath. On examination, he was afebrile with a blood pressure of 179/80 mm Hg, pulse of 90 beats per minute, and peripheral oxygen saturation of 92% on room air. There were bilateral fine chest crepitations on auscultation. He had lower abdominal tenderness on deep palpation but no signs of peritonism. Bowel sounds were sluggish and no hernias were identified clinically.2), and serum potassium of 5.65 mmol/L. His international normalized ratio (INR) was 2.71, C-reactive protein 88.6 mmol/L, and lactate 0.4 mmol/L. A chest X-ray identified a chronic left sided pleural effusion with fibrotic changes in the right lower lung zone. Evidence of a healed left seventh rib fracture and a large hiatus hernia were also present. On abdominal X-ray, multiple dilated loops of small bowel were noted.Urgent laboratory investigations revealed a normal white blood cell count (4.52  × 109/L), microcytic anemia , acute kidney injury , 30 mL/min/1.73mHis past medical history was remarkable for hypertension, congestive cardiac failure, cerebrovascular accident, right subclavian vein thrombosis, left carotid endarterectomy, right pulmonary empyema, left sided pneumothorax with surgical emphysema following fifth and sixth rib fractures in 2011 (requiring chest drain), and traumatic left 6 to 12th rib fractures in 2014. He was also an exsmoker and exhibited alcohol misuse. His medications included warfarin (dose adjusted according to INR), folic acid 5 mg daily, lactulose 15 mL daily, simvastatin 10-mg nocte, and bumetanide 0.5 mg daily.A noncontrast computed tomography (CT) scan of the abdomen and pelvis showed distended jejunal loops containing air/fluid levels likely resulting from herniated jejunum between the left chest wall and left diaphragm . No obvPostoperatively, the patient made a steady recovery. Regular chest physiotherapy was performed and thromboembolic deterrent (TED) stockings applied. The drain was removed 4 days later and antibiotics stopped after 9 days. He was deemed fit for discharge home 10 days into his admission.Meckel's diverticulum is often an incidental finding during laparoscopy or laparotomy performed for nonrelated reasons. According to Johann Friedrich Meckel himself, the incidence of complications associated with Meckel's is 25%, although risk of complications in recent research ranges from 4 to 16%. Complications tend to occur more in males and decrease with age with the majority of them occurring in the pediatric population. Common presentations for a symptomatic Meckel's are intestinal obstruction, gastrointestinal hemorrhage, and diverticulitis with or without perforation.Littre's hernia is an unusual complication of Meckel's diverticulum. Usual sites of Littre's hernia include inguinal (50%), umbilical (20%), and femoral (20%).Meckel's diverticulum has herniated through the diaphragm in other reported cases, but these were of congenital origin.The incidence of acquired diaphragmatic hernia following trauma ranges from 0.8 to 1.6%.A particular challenge in the successful treatment of acquired diaphragmatic hernia is early diagnosis which can sometimes be excessively delayed, especially in cases when the degree of diaphragmatic defect has not been established in the acute period of trauma. It is relatively common for a diaphragmatic hernia to present very late after the initial trauma with an increased risk of life-threatening complications.Herniation of abdominal contents in the chest cavity causes respiratory distress and requires urgent surgical correction. Laparotomy is the most common approach in cases of acute trauma associated with intra-abdominal injuries.Surgery usually involves reduction of hernia contents followed by repair of the diaphragmatic defect. In our case, a midline supraumbilical laparotomy was performed. Repair of Littre's hernia consists of resection of the diverticulum and herniorrhaphy. Pneumothorax and septicemia are the most common complications following surgical intervention and these were absent in our case.This case addresses an extremely rare finding both in terms of the presence of Meckel's and in the way it caused complications. As identified in a single previous case report, Meckel's herniation through a diaphragmatic defect in an adult typically occurs as a late complication of surgery. Our report is the first of its kind involving repeated trauma to the lower ribs on the left side. This contrasts with more common forms of internal Littre's hernia which are mostly of congenital nature. While the etiology of small bowel obstruction is diverse, especially with a history of abdominal or pelvic surgery, a clinician must not be caught out by the less common precipitants.


Solitary fibrous tumor (SFT) is a rare soft tissue tumor originally reported in the pleura. Although it has been reported in various extra-pleural sites, the occurrence of SFT in the scrotum is extremely rare. Herein, we present a 48-year-old man who had scrotal SFT. There are very few reported cases of genitourinary SFTs, this is only the fifth report of SFT of the scrotum in the English medical literature.In this study, we report on a 48-year-old man who presented with a 5 × 8 cm scrotal mass between his testes. Physical examination revealed a 4.7 × 8.5 cm lobulated tumor mass located between his testicles. Surgical excision of the tumor with scrotal approach was done and pathology reported a SFT. The patient was alive without tumor recurrence or distant metastasis during ongoing follow-up for 9 months post-operatively..Scrotal SFTs are very rare and only five cases have been reported in English literature to date. Treatment often involves surgical resection, and a definite diagnosis is made with the help of immunohistochemistry. The current general consensus for the management of SFTs is long-term follow-up after surgical excision of the tumor. Solitary fibrous tumor (SFT) is a rare mesenchymal spindle cell neoplasm usually originating from the pleura. It was first described by Klemperer and Rabin in 1931 –9. TreatA 48-year-old male presented with a slow-growing right scrotal mass for the past 2 years. This clearly-demarcated nodular mass was located over the middle-to-right side of the scrotum. The tumor had rapidly increased in size over the past 3 months, but there was no obvious pain or other symptoms. Physical examination revealed a 4.7 × 8.5 cm lobulated tumor mass located between his testicles, non adherent to the scrotum. It was freely movable with elastic consistency on palpation. The penis and testicles were normal in appearance. Testis tumor markers were all normal .Scrotal ultrasonography showed a hypoechoic extra-testicular mass with clear contours and rich blood flow. Computed tomography (CT) showed a hypervascularized lobulated mass (4.7 × 8.5 cm) with contrast media enhancement in the midline of the scrotum Fig. . He thenThe resected specimen consisted of three lobulated and contiguous firm tumors, measuring 7.5 × 6.3 × 3.8 cm in size and weighing 76.5 g in total Fig. a. Cut seSFT is a soft tissue tumor which usually presents as a firm, grey-to-white colored, well-circumscribed solid mass. It was first reported by Klemperer and Rabin in 1931 in the pleura. Although the disease most commonly occurs in the pleura, extra-thoracic SFTs have been reported in many sites including the head and neck , intracrThere are very few reported cases of genitourinary SFTs, and SFTs of the scrotum or para-testicular SFTs are extremely rare Table . This isThe diagnosis of extra-pleural SFTs is challenging and relies on its clinical manifestations or imaging studies. Differential diagnosis of SFTs arising from para-testicles soft tissues can be challanging since it can show great similarity to those spindle cell fibroblastic associated tumors, such as angiomyolipomas, leimyoma, fibrosarcomas and gastrointestinal stromal tumors (GIST). Therefore, immunohistochemistry plays an important role in the diagnosis of SFTs. Traditionally, CD34 and BLC-2 have a high sensitivity for SFTs, and CD34 is only absent in 5 to 10% of typical SFTs . BCL-2 iSFTs are usually considered to follow a benign clinical course with a low potential of malignant change, and surgical excision of the lesion is usually sufficient. There were no current consensus on how the surgical approach should be done. Since SFTs usually presents as a low-malignant potential tumor, both inguinal and scrotal approach for tumor exploration and excional had been reported. However, malignant pathology and behavior have been described in about 20% of SFTs . The diaIn conclusion, SFT is a rare mesenchymal spindle cell neoplasm originally reported in the pleura, but it can be found in various sites throughout the body. Scrotal SFTs are very rare and only five cases have been reported to date. Treatment often involves surgical resection, and a definite diagnosis is made with the help of immunohistochemistry, especially the nuclear expression of STAT6. SFTs are often benign but there is a slight chance of malignancy. Patients with histologically malignant features may have worse prognosis. The current general consensus for the management of SFTs is long-term follow-up after surgical excision of the tumor. In our experience, we performed surgcial resection of the tumor via scrotal approach without additional interventions. 6-month follow-up with CT scan was conducted, we will continue the follow-up of this case to monitor long-term outcome of this rare disease.


A case of a short circuit (Retzius shunt) from the inferior mesenteric vein (IMV) to the inferior vena cava (IVC) without accompanying portal hypertension due to liver cirrhosis is rare.An 83-year-old woman who was followed after surgery for thyroid and breast cancer was incidentally found to have rectal cancer on computed tomography (CT). Preoperative three-dimensional CT showed a venous malformation forming a short circuit (Retzius shunt) from the IMV to the IVC. Laparoscopic anterior rectal resection was performed. Operative findings included the Retzius vein crossing the abdominal aorta and the inferior mesenteric artery (IMA) to the IVC and a number of engorged vessels in the mesentery. The Retzius vein and IMA were clipped without major bleeding, and tumor-specific mesorectal excision was then performed. The patient’s postoperative clinical course was good, and she was discharged without complications.Preoperative imaging enabled identification of an unexpected rare disease, thus reinforcing the importance of preoperative imaging. The veins of Retzius are rare and may be primary  or secondary due to portal hypertension or trauma, or iatrogenic. A case of a short circuit (Retzius venous short circuit) from the inferior mesenteric vein (IMV) to the inferior vena cava (IVC) without accompanying portal hypertension due to liver cirrhosis is extremely rare –3. The vA case in which an unexpected Retzius venous short circuit between the IMV and IVC was discovered during three-dimensional computed tomography (3D-CT) prior to surgery is presented. Using this information, laparoscopic anterior resection was safely performed. Preoperative 3D-CT is useful for understanding the anatomy to ensure a safe, precise operation. The present case study highlights the importance of preoperative imaging.The case of an 83-year old woman with a history of thyroid cancer, breast cancer, and rheumatoid arthritis is presented. She had no relevant family history. After surgery for thyroid and breast cancers, elevations of carcinoembryonic antigen and carbohydrate antigen 19-9 were observed. She had no abdominal tenderness, and no mass was palpable. Laboratory results were unremarkable. Colonoscopy showed a type 2 tumor localized in the upper rectum (Fig. Based on these findings, upper rectal cancer with a Retzius shunt from the IMV to the IVC was diagnosed. Laparoscopic anterior resection was performed. Laparoscopic observation showed a number of engorged vessels in the mesentery Fig. a and theThe present case highlights the usefulness of preoperative examination. Careful preoperative examination showed a rare venous malformation, which reduced the stress of the surgeons and improved operative accuracy.Retzius reported cases of a short circuit from the duodenum to the IVC and from the left colon to the left renal vein in 1835 , 3. The Preoperative risk assessment is an important aspect of surgical planning because it helps surgeons identify patients with an increased risk of a poor postoperative outcome . CT imagIn the present case, preoperative imaging enabled the discovery of an unexpected rare venous malformation. The information obtained from this preoperative examination was sufficient to enable adequate safety precautions and to perform curative resection with laparoscopic surgery, without any complications. The present case re-affirms the importance of preoperative diagnosis for patients with vascular abnormalities.


Primary chordoma of the nasopharynx is an extremely rare malignant tumor of notochordal origin in the extra-osseous axial skeleton. It presents as a soft tissue mass without involvement of the skull base bone (clivus) and may mimic other lesions of the nasopharynx. A 26-year-old male patient is presented with nasal obstruction and congestion for the last 3 years. Physical and radiological examination revealed a mass in the naso-oropharyngeal region. It was suspected to be a cystic mass or abscess on radiological imaging. However, histopathological examination revealed a chordoma. We review all 20 cases of primary nasopharyngeal chordoma reported previously in the literature. Nasopharyngeal chordoma should be considered in the differential diagnosis of nasopharyngeal mass due to its unspecific appearance on clinical and radiology examination. Chordoma is a rare malignant bone tumor that occurs in any site along the course of embryogenic notochord and typically in the axial skeleton . It affephysalis: bubbles) with vesicular nuclei embedded in a homogenous, intercellular substance. The vacuoles contain either mucinous substance or glycogen. The intercellular substance is considered as partly the result of cell secretion and in part of products of cell degeneration [Histologically, the hallmark of chordoma is the presence of large physaliphorous cells  but they were superficial and did not reach the tumor itself. Histologically, they showed nonspecific chronic inflammation. The patient was treated with antibiotic and anti-inflammatory drugs, without any clinical improvement.A deep biopsy from the mass was eventually obtained, and it revealed a chordoma of the nasopharynx. The patient underwent surgery to excise the mass with trans-palatal rhinotomy approach, under general anaesthesia. Approximately 30 cc of fragmented tissue was received. Macroscopically, the tumor tissue was firm and lobulated, some was covered by brownish white mucosa. Microscopic examination showed cords and lobules of physaliphorous cells embedded within extensive myxoid stroma, separated by fibrous septa. Some of tumor cells already invaded adjacent skeletal muscle. The physaliphorous cells were variable in size, with abundant eosinophilic to clear cytoplasm, some were vacuolated, with prominent vesicular nuclei. Other tumor cells were small with pyknotic nuclei. Mitotic figures were inconspicuous .The immunohistochemical studies revealed positive cytoplasmic staining for cytokeratin and vimentin. Meanwhile, expression of S100 was slightly weaker . ImmunosChordoma is a rare malignant bone tumor, primarily involving both ends of the axial skeleton, and arise from the remnant of the notochord. It was first described by Virchow in 1857 as a tumor made up of vacuolated or physaliphorous cells derived from rests of embryonic notochord along the midline central nervous system axis .The notochord is a rod-like aggregate of cells extending the entire length of the embryo on the midline, ventral to the developing neural tube. The embryonic notochord degenerates early in fetal development after being surrounded by sclerotome mesenchymal cells and remains as the nucleus pulposus within intervertebral disc. However, in some cases residual notochord cells remain outside the inter-vertebral disc and may become neoplastic .Primary nasopharyngeal chordoma arises in the extraosseous nasopharyngeal soft tissues and may or may not have a smaller intraosseous component along the course of the medial basal canal. The medial basal canal is considered the cephalad exit tract of the notochord as it moves from its intraclival location ventrally into the midline nasopharyngeal soft tissues . ChordomTo the best of our knowledge, only 20 cases of primary nasopharyngeal chordoma had been previously reported, with 5 of them presented without any bone involvement , 13–19. Primary nasopharyngeal chordoma commonly occurs in young adults with an average age of 39.6 years, ranging from 8 to 80 years. It affects males (57.1%) more than females (42.8%) with a male to female ratio of 1.3 : 1. The main clinical presentation is nasal obstruction, reported in 16 cases (76.2%). Other symptoms include hearing impairment, headache, difficulty in swallowing, dryness of the mouth, nasal bleeding, diplopia, numbness, dropping of upper eye lid, difficulty in breathing, and nasal speech. Almost all patients with primary nasopharyngeal chordoma show unremarkable laboratory findings and normal general physical examination. The tumor size ranged from 2.5 to 8.2 cm and all were lobulated. Chondroid chordoma was found in a single case with progressive manifestation within 3 months prior to diagnosis, and chondrosarcoma cannot be excluded in the differential diagnosis. Twenty cases were identified as classical chordoma (95.2%) and 6 cases (28.6%) showed slow growing tumors, with clinical manifestation observed within 6 months up to 5 years prior to the diagnosis. 5 cases had no further information regarding the onset of their clinical manifestation.Histopathologically, 20 cases (including the current case) were classical chordomas showing numerous physaliphorous cells arranged in cord or lobules with myxoid stroma separated by fibrous tissue, meanwhile 1 case was chondroid variant, showing chondroid differentiation resembling chondrosarcoma . RadioloIn all cases of reported nasopharyngeal chordomas, metastasis was not observed. However, there had been reports of cases of chordoma that metastasized to the peritoneum, pleura , liver , 24, lunThis case report was interesting on several counts. We experienced some difficulties during the pre-operative diagnosis due to the nonspecific features of chordoma on clinical and radiological examinations. Moreover, particular location of the tumor was difficult to be reached and biopsied. Those factors resulted in delayed accurate diagnosis. Because of the location in nasopharynx region, the differential diagnosis of chordoma should be made from other nasopharyngeal masses. Although CT and MRI features are nonspecific, they may be suggestive of chordoma including midline location, expansible or lobular soft tissue mass with well-defined margin. Other nasopharyngeal malignancies may destroy clival bone but do not demonstrate this midline tract. CT is ideal for evaluating the bony involvement, whereas MRI is useful in evaluating the surrounding soft tissues and extension into adjacent structures . ChordomPrimary chordoma of the nasopharynx is a rare but an important lesion to be considered when a midline nasopharyngeal mass is found with or without involvement of clival sinus tract. It has no specific appearance on clinical and radiological examination. Obtaining the right biopsy material is required for proper histopathological diagnosis of the tumor.


Even though mesh migration is a rare complication, it must be considered in the differential diagnosis when investigating abdominal pain and digestive complications in patients with history of abdominal operations. Patient history includes a well‐controlled hypertension (treated for 7 years). Surgical history includes an umbilical hernia, operated 5 years ago, as well as a recurrence of the umbilical hernia, treated as an incisional hernia, 2 years ago. Both operations were performed at another surgical department; as a result, information concerning the techniques used for the repairs was unavailable. The patient was scheduled for a recurrent hernia repair. Prior to surgery, she was otherwise asymptomatic, with no signs of bowel obstruction. Physical examination revealed a periumbilical defect of about 7 cm in the abdominal wall. Intraoperatively, an enteroenteric fistula in close relation to the abdominal wall was discovered, as well as a palpable hard mass which was located next to the fistula. Adhesions were also present. During adhesiolysis, the small intestine was incised and a mesh was discovered inside the lumen Figure . Partial3An incisional hernia is a common complication of abdominal surgery. The use of meshes is now the treatment of choice. A mesh facilitates the application of tension‐free techniques. Nonetheless, complications related to the artificial materials used have been reported. These include seroma, foreign body reaction, organ injury, infection, mesh rejection, fistulas, and mesh migration.Even though mesh migration is a rare complication, it must be considered in the differential diagnosis when investigating abdominal pain and digestive complications in patients with history of abdominal operations. Migration of mesh into the urinary bladder might present with hematuriaIn this case report, we describe a case of an asymptomatic ventral mesh migration which resulted in the erosion of the intestinal wall and the formation of an enteroenteric fistula. To our knowledge, this is the first case, an asymptomatic migration of mesh discovered as an incidental finding in a programmed hernia recurrence repair.As it has been already described by Aziz et al,None declared.IA: received the patient in our outpatient department. OT: was the principal surgeon. ET: drafted the manuscript and was also a major contributor in revising the manuscript. DK: revised the manuscript critically for important intellectual content. ET and IA: analyzed and interpreted the patient data. AS: assisted in drafting the manuscript. ET: served as an auxiliary surgeon and had significant contribution to conception and design of the manuscript. OT: was responsible for the overall treatment of the patient, revised critically the manuscript, and has given final approval of the version to be published. All authors read and approved the final manuscript.


Villous adenomas are dubiously benign lesions, which are difficult to interpret because of their malignant potential. Distal villous adenomas present with bleeding or mucus discharge. Giant adenomas are not amenable for endoscopic or transanal resection. Only few isolated cases have been reported by laparoscopic resection. We present our case of a circumferential giant villous adenoma of the rectum managed successfully by laparoscopic ultra-low anterior resection with colo-anal anastomosis with a review of literature in regard to their malignant potential.A 62-year-old lady presented with complaints of painless bleeding per rectum and a fleshy mass protruding from the anal canal which on digital rectal examination appeared a large soft velvety flat mass with mucus discharge. Colonoscopy showed circumferential irregular, friable, edematous mucosa in rectum extending for 15 cm. Computed tomography showed a large heterogeneously enhancing polypoid mass lesion in the rectal wall involving the entire rectum. The patient underwent laparoscopic low anterior resection with colo-anal anastomosis and protecting loop ileostomy. Histopathological examination of the resected specimen revealed villous adenoma of the rectum with moderate to severe dysplasia.Villous adenomas are sessile growths lined by dysplastic glandular epithelium, whose risk of malignancy is especially high up to 50% when greater than 2 cm in size. Large size, villous content, and distal location are all associated with severe dysplasia in colorectal adenomas. Large villous rectal tumors, particularly of circumferential type pose a great challenge for endoscopic or transanal removal. Henceforth, open or laparoscopic surgery is required for these cases.Giant rectal villous polyps are usually unresectable by endoscopic methods or transanal endoscopic microsurgery and are associated with a high rate of unsuspected cancer which requires a formal radical oncologic resection. As per current data, the combined risk of dysplasia/malignancy is about 83% with 50% risk of dysplasia and frank malignancy in 33% of cases of giant rectal villous adenomas of more than 8 cm in size. Laparoscopic colorectal resection is safe and effective. The prevalence of adenomatous polyps of the colon and rectum was reported in approximately 25% of the population aged over 50 years . A recenA 62-year-old lady presented to our outpatient department with complaints of painless bleeding per rectum from 8 months and a fleshy mass protruding from the anal canal from 3 months. The mass was reducible on manual palpation and associated with profuse mucus discharge. The hydration status of the patient was adequate, she had mild pallor and abdominal examination was essentially normal. On digital rectal examination, a soft velvety flat mass was appreciated about 3 cm from anal verge extending from 9 o’ clock to 5 o’ clock along with mucus discharge. The upper extent of the lesion could not be reached. The laboratory investigations revealed hemoglobin 8.3 g%, normal electrolytes with no other abnormalities; carcinoembryonic antigen (CEA) was also within normal range. Initial biopsy revealed villous adenoma with focal moderate dysplasia. A colonoscopy was done which showed irregular, friable, edematous mucosa in rectum extending for 15 cm nearly circumferentially with no other lesions in the entire colon. Contrast-enhanced computed tomography showed a large heterogeneously enhancing polypoid mass lesion in the rectal wall (max thickness 2.5 cm) involving the entire rectum   and protected by a proximal loop ileostomy. The patient recovered well and was discharged on the fourth post-op day. At the time of discharge, digital rectal examination revealed a preserved anal sphincter tone and no troublesome mucus discharge.On examination of the specimen, the lesion was seen to measure 16 × 12 cm, involving almost whole circumference with a 20 cm proximal and 1 cm distal margin Fig. . HistopaThe natural history of colorectal carcinomas has been extensively studied in correlation with the underlying accumulation of genetic alterations as understood by the adenoma-carcinoma sequence. Adenomas are precursor lesions defined by the presence of intraepithelial neoplasia, characterized by varying degrees of nuclear stratification and loss of polarity. Polyps develop as mucosal excrescence as a consequence of accelerated crypt fission resulting from APC gene mutation .The ‘villous tumor’ of the rectosigmoid was first described by Que’nu and Landel in 1899. They described very large broad-based rectal tumors associated with secretory diarrhea . The ageGiant polyps are usually defined as more than 3 cm on endoscopy , 11; howThe risk of malignant degeneration is related to both the size and type of polyp , 9, 10. A recent search on PubMed for keywords ‘giant rectal villous adenoma’ and ‘giant rectal villous tumor’ from 2005 to 2018 yielded 33 and 31 results respectively. The keyword ‘malignant potential of rectal villous adenoma’ yielded 24 results A total of 25 giant villous tumors (including our case) with size range 5–31 cm were reviewed which showed carcinoma (including invasive) in 8, high-grade dysplasia in 6, low- to moderate-grade dysplasia in 6 whereas rest negative for malignancy (Table In a study that analyzed 7590 adenomatous polyps to determine risk factors for high-grade dysplasia or invasion, the size was the strongest predictor. The percent of adenomas with high-grade dysplasia or invasive cancer based on the size of the polyp was as follows: <5 mm, 3.4%; 5–10 mm, 13.5%; and > 10 mm, 38.5% . Large sMost patients with adenomas are asymptomatic, especially when their neoplasm is identified by screening or surveillance , 9, 10. Colonoscopy is the procedure of choice for diagnosing colorectal polyps as it is the most accurate method for detecting polyps of all sizes, and it allows biopsy of lesions and resection of most polyps , 33, 34.Carditello et al. treated 104 villous tumors of the rectum with a mean size of 3 cm surgically by local or wide excision. The malignant potential of the tumors was 30%, including 10% invasive malignancy and recurrence rate was 24 after a mean follow up of 6.5 years . A recenGiant villous adenoma is a high-grade dysplastic lesion with conversion rate from adenoma to carcinoma approaching 17–33%. Troublesome mucus discharge and bleeding may result in severe hemodynamic alterations. As per current data, the combined risk of dysplasia/malignancy is about 83% with 50% risk of dysplasia and frank malignancy in 33% of cases of giant rectal villous adenomas of more than 8 cm in size. Giant rectal villous polyps are usually unresectable by endoscopic methods or transanal endoscopic microsurgery and are associated with a high rate of unsuspected cancer which requires a formal radical oncologic resection. Laparoscopic colorectal resection is safe and effective.


Wandering spleen (WS) is an uncommon congenital or acquired condition where the spleen is displaced from its normal position at the left hypochondrium to anywhere within the abdominal or pelvic cavity. The incidence is extremely rare in the geriatric population.We present a rare case of WS torsion in a 69-year-old elderly patient who presented with an acute abdomen. Physical examination revealed a tender right lower quadrant abdominal mass. Imaging studies confirmed the diagnosis of WS torsion with features of infarction. Subsequently, an emergency laparoscopic splenectomy was performed.A WS occurs due to the hypermobility of the spleen secondary to the absence or laxity of splenic suspensory ligaments. It is more commonly seen in children and adults in the third decade of life. Symptoms are usually attributed to the consequences of splenic vascular pedicle torsion. Exhibited symptoms might be unspecific; thus, radiological modalities are essential to determine the diagnosis and aid in planning its management. The treatment of choice is either open or laparoscopic splenopexy or splenectomy.Due to potentially life-threatening consequences and the rarity of such cases, a thorough history, detailed physical examination, and objective investigation are the pillars to attain a prompt diagnosis for appropriate management to be conducted as soon as possible to minimise complications. This ab[3][10]2We present the case of a 69-year-old female with WS torsion. She has complained of intermittent right lower quadrant abdominal pain since a month ago, and the pain worsened over the previous five days. She did not feel feverish and denied any gastrointestinal symptoms. She did not experience symptoms of urinary tract infection. No family history of connective tissue disease or malignancy was identified. She bore to two children three decades ago via vaginal deliveries. Last year, she underwent an open cystectomy for a symptomatic functional ovarian cyst.Upon examination, we noted an intra-abdominal mass at the right lower quadrant of the abdomen, measuring roughly 10 cm × 7 cm. The smooth-surfaced mass was mobile, firm, and tender upon palpation. Other physical examinations were normal.We proceeded with an abdominal ultrasonography, revealing a heterogeneous mass within the right abdominal cavity and absence of the spleen in its normal position . The feaHaemophilus influenza type b were given to the patient post-operatively.Soon after, an emergency exploratory laparoscopy was planned for the patient. Intra-operatively, the enlarged spleen was found in the right iliac fossa, displaced from the left hypochondrium . The pto3A WS is also known as a displaced, ectopic, pelvic, or ptotic spleen ,10,13,16The pathophysiology of WS is characterised by the hypermobility of the spleen secondary to the absence or excessive laxity of the following primary splenic suspensory ligaments: gastrosplenic, splenorenal, splenocolic, splenophrenic, pancreaticosplenic, and pre-splenic folds ,13,16. DMost patients with WS are asymptomatic . CommonlDue to the non-pathognomonic history and clinical manifestation, radiological modalities are paramount in attaining a definitive diagnosis . ImagingMoreover, 65% of asymptomatic WS patients treated conservatively developed complications, such as torsion, compression of other organs, and susceptibility of the spleen to trauma; thus, non-operative management is inadvisable ,6,7. A W[9]4Ultimately, due to potentially life-threatening consequences and the rarity of such cases, a thorough history, detailed physical examination, and objective investigation are the pillars to attain a prompt diagnosis of WS for appropriate management to be conducted as soon as possible to minimise complications.No source of fundingEthical approval has been exempted by our institution as this publication is a case report and not a randomized trial or a case series, provided that the patient gave her written consent both for operation and the publication of this case.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.Novia A. Nastiti is the first author of this paper. Muhammad S. Niam is the surgeon in charge. Novia A. Nastiti and Muhammad S. Niam were involved in the surgery. Phong Jhiew Khoo is the corresponding author and performed critical revision to this paper. Novia A. Nastiti, Muhammad S. Niam, and Phong Jhiew Khoo participated in the writing of this paper. All authors read and approved the final manuscript.Not applicable.Novia A. NastitiMuhammad S. NiamPhong Jhiew KhooNot commissioned, externally peer-reviewed.No conflicts of interest.


Undifferentiated pleomorphic sarcoma is a very rare and aggressive type of primary cardiac tumors. Most cardiac sarcomas result in rapid growth and quick death. According to different sources the median survival is typically 6 to 12 months. We are presenting a case of primary cardiac sarcoma with 26 months disease free survival following cytoreductive surgery and chemotherapy.A 48-year-old woman with progressing symptoms of dyspnea and palpitations for over 2 months was referred to a cardiologist. With the help of echocardiography and cardiovascular magnetic resonance cardiac sarcoma was suspected. Open biopsy and cytoreductive surgery were performed, complete resection of the tumor was not possible. Histology revealed undifferentiated pleomorphic sarcoma. Seven cycles of chemotherapy with Doxorubicine and Ifosfamide were completed. Cardiovascular magnetic resonance revealed a complete response – only signs of fibrosis without any signs of tumor were visible. Follow ups with echocardiography, cardiovascular magnetic resonance and chest, abdomen and pelvic computed tomography is performed every 3 months. Twenty-six months from initial diagnosis the patient is still free of recurrence of tumor with no compromises of the quality of life.Standard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival. Undifferentiated pleomorphic sarcoma (UPS), also known as malignant fibrous histiocytoma is an extremely rare type of primary cardiac tumor , 2. AccoA 48-year-old female with progressing symptoms of dyspnea and palpitations for over 2 months was referred to cardiologist. Her medical history was unremarkable, she only had allergy to various chemical substances as she was working as a tailor. Auscultation of the heart revealed a loud systolic murmur throughout entire precordium with irradiation to the left shoulder blade. Hemodynamic parameters were normal, while the ECG showed left ventricular hypertrophy (LVH). Laboratory findings revealed a normocytic normochromic anemia  and elevated level of lactate dehydrogenase (LDH) without liver or renal dysfunction.Transthoracic echocardiography (TTE) was performed. Several large masses in the left ventricle (LV) close to anterior and anterolateral mid-ventricular and apical segments were observed with one tumor (approximately 2.0 cm in length) partially obstructing LV outflow tract. Additional smaller tumor was seen in left atrium (LA) attached to the interatrial septum (IAS).Cardiovascular magnetic resonance (CMR) was performed to assess specific characteristics of masses using a 1.5 T scanner  with an 18-channel phased array coil. Cine images  were given. According to CTCAE (Common Terminology Criteria for Adverse Events) only I grade neutropenia and thrombocytopenia were observed. Totally, 7 cycles were completed . Echocardiography and CMR were performed to assess the results of chemotherapy treatment and revealed a complete response – only signs of fibrosis without any signs of tumor were visible in CMR images : 25.1 in 1973 to 1989, 30.2 in 1990 to 1999, and 46.6 in 2000 to 2011. During those years the incidence of sarcomas and lymphomas increased and the incidence of mesotheliomas decreased [Most sarcomas grow very fast, and cause death through widespread infiltration of the myocardium, obstruction of blood flow through the heart, and/or distant metastases. Even with complete resection, most patients develop recurrent disease rapidly, thus according to different sources the median survival is typically 6 to 12 months . The 32-UPSs are aggressive and locally invasive tumors, frequently making complete surgical excision unfeasible, which leads to a poor prognosis and a low survival rate . RecentlNeoadjuvant or adjuvant chemotherapy has been used to improve outcomes of the poor results with resection alone. Adjuvant chemotherapy with doxorubicin and ifosfamide is usually recommended postoperatively . A studyThe case we presented is unique because without radical surgery and after chemotherapy the patient got into complete remission which lasts already 26 months. This already overcomes all median survival rates cited in literature even with the complete resection of tumor. We believe this is due to aggressive multidisciplinary approach.The review is limited because the disease is very rare, the data are scarce, mostly case reports and experiences of single institutions are published. Therefore, individual approach to every case is extremely important and treatment options should be discussed thoroughly in the multidisciplinary team.


Diffuse cavernous hemangioma of the rectum (DCHR) is a relatively rare disease. A 40-year-old man presented with long-standing lower abdominal discomfort and hematuria. At the time of hospitalization, his vital signs and hemoglobin level were normal. Colonoscopy showed markedly dilated blood vessels in the sigmoid mucosa, which was confirmed on magnetic resonance imaging and computed tomography as cavernous hemangioma. Without surgery, there have been no signs of progression of DCHR during a 3-year follow-up period. Diffuse cavernous hemangioma of the rectum (DCHR) is a rare benign vascular tumor that is characterized by delayed diagnosis.We report a case of a 40-year-old man who was admitted to our hospital due to long-standing lower abdominal discomfort and hematuria. He had no history of fever, abdominal pain, intermittent rectal bleeding, or weight loss. He also had no notable medical history or family history. His vital signs were normal and laboratory testing revealed a normal complete blood count without anemia.Colonoscopy showed markedly dilated blood vessels in the sigmoid mucosa Fig. . AbdominDCHR is a rare clinical condition, which mainly affects young adults. Since the first case of rectal hemangioma reported by Phillips in 1839, there have been only approximately 350 cases reported worldwide . HoweverAlthough this disease presents with nonspecific symptoms, colonoscopy and abdominal CT and MRI can reveal specific findings that can serve as diagnostic clues; these typical and characteristic findings are irregularly thickened rectal wall and multiple calcified foci of pelvic phleboliths. In DCHR, biopsy is contraindicated because of the high risk for bleeding; the findings on CT or MRI are usually sufficient to make the diagnosis . MultiplGenerally, complete surgical resection of DCHR is the only treatment and most cases are operated to control rectal bleeding . AlternaIn conclusion, colonoscopy, abdominal CT and MRI are useful to make a diagnosis of DCHR, and some case, especially those without a history of bleeding, could be followed-up efficiently without excessive invasive examinations.


Background: Correct preoperative diagnosis of hepatobiliary disease can be challenging—even with current advances in radiographical imaging, laboratory testing, and endoscopic evaluation.Case presentation 1: A 65-year-old female with painless jaundice and weight loss was found to have cholelithiasis complicated by the Mirizzi syndrome.Case presentation 2: A 71-year-old female with new-onset painless jaundice and impacted stone in the gallbladder neck was found to have a cholangiocarcinoma.Case presentation 3: A 70-year-old male with progressive painless jaundice and weight loss was found to have a pancreatic adenocarcinoma.Conclusion: Proper diagnosis and management of patients with painless jaundice can be difficult in the preoperative setting and may require surgical exploration to obtain a definitive diagnosis. At times, additional highly invasive diagnostic maneuvers, such as diagnostic laparoscopy or even open exploration, are required. Herein, we report three interesting cases with similar presentations and diagnostic evaluations, but vastly different underlying pathologies and management strategies.Insidious obstruction of the extrahepatic bile duct is a common cause of painless jaundice in adult patients. The differential diagnosis of posthepatic biliary obstruction is broad. Malignant causes include periampullary cancers of the pancreas or duodenum, primary cholangiocarcinoma, ampullary carcinoma, gallbladder carcinoma, lymphoma, or extrinsic compression from another gastrointestinal malignancy. Benign causes of painless jaundice often mimick carcinomas and include bile duct strictures secondary to pancreatic pseudocysts or chronic pancreatitis, sclerosing cholangitis, external compression by gallstones in the neck of the gallbladder (Mirizzi syndrome), or intraductal parasites—such as liver flukes or ascariasis. Advances in laboratory, imaging, and endoscopic techniques have improved preoperative diagnostic capabilities.A 65-year-old female presented with fatigue, 20 lb weight loss, and intermittent painless jaundice. Initial laboratory investigations revealed an elevated bilirubin of 9.8 mg/dL , alkaline phosphatase of 977 U/L , and carbohydrate antigen (CA) 19-9 of 267 U/mL . Abdominal ultrasound revealed cholelithiasis with possible central biliary tree dilation. Endoscopic retrograde cholangiography (ERC) with sphincterotomy showed a malignant-appearing stricture in the common hepatic duct but yielded equivocal brushings and biopsies . A laparDuring open surgical exploration, the gallbladder was markedly inflamed with dense adhesions to the duodenum, without signs of disease dissemination. On dissection of the gallbladder, an obvious fistula was identified between the infundibulum of the gallbladder and the biliary bifurcation. Intraoperative frozen section analysis was negative for malignancy. After resection of the damaged extrahepatic bile ducts, separate right and left hepatic ducts remained. Reconstruction was performed with bilateral hepaticojejunostomies to a Roux-en-Y segment of the jejunum. Pathological analysis  of the sA 71-year-old previously healthy female presented with 2 weeks of painless jaundice. Ultrasound evaluation showed dilation of the intrahepatic and extrahepatic bile ducts as well as an impacted calculus in the gallbladder neck. Computed tomography (CT) and MRCP  confirmeOn operative exploration, a gallstone was found impacted in the neck of the gallbladder. However, a dense mass was also found at the junction of the gallbladder and the bile duct, encasing the right hepatic artery and portal vein—intraoperative frozen section revealed adenocarcinoma. Given the presence of vascular encasement, the most effective palliative measure to drain the biliary tree was determined to be resection of the damaged and partially obstructed bile ducts and performance of a palliative hepaticojejunostomy bypass. Pathological analysis of the resected specimen revealed a moderately differentiated adenocarcinoma, involving the cystic and common hepatic ducts, perineural and angiolymphatic invasion, and metastatic carcinoma in one station 12 lymph node . After rA 70-year-old male with a history of smoking and long-standing diabetes presented with a 60 lb weight loss, fatigue, constipation, and painless jaundice. Initial MRCP showed significant intrahepatic and extrahepatic biliary ductal dilation. In addition, it also showed a dilated pancreatic duct and ill-defined hypoechoic mass within the head of the pancreas . The patThe patient underwent operative exploration and was found not to have any evidence of disseminated disease. A firm mass was noted involving the head and uncinate process of the pancreas. He underwent a cholecystectomy and pylorus-preserving pancreaticoduodenectomy with standard reconstruction with an invagination pancreaticojejunostomy, hepaticojejunostomy, and end-to-side duodenojejunostomy. Pathological analysis revealed a successful R0 resection of a T3N1M0 moderately differentiated invasive pancreatic ductal adenocarcinoma, invading into the peripancreatic soft tissue, ampulla, duodenal wall, and bile duct . The patPainless obstructive jaundice has a wide differential that may require open exploration to establish a definitive diagnosis. These three cases illustrate the importance of preoperative counseling and exhaustive evaluation by an interdisciplinary group of endoscopists, radiologists, and surgeons. The etiology of jaundice, a clinical presentation of hyperbilirubinemia, can be categorized as prehepatic, hepatic, or posthepatic. Prehepatic causes of jaundice include spherocytosis, Gilbert's syndrome, sickle cell disease, or thalassemia major. Intrahepatic causes include hepatitis—viral, alcoholic, or drug induced—alcoholic cirrhosis, or primary biliary cirrhosis. Posthepatic jaundice is caused by an interruption of biliary drainage by either intrinsic or extrinsic obstruction.1 They are effective, however, in monitoring for recurrence after initial treatment with resection, chemotherapy, or radiation. Following relief of obstruction, CA 19-9 levels will typically normalize. If they remain elevated, this may indicate an underlying malignant pathology.The accurate diagnosis of the etiology of obstructive jaundice first requires a thorough history and physical examination, focusing on timing and duration of gastrointestinal symptomatology, including pain, nausea, vomiting, and change in bowel habits. Jaundice is often first recognized by friends or family members, rather than the patients themselves. Laboratory studies, including cell counts, blood chemistries, liver function tests, and pancreatic enzymes, are important to target the level of the pathology. If malignancy is suspected, tumor markers—such as alpha-feto protein, CA 19-9, and CEA—are useful in diagnosis, although it must be recognized that they have low sensitivity and specificity.Cross-sectional imaging techniques, such as CT and MRCP, are essential to elucidating the cause of posthepatic jaundice. MRCP is particularly useful in delineating biliary anatomy. Endoscopy has played a growing role in diagnosis—through ERC, cholangioscopy, and biopsy—and treatment—through stone extraction, sphincterotomy, and stent placement. With regard to diagnosis, MRCP and ERC have comparable sensitivity, specificity, and accuracy for distinguishing malignant from benign bile duct strictures. Nearly a quarter of biliary strictures are the result of a benign etiology, emphasizing the importance of stricture characterization and tissue biopsy. A lengthy stricture with irregular borders or a “shelf sign” is more suggestive of malignancy, whereas a short stricture with regular borders and a smooth taper indicates a benign stricture. A stricture associated with Mirizzi syndrome is usually long and regular, whereas strictures from autoimmune processes such as primary sclerosing cholangitis are often multifocal.1 ERCP is the diagnostic modality of choice but has limited accuracy ranging widely from 55% to 90% in the published literature.1 Elevated levels of CA 19-9 are not common given the underlying biliary obstruction, further confounding the diagnostic dilemma. Of note, on stenting the patient in Case 1, tumor markers returned to within the normal range, whereas in Case 3, they remained elevated. The preoperative diagnosis of Mirizzi syndrome is important when it is possible to aid in surgical planning. Mirizzi syndrome is often differentiated from malignant causes of obstructive jaundice by symptomatology related to the underlying chronic cholecystitis, emphasizing the importance of a thorough history. However, even in retrospect, the patient in Case 1 was unable to describe any of these symptoms.Biliary obstruction caused by compression of the biliary tree due to impacted gallstones in the neck of the gallbladder is a syndrome first described by Pablo Luis Mirizzi in 1948. Mirizzi syndrome is a rare complication, affecting 0.5–2.5% of patients with gallstone disease. Mirizzi syndrome can lead to the development of a cholecystobiliary fistula. Type 1 Mirizzi syndrome describes external compression without development of a fistula, whereas types II–IV describe progressive levels of severity of these fistulae.2Correct diagnosis and effective surgical management of Mirizzi syndrome are especially pertinent, considering the strong association with gallbladder carcinoma. Several retrospective and prospective studies have shown an increased incidence of gallbladder carcinoma in Mirizzi patients (5.3–11%) compared to patients with uncomplicated gallstone disease (1%). Some of these malignancies are only identified at the time of final pathological analysis. Although this association has yet to be completely characterized, it is critical to keep in mind when suspecting a possible Mirizzi syndrome case.3 Aggressive radical resection has long been the mainstay of therapy; however, only 30% of these cases will qualify for surgical intervention at the time of diagnosis. In addition, given the lack of effective systemic therapy, many patients have a poor prognosis. Identifying patients early who are candidates for margin negative resection is important for long-term survival. Even with surgical resection, 5-year survival for extrahepatic cholangiocarcinoma is only 27–37%. Liver transplantation is a controversial and rarely used therapy for cholangiocarcinoma.Gallbladder cancers and cholangiocarcinoma are relatively rare, accounting for only 0.6% (9810) of new cancer diagnoses in 2012.3 As is the case for distal cholangiocarcinoma, curative therapy for pancreatic cancer is only achieved through radical resection through a pancreaticoduodenectomy. However, only 20–30% of patients qualify for resection at the time of diagnosis because of the prevalence of locally advanced and distant disease spread in the majority of patients. Even with successful resection and adjuvant therapy, survival rates remain low at 15–25% at 5 years.Pancreatic cancer is slightly more common, 2.7%  of new cancer diagnoses in 2012, but more importantly, it is the fourth leading cause of death from cancer.4As in the three cases described here, open surgical exploration for patients with obstructive jaundice may be required to obtain a definitive diagnosis. Laparoscopic approaches in this situation have limited usefulness and have been associated with increased morbidity and mortality. Conversion to an open procedure is often required to safely identify the portal structures. A prospective study of 13,023 cholecystectomy patients at the Swiss Association for Laparoscopic and Thoracoscopic Surgery revealed a conversion rate of 74% for Mirizzi syndrome patients.Management of Mirizzi syndrome requires a multidisciplinary approach of palliative endoscopic stenting and definitive surgical therapy through open cholecystectomy, removal of stones, and, in the presence of fistulas, biliary enteric anastomosis. Management of cholangiocarcinoma and pancreatic adenocarcinoma necessitates assessment of resectability and risk stratification for surgical management. However, even with the advancements in diagnostic imaging, proper diagnosis and management of these patients may often require open exploration and astute intraoperative decision-making.


Simultaneous spontaneous bilateral quadriceps tendon rupture is a rare orthopedic injury; its initial diagnosis is misdiagnosed in up to 50% of patients with secondary hyperparathyroidism. Early diagnosis and surgical repair are important to achieve an excellent functional outcome.We report a case of simultaneous spontaneous bilateral quadriceps tendon rupture associated with secondary hyperparathyroidism.Magnetic resonance imaging showed that the quadriceps tendon was completely ruptured at the osteotendinous junction. We then found bilateral quadriceps tendon rupture during the operation.The patient underwent successful tendon repair surgery.The 31-year-old female patient regained full active movement of both knee joints and was able to participate in her activities of daily living.Simultaneous spontaneous bilateral quadriceps tendon rupture in a patient with secondary hyperparathyroidism  is a rare orthopedic injury that can be easily overlooked at the initial presentation. Early diagnosis and surgical repair is important to achieve an excellent functional outcome. For patients with secondary hyperparathyroidism receiving hemodialysis, strict systematic treatment of hyperparathyroidism is needed to prevent rupture or re-rupture of the quadriceps tendon. Although the exact mechanism responsible for the rupture is controversial, it is primarily affected by secondary hyperparathyroidism associated with long-term hemodialysis treatment. Timely surgical repair is the recommended standard treatment, which leads to superior outcomes compared to delayed repair. Herein, we report a case of spontaneous concurrent bilateral rupture of quadriceps tendon in a patient with secondary hyperparathyroidism who was receiving hemodialysis.Spontaneous simultaneous bilateral quadriceps tendon rupture is a rare orthopedic injury in patients with secondary hyperparathyroidism who are receiving hemodialysis. The initial diagnosis of this injury is missed in up to 50% of cases. Moreover, it is complicated by concurrent use of steroids, quinolones, and statins2Informed written consent was obtained from the patient for publication of this case report and accompanying images. Ethics committee approval was waived because this is a case report.A 31-year-old female was admitted to the Department of Orthopedics in November, 2017, complaining of an inability to actively extend her bilateral knees. During detailed questioning, she stated that both her knees were “giving way” while she was walking downstairs since 6 months ago. Since then she was unable to bear weight without help and to actively extend both knees. She was sent to our emergency room for X-ray examinations; however, there was no obvious finding after viewing plain radiographs of both knees. She was diagnosed with ligament damage and both knees were immobilized to the extension position. She had no history of injury or long-term use of medications. Two weeks later, she presented to the Department of Orthopedics and again left without a clear diagnosis. The patient had received hemodialysis at our hospital over the past 5 years due to uremia.After admission to our department, a detailed physical examination showed a palpable gap above in both suprapatellar regions Fig. A. BilateA diagnosis of bilateral quadriceps rupture and secondary hyperparathyroidism was made. A longitudinal incision was made overlying the patella and quadriceps tendon of each leg. Complete bilateral rupture between the quadriceps tendon and patella was identified at the osteotendinous junction Fig. B.We debrided the frayed soft tissue, refreshed the tendon ends, and roughened the proximal upper pole of the patella. Two vertical bone tunnels in the left patella and 3 vertical bone tunnels in the right patella were created. Two anchors (following drilling) were implanted into the left patella and 3 anchors into the right patella. Quadriceps tendons were sutured to the patella using the same technique used for Bunnel suture in full extension. The transverse diameter of the 4.5 mm tunnel was established on the upper third of the patella using the tunnel guide. We took the autologous semitendinosus and gracilis tendon and passed them through the transverse tunnel. Meanwhile, we established the tendon tunnel at the quadriceps tendon, inserted the transplanted tendon into the tunnel, and strained and sutured the ends of the transplanted tendon.Reinforcement suturing was eventually carried out with a nonabsorbable suture between the transplanted tendon and surrounding tissue. Intraoperatively, both knees could be flexed up to 110° without causing tension to the sutured quadriceps tendon. Postoperative T2-weighted sagittal magnetic resonance images of bilateral knees revealed normal continuity of the repaired quadriceps tendon with the superior pole of the patella  on the Medical Research Council scale. The patient regained full active movement of both knee joints and was able to participate in her activities of daily living it is a rare orthopedics injury and the diagnosis of quadriceps tendon rupture may not be initially considered;(2)owing to the presence of swelling and hemarthrosis, a palpable suprapatellar gap may not be identified and knee pain could be attributed to many different causes;(3)no obvious trauma observed at the time of rupture; and(4)when the rupture is bilateral, no obvious difference was observed in comparative physical examination.To avoid a misdiagnosis of this rare disease, imaging techniques such as MRI and ultrasound can assist in confirming the diagnosis. MRI is the most preferred method to investigate tendon rupture despite its high cost and limited availability in less developed countries. MRI was used to confirm the diagnosis preoperatively and the good continuity of repaired tendon postoperative before the patient was discharged. Ultrasonography is a less expensive and important tool, which can be quickly and easily obtained, but is operator dependent.,10 We reattached the ruptured tendon onto the patella to recover the continuity of quadriceps tendon by using nonabsorbable sutures or an anchor and we used autologous semitendinosus and gracilis tendon to further reduce tension and enhance strength.Surgical repair is the recommended standard treatment for complete rupture of bilateral quadriceps tendon and early surgical repair will result in superior outcomes compared to delayed repair.In a patient with secondary hyperparathyroidism receiving hemodialysis, strict treatment for hyperparathyroidism is needed to prevent further spontaneous tendon rupture. Total parathyroidectomy , early use of vitamin D analogs, phosphate binders, and calcimimetics are recommended to prevent future tendon rupture. For patients who refused treatment for hyperparathyroidism, we should pay attention to the occurrence of further spontaneous tendon rupture and infection. In our case report, the incision took almost 6 months to heal.In summary, spontaneous simultaneous bilateral rupture of quadriceps tendon in a patient with secondary hyperparathyroidism undergoing hemodialysis is a rare orthopedic injury that can be easily overlooked at the initial presentation. This case report emphasized the importance of diagnosing quadriceps tendon rupture early. Early diagnosis and surgical repair are important to achieve an excellent functional outcome. For patients with secondary hyperparathyroidism receiving hemodialysis, strict systematic treat measures of hyperparathyroidism are needed to prevent rupture or re-rupture of the quadriceps tendon.Data curation: Jianwei Ruan, Yang Huang.Formal analysis: Jianwei Ruan, Haibao Wang, Wenbiao Zheng.Funding acquisition: Haibao Wang.Investigation: Weiqian Wu, Chongyang Wang.Methodology: Weiqian Wu, Chongyang Wang, Yang Huang, Fanghu Chen.Project administration: Weiqian Wu, Wenbiao Zheng, Fanghu Chen.Supervision: Chongyang Wang.


Primary angiosarcoma of the central nervous systemis unusual.We encountered a case of radiation-induced angiosarcoma of the brain. A 65-year-old male was referred to our hospital with drowsiness for the last 6 months. He had undergone radiation therapy for pituitary adenoma 43 years ago. An MRI revealed a right temporal lobe tumour that consisted of a well-demarcated haemorrhagic lesion and an avid contrast enhancement, with significant vasogenic oedema. Surgical resection was performed and a post-operative pathological diagnosis of an angiosarcoma was made. A Thorotrast-associated angiosarcoma has been, hitherto, the only reported case of radiation-induced angiosarcoma of the brain. We present an extremely rare case of primary angiosarcoma of the brain, occurring after external beam radiotherapy. He had presented to another hospital 6 months earlier with drowsiness and a 54-mm diameter mass had been observed in the right temporal lobe on non-contrast CT scan. The patient had undergone surgery and cobalt-60-based external beam radiotherapy for pituitary adenoma 43 years ago. Parallel–opposed lateral radiotherapy targeting the pituitary lesion was employed with a total dose of 50 Gy in 25 fractions. The patient had recovered without residual symptoms but required hormone replacement for panhypopituitarism. He had also developed pontine infarction at 57 years of age. The results of physical and neurological examinations at the time of admission were normal. Laboratory evaluations of complete and differential blood counts, and serum chemistry were also normal.T2 weighted imaging. There was extensive vasogenic oedema around the mass. The T1 weighted image showed a hypointense mass with hyperintense areas in the periphery, suggesting haemorrhagic foci. Contrast-enhanced T1 weighted image of the brain showed significant contrast enhancement in the hypointense area on the pre-enhanced T1 weighted image. The mass was supplied by the inferior branch of the right middle cerebral artery, and conventional angiography showed spotty stains in the arterial phase and spreading and pooling of the contrast medium in the venous phase, which suggested the presence of blood sinus-like structure in the mass  . These pComplete surgical resection was achieved and no residual tumour was evident on the postoperative MRI . No surg7 Males (65%) were more frequently affected than females (35%).6 Most angiosarcomas of the CNS occur in the brain parenchyma and a few in the meninges. Patients range in age from neonates to the elderly. There is, usually, a rapid onset of neurological symptoms that relate to the tumour’s location.Primary angiosarcoma of the CNS is extremely rare, with only 31 cases reported in the literature.8 Radiation-induced angiosarcoma of the brain was reported in a single case of Thorotrast-associated angiosarcoma.2 Thorotrast, which was used as a contrast medium in the 1930s and 1940s, contains particles of the radioactive compound thorium dioxide.2 To the best of our knowledge, an angiosarcoma of the brain arising after external beam radiation therapy has not been reported.Although the aetiology of CNS sarcoma remains unknown, various possible aetiologies have been proposed, such as previous radiation therapy.4 The CT scan and MRI findings of our case too were similar to those reported previously.4 Although angiographic findings of CNS angiosarcoma have not been reported previously, hepatic angiosarcoma was reported to show a peripheral tumour stain, and the appearance of puddling during the mid-arterial phase that persisted for as long as 34 s.9 A peripheral enhancement was not obvious in our case, but contrast medium pooling, similar to puddling, was observed. The differential diagnosis of an angiosarcoma of the brain includes assessments for cavernous malformation,7 glioblastoma and metastasis. Particularly, cavernous malformation and glioblastoma, similar to angiosarcoma, can also arise secondarily to radiation therapy.11 An angiosarcoma is thought to be more progressive and more strongly enhanced than a cavernous malformation. It may have more demarcated margins compared with a glioblastoma. Brain metastasis is determined based on clinical history and further careful exploration of the whole body.Imaging studies of CNS angiosarcoma have demonstrated a haemorrhagic, well-demarcated lesion with avid contrast enhancement and significant vasogenic oedema.5While the primary therapy for CNS angiosarcoma is surgical resection, a few patients have benefited from radiation therapy and/or chemotherapy too. Although chemotherapy for angiosarcoma in the CNS is not established, the use of temozolomide, doxorubicin, paclitaxel and bevacizumab, or their combination, has been reported.1 The remaining two young patients were alive at 39 and 102 months after surgery.An angiosarcoma of the brain is characterized by a high rate of local recurrence and a short median survival time. In one series of seven patients, four died within 4 months of surgery and one after 30 months.Although an accurate preoperative diagnosis of an angiosarcoma of the brain is difficult, radiologists and neurosurgeons need to be aware that it may occur long after external beam radiotherapy.Angiosarcoma of the brain may occur long after external beam radiotherapy.Angiosarcoma of the brain shows a haemorrhagic mass with avid contrast enhancement and significant vasogenic oedema.Contrast medium pooling on angiography may be a characteristic finding of angiosarcoma.


Calcifying nested stromal-epithelial tumor (CNSET) is an extremely rare, indolent tumor of the liver of uncertain cellular origin. With only 38 cases reported in the literature, pathogenesis and optimal therapeutic approach are not well characterized. Based on the available literature, the risk of recurrence is low with surgical resection with negative margins.In this case report, we describe an adolescent patient with CNSET who underwent right trisectionectomy.In order to avoid posthepatectomy liver failure, special consideration must be given to the amount of residual liver parenchyma after resection when considering surgical approach. Single stage right trisectionectomy and two stage via associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) were both considered for surgical approach in this case in order to provide sufficient functional liver parenchyma remnant.Given that obtaining negative margins is important in reducing the risk of recurrence, the method of surgical resection utilized is based on the amount of future functional residual hepatic parenchyma. It is characterized by nests of epithelioid and spindle cells with a desmoplastic myofibroblastic stroma and variable calcification and ossification. There have been 38 cases reported in the literature, with the tumor occurring most frequently in women  ,5. Treat2A 15 year old athletic female with past medical history of asthma presented to the emergency department with a 4 day history of acute onset right abdominal pain and shortness of breath. She had been in her usual state of health prior to this, maintaining an active lifestyle. She did not have significant change in weight or appetite. She did not endorse nausea, vomiting, fever or chills.The patient had no history of tobacco, alcohol, or illicit drug use. The results of laboratory workup were unremarkable. It revealed an ALT 12 U/L, AST 19 U/L, Alkaline Phosphatase 84 U/L, TB 0.2 mg/dL, INR 0.94 and an Albumin 4.6 g/dL.Radiographic imaging was obtained at her initial presentation. An abdominal ultrasound revealed a large heterogenous mass in the liver. An abdominal computerized tomographic (CT) scan revealed a mass with scattered calcifications and a central non-enhancing region (10.8 × 13.7 × 12.8 cm) in the right hepatic lobe predominantly in segments 7 and 8, along with ovarian cysts. A triphasic scan showed a hypodense lesion which demonstrated heterogenous enhancement on arterial phase imaging . No obviTwo options for surgical resection were discussed. The first, a single stage right trisectionectomy due to possible involvement of the middle hepatic vein, would be the preferred approach as it is a single operation and the potential for regeneration is high given the patient’s age. The second option would require two stages and would be utilized if the first option were not feasible based on the size of the FLR. The second option would require portal vein embolization (PVE) or ALPPS.A volumetric rendering of the patient’s liver and mass (MeVis scan) were obtained in order to best approximate residual hepatic parenchyma following resection of the mass . Based oUpon exposure via Muakuchi incision, the liver was found to be of normal color and normal surface with sharp edges and no signs of fibrosis. The rest of the abdomen had no evidence of tumor burden. Following mobilization of the right and caudate lobes of the liver from the inferior vena cava, intraoperative ultrasound allowed for identification of the tumor in contact with the middle hepatic vein occupying a large portion of the right side of the liver. It was estimated that the patient would have greater than approximately 40 % of her functional liver parenchyma remaining given the volume occupied by the non-functional tumor. Thus, a right trisectionectomy of segments 1, 4, 5, 6, 7, 8 was completed to surgically resect the tumor and a two stage approach was not necessary. The tumor measured 15.0 × 12.6 × 10 cm with negative margins and no extracapsular extension . She rec3CNSET are extremely rare, large, indolent tumors of the liver of uncertain cellular origin. They have distinct histopathological appearance and a relatively invariable appearance on imaging studies. It is a nonhepatocytic and nobiliary tumor of the liver of uncertain histogenesis with no normal cellular counterpart. CNSET has been called by several other names in the literature, such as ossifying malignant mixed epithelial and stromal tumor, ossifying stromal-epithelial tumor, and desmoplastic nested spindle cell tumor . PatientThese tumors display consistent radiographic findings. They are large, well circumscribed, heterogenous masses that have dense calcification. This calcification varies from being located centrally to diffusely throughout the tumor . The difCNSET affects females to males in a ratio of 2.5:1 and most frequently involves the right hepatic lobe (65 % of cases) . TreatmeWhile a right trisectionectomy in a single operation was utilized in this case, an ALPPS was strongly considered due to the size of the tumor and concern for the amount of residual liver parenchyma following resection. This is a concern for an inadequate future liver remnant resulting in posthepatectomy liver failure after any liver resection. In cases where hepatectomy would result in an insufficient amount of remaining liver parenchyma, the options for staged hepatectomy include ALPPS, portal vein embolization and hepatectomy, and two-stage hepatectomy. ALPPS combines portal vein ligation and transection of the liver between the now deportalized portion and the planned remnant . The benAs there are few reported cased of CSNET, the optimal therapeutic approach is not standardized. Though the origin of these tumors is not known, the risk of recurrence is low with negative margins based on published reports. It is clear that surgical resection of these masses is the current treatment of choice if there is an adequate future liver remnant.No direct funding sources to report.As a case report, this article does not meet DHHS definition of research and thus does not require review by our institution’s IRB. Efforts were made by all authors to ensure compliance with HIPAA requirements.Written informed consent was obtained from the patient's parents 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.Nicholas Olin: primary authorAnkit Patel: writer and editorSusan S. Baker: editor, study designRoberto Hernandez-Alejandro: study design/concept initiatorN/A.Nicholas OlinAnkit PatelRoberto Hernandez-AlejandroNot commissioned, externally peer-reviewedNo conflicts of interest.