Juvenile musculoaponeurotic fibromatoses are benign tumors which arise from musculoaponeurotic stromal cells. They rarely occur in lower extremity and more rarely in children. They are locally invasive tumors with a high incidence of recurrence after surgery. Hence, wide local excision is the treatment of choice for such tumors. However, complex reconstruction is often required to cover the resulting soft tissue defect. This report presents a 12-year-old boy with a juvenile musculoaponeurotic fibromatosis in the anteromedial aspect of the upper third of a left leg. Following wide local excision, two local flaps, medial gastrocnemius and a distally based peroneal artery perforator flap, were used to reconstruct the soft tissue defect. Reconstruction has provided an acceptable functional and cosmetic result. Stout  first deThis report presents a case of juvenile musculoaponeurotic fibromatosis in a 12-year-old boy. Following a local wide excision, the defect was covered using a medial gastrocnemius flap proximally and a distally based peroneal artery perforator fasciocutaneous flap for the distal part. Since surgical margins were tumor free, no postoperative radiotherapy was required. Patient was followed for 2 years and no recurrence was observed.A 12-year-old boy presented with an 18-month history of swelling over the proximal part of left leg (Fig. A plain radiograph of the area revealed soft tissue expansion with no bony involvement Fig. . A MRI sThe patient was operated under spinal anesthesia and tourniquet control. The tumor was excised with 3-cm margin beyond the palpable edge of the tumor Fig. . The resHistopathological examination confirmed the diagnosis of a juvenile aponeurotic fibromatosis with negative margins and base. Hence, no post-op radiotherapy was required. No recurrence was recorded on follow-up after 2 years Fig. .Fig. 7FoJuvenile musculoaponeurotic fibromatoses are also termed as “aggressive fibromatoses.” These lesions encompass a wide variety of benign fibrous tissue proliferations having similar microscopic findings. The biological behavior of these tumors is intermediate between benign fibrous tissues and fibrosarcomas . These tCT and MRI are useful investigations to evaluate the size and extension of desmoid tumors. However, MRI is considered to be the investigation of choice for these tumors due its multiplanar imaging capabilities. It demonstrates the masses of low signal intensity relative to muscle on T1-weighted images. T2-weighted images show variable signal intensity relative to muscle .Wide local excision is indicated in the management of such tumors due to their local aggressive nature. The aim of surgery is to achieve a tumor-free margin to reduce the risk of recurrence and morbidity . The extThe presented case is a rare case of juvenile musculoaponeurotic fibromatosis in a 12-year-old boy. The case highlights the typical presentation of such tumors. The option of a free flap was excluded due to the available local options and the increased risks associated with such a major reconstruction if the more complex microvascular route was selected.It is important that the diagnosis and tumor-free margins are confirmed intraoperatively by frozen section analysis, but this facility is unavailable in our center. The rate of local control after surgery is variable and depends on the status of the margins of resection. A control rate of 72 and 41 % has been described in the literature for tumor-negative and tumor-positive margins, respectively. Addition of postoperative radiotherapy gives better results with a local control rate of 94 and 75 %, respectively, for tumor-negative and tumor-positive margins . Since tIt is important to widely excise Juvenile musculoaponeurotic fibromatoses to get a tumor-free margin. Appropriate surgical options should be chosen for adequate coverage, to get a functional limb and to achieve an acceptable cosmetic appearance. Due to its high recurrence rate, the patient must be kept on regular postoperative monitoring.


A 60-year-old man presented for evaluation of a mobile subcutaneous mass located on the proximal radial aspect of the right-hand small finger. The mass was tender on deep palpation, had a negative Tinel sign, was nonmobile in the axial direction, but was mobile in the volar/dorsal direction.What is the differential diagnosis of a soft-tissue hand mass?How do schwannomas usually present?How do schwannomas differ from neurofibromas and malignant peripheral nerve sheath tumor (MPNST)?How are schwannomas diagnosed and managed?Understanding the differential diagnosis of tumors of the hand is imperative for effective care. A detailed history and physical examination cannot be overemphasized. The differential includes the follow soft-tissue masses: ganglions, epidermal inclusion cysts, foreign-body granulomas, fibromas, tophaceous pseudogout, vascular aneurysms, vascular malformations, giant cell tumors of the tendon sheath, fibroma of the tendon sheath, lipoma, extraosseous chrondroma, leiomyoma, granular cell tumor, MPNST, and, finally, schwannomas. The most common soft-tissue and bone mass in the hand is a ganglion and an enchondroma, respectively.1-2-2-6Schwannomas, also known as neurilemmoma, are benign tumors originating from Schwann cells. Of the benign peripheral nerve tumors, which include schwannomas, neurofibromas, granular cell tumors, neurothekeomas, lipofibromatous hamartomas, or sclerosing perineuriomas, schwannomas are the most common. Schwannomas usually present in the third to fifth decades of life as solitary encapsulated lesions, which are commonly associated with neurofibromatosis type 2; however, incidence is reported as 0.62 per 100,000 population.2MPNSTs in the general population have a reported incidence of 0.001%; however, in patients with neurofibromatosis, 1 incidence is reported at 8% to 13%. When schwannomas and MPSTs are compared, MPSTs are more commonly found on the limb, are painful, and found deep to the fascia whereas schwannomas are more commonly found in the head, neck, and trunk, are painless, and are superficial to the deep fascia.1Diagnosis is typically made by history, physical examination, radiographic investigation, and final pathology after incisional versus excisional biopsy. Schwannomas are encapsulated and can be completely enucleated during surgery. Surgical excision is the most effective method of therapy while preserving the nerve function.6Clinical diagnosis of a schwannoma is not always straightforward due to the large differential diagnosis. Radiographic investigation such as MRI may be helpful in establishing a correct diagnosis. Treatment is achieved by enucleation with preservation of nerve function.


Solitary peripheral osteoma is a benign, slow-growing osteogenic tumor arising from craniofacial bones such as the sinus, temporal, or jaw bones but rarely originating from the mandible. Osteoma consists of compact or cancellous bone that may be of peripheral, central, or extraskeletal type. Peripheral osteoma arises from the periosteum and is commonly a unilateral, pedunculated mushroom-like mass. Solitary peripheral osteomas are characterized by well-defined, rounded, or oval radiopaque mass in the computed tomography. Although multiple osteomas of the jaws are a hallmark of Gardner's syndrome , nonsyndromic cases are typically solitary. Herein, we report a rare case of solitary peripheral osteoma of the angle of the mandible in a 27-year-old female with clinical, radiologic, and histopathologic findings. Solitary peripheral osteomas of facial bones are benign osteogenic tumors characterized by the proliferation of compact or cancellous bone. They originate from the craniomaxillofacial region such as temporal bones, sinuses, or maxilla or mandible. They are mostly seen occurring between 2nd and 5th decades, but they may be seen at any age. Three subtypes of osteomas are seen, peripheral , central , and extraskeletal (osseous choristoma osteoma) , 2.Histologically, osteomas may be of two types: (1) compact and (2) cancellous. Compact osteomas consist of dense, compact bone with a few marrow spaces, while cancellous osteoma is characterized by bony trabeculae and a fibrofatty marrow enclosing osteoblasts which resembles mature bone .The most common location of the peripheral osteoma is the skull. However, lesions do seldom occur in the mandible, especially on the lingual aspect, inferior border, and body of the mandible and at the angle region .Multiple osteomas of jaws are a hallmark of Gardner's syndrome , an autosomal dominant disease caused by a mutation in the APC tumor suppressor gene. In this syndrome, patient presents with multiple osteomas of the jaws along with multiple premalignant colorectal adenomas, which if left untreated progresses to colorectal carcinoma by middle age .In this case report, we present a case of a solitary peripheral osteoma arising at the angle of the mandible with its clinicopathological and radiological findings along with differential diagnosis and treatment plan.A 27-year-old female patient reported to the Department of Oral and Maxillofacial Surgery with the chief complaint of hard swelling in the posterior region of the mandible. She gave the history that the swelling was first noticed about 2 years back, was insidious in onset, and gradually increased to the present size.The swelling was not associated with any pain, discharge, fever, paresthesia, or difficulty in mastication. The patient did not give any history of trauma or infection in that region. On examination, a solitary, nonpulsatile, nontender, approximately 1.5 cm sized, bony hard mass was palpated at the right angle region of the mandible . The sweThe computed tomography (CT) scan showed a well-circumscribed, hyperdense image with a lobulated surface located on the right angle of the mandible . The 3D The lesion was completely excised using an extraoral approach under general anesthesia . MacroscAs solitary peripheral osteoma may be clinically silent for years without symptoms, it is usually diagnosed when it becomes enlarged or is incidentally discovered by radiological examination such as panoramic radiography or CT . In our Radiographically, the presence of an oval, radiopaque, well-circumscribed mass attached by a broad base or pedicle to the affected cortical bone is a hallmark of peripheral osteomas . SimilarThe pathogenesis of peripheral osteomas is unclear. Some investigators considered it a true neoplasm, while others classified it as a developmental anomaly. Peripheral osteomas exhibit continuous growth rather than growth cessation at adulthood, and this characteristic is the major feature distinguishing them from other bony exostoses supporting a neoplastic origin. In addition, the possibility of a reactive mechanism, triggered by trauma or infection, has also been suggested , 9. KaplGardner's syndrome should be suspected with the detection of facial osteoma. Gardner's syndrome may present with rectal bleeding, diarrhea, and abdominal pain and may be characterized with colorectal polyposis, multiple osteoma, skin and soft tissue tumors, and multiple impacted or supernumerary teeth . In our A peripheral osteoma can be completely treated by surgical intervention. Surgery consists of removing the lesion at the base where it enters the cortical bone. Extraoral approach is preferred for peripheral osteomas that are located in the posterior region of the mandible. Recurrence after complete excision is very rare and malignant transformation has not been reported in the literature , 12. In Even though the solitary peripheral osteomas are benign lesions, surgical excision is recommended. Though recurrence is not commonly reported, radiographic follow-up every 6 months for 2-3 years with annual radiographs thereafter is advised.


Background. Subcutaneous fat necrosis (SFN) is a rare, temporary, self-limited pathology affecting adipose tissue of full-term or postmature neonates. It is a rare entity especially in Nigeria and usually occurs in the first weeks following a complicated delivery. Because it is not very common, diagnosis is easily missed. It may resolve spontaneously without sequelae but patients need to be followed up because of development of late complications especially hypercalcemia. We report a case of SFN of the newborn noted within one week of birth and highlight the need for proper prompt diagnosis and the need for follow-up to assess possible complications. Subcutaneous fat necrosis (SFN) is a rare, temporary, self-limited pathology affecting adipose tissue of full-term or postmature neonates , 2. It iOur patient is a male infant who was born term and presented to the emergency room of our hospital when he was 9 weeks old. His complaints were recurrent fever since discharge from our newborn special care unit at 1 week of birth. He also had developed recurrent vomiting that was usually postprandial with associated significant weight loss. Birth history revealed that he was macrosomic with a birthweight of 4.5 kg. He was born via caesarian section due to cephalopelvic disproportion. Other important perinatal histories were that of meconium-stained liquor, perinatal asphyxia , and neonatal seizures on 1st day of life. During the initial newborn admission at about 4th day of life, mother noted that the patient had extensive purplish, nontender, firm subcutaneous nodules of uncertain diagnosis. The nodules were localized to the buccal fat region where they were first noted and then later spread to involve the neck, arms, forearms, thighs, and calves Figures , and 3. Subcutaneous fat necrosis of the newborn (SFN) is a rare form of panniculitis, an inflammation of the subcutaneous and adipose tissue . It typiAnother speculation for hypercalcemia is as observed in other conditions that have granulomatous lesions such as sarcoidosis ; the preSusceptible children who may have to undergo body cooling for the management of perinatal asphyxia could also develop subcutaneous necrosis of fat and adipose tissue . Our patThe typical skin lesions appear as erythematous to purplish, firm subcutaneous nodules  and are Among symptoms our patient presented with was persistent fever. Shumer et al.  noted feWe could also attribute the fever to the possible urinary tract infection as comorbidity which patient had. It may also be part of the symptom complex of SFN. Occurrence of hypercalciuria secondary to hypercalcemia especially in patients diagnosed with rheumatic disease and having a renal comorbidity may lead to sterile leukocyturia . This coThe presence of hypercalcemia may also be asymptomatic. Shumer et al.  also notApart from clinical diagnosis, SFN can be further confirmed by histopathology following a tissue biopsy, which is an invasive procedure. When the carefully collected biopsy is subjected to histopathology, the following are diagnostic: radially arranged clefts of crystalline triglyceride within fat cells, granulomatous cellular infiltrate composed of lymphocytes which confirms fat necrosis, and presence of histiocytes, multinucleated giant cells, and fibroblasts . Our patSFN usually runs a self-limiting course . When ouThe possible differential diagnoses to be considered alongside SFN include skin infections in the new born such as bacterial cellulitis, erysipelas, CMV infection, sclerema neonatorum, steroid-induced fat necrosis, deep infantile hemangioma, dermohypodermitis, neurofibromatosis, lipogranulomatosis (Farber disease), sarcomas including pediatric rhabdomyosarcoma, and other panniculitides , 21. TheAs part of management of these babies who have SFN with raised calcium levels, it is recommended that at least biweekly follow-up visits are done for up to a period of 6 months , 22. ThiSubcutaneous fat necrosis is a rare finding in our environment and can present with complications such as hypercalcemia. Presenting this case highlights the need for a high index of suspicion for the medical personnel, to aid early diagnosis and appropriate intervention. If correct diagnosis is made and child is properly followed up, the possible complications arising from hypercalcemia can be prevented or properly managed. Follow-up following resolution of skin lesions is also emphasized. This will help in reducing morbidity or mortality from SFN in the newborn.


A 51-year-old previously healthy male underwent a routine medical examination. Computed tomography and ultrasonography showed an oval-shaped mass that was about 50 × 40 mm in size in the left iliac fossa. Prior to surgery, the lesion was suspected to be a teratoma with core calcification or stromal tumor derived from the rectosigmoid colon. During the procedure, a yellow-white, egg-shaped mass was discovered that was completely free from the pelvic cavity in front of the rectum. The giant, peritoneal loose body was taken out through the enlarged port site. Histological examination showed that the mass consisted of well-circumscribed, unencapsulated, paucicellular tissue, with an obviously hyalinized fibrosclerotic center. A giant peritoneal body is extremely rare. We report such a case and review previously published literature. Peritoneal loose bodies are rare. They are usually found at laparotomy or autopsy by accident. In most cases, these bodies are derived from appendix epiploica. The most common size of loose bodies is about from 5 to 20 mm in diameter. Occasionally, they grow to larger than 50 mm by absorbing protein from peritoneal serum ,2. We reA previously healthy 51-year-old man underwent a routine medical examination. An incidental pelvic solid mass was detected on ultrasonography Figure . The ovaBased on the present imaging findings, the preoperative diagnosis of teratoma with core calcification or stromal tumor derived from the rectosigmoid colon was suspected. Accordingly, diagnostic laparoscopic surgery was performed. A yellow-white, egg-shaped body that was completely free from the pelvic cavity was found in front of the rectum Figure A. FurtheOn gross pathologic examination, the peritoneal loose body measured 50 × 40 × 40 mm. It was yellow-white, oval in shape, and it had a bony-hard, smooth surface. The cross section displayed a thread-like appearance. There were two calcified cores filled with yellow cheese-like material, and the interval distance between the two cores was about 5 mm  appendix epiploica, (2) omentum , (3) aut appendixThe differential diagnosis associated with peritoneal loose body include the following: (1) benign disease: leiomyoma, rhabdomyomas, teratoma, and fibroma; (2) malignant disease: colorectal cancer, ovarian cancer, and metastases; (3) calculous disease: urinary stones, gallstones, and appendix stones; (4) tubercular granuloma; and (5) others: calcification of lymph nodes, lymphoma, and foreign bodies. CT and MRI can be performed to distinguish peritoneal loose bodies from other lesions. For example, leiomyoma and some tumors enhance after injection of a contrast agent, while the appearance of peritoneal loose bodies remains unchanged.Treatment is surgical removal because it is not easy to establish definite diagnosis preoperatively via physical examination and imaging technologies. Laparoscopic exploration is recommended ,13-17. LUntil now, there have been no reports about the leading cause of death or recurrence in patients with peritoneal loose body. No harm has been shown to patients who receive active treatment.Peritoneal loose bodies are generally found incidentally. Clinically, if CT or other imaging shows an oval-shaped mass with or without calcifications in the central region, peritoneal loose body should be considered. Surgical removal is recommended for the patient with acute retention of urine or intestinal obstruction. Additionally, laparoscopy may be the best choice when the preoperative diagnosis is not clear and the lesion does not cause any clinical symptoms.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The authors present a rare case of osteoma cutis miliaris and briefly update the current knowledge about its clinic, pathogenesis, and therapeutic options. Miliary osteoma cutis is a rare entity, with just over 50 cases described in the literature . It is cClinically, it presents as papules and normochromic hardened nodules similar to milia, which particularly affect the face, with a preference for women . It may The authors report a rare case of miliary osteoma cutis and briefly comment on the current knowledge about its clinical presentation, pathogenesis, and therapy.A 62-year-old female patient complained about long standing lesions on the face. She denied any symptoms but wanted an aesthetic improvement of the lesions. She had a previous history of acne in adolescence without specific treatment.The examination showed several normochromic papules, hardened on palpation, grouped in the malar regions .The very hard consistency of lesions caught our attention and this is why an ultrasound of the face was requested, which revealed small punctate echogenic spots with posterior acoustic shadowing, distributed diffusely through the dermis of the cheek, which could correspond to calcium deposits.Laboratory tests revealed no changes in calcium metabolism or renal function.Histopathological examination showed a fragment of mature bone in the dermis, which became detached during the histologic processing, also containing bone marrow Figures  and 3. TAs the patient refused to be submitted any surgical procedure, we chose to use retinoid acid cream at 0.1%. After 6 months of treatment, we did not notice any improvement in the number or appearance of the lesions. However, the patient was satisfied and believed that the lesions were less evident, and therefore did not want to undergo any complementary therapy.Cutaneous ossification stages are rare and are divided into primary and secondary. Rare genetic syndromes, often progressive and severe, are among the primary causes, each with phenotypic characteristics of their own, namely, progressive fibrodysplasia ossificans, progressive osseous heteroplasia, Albright's hereditary osteodystrophy, and plaque osteoma cutis –3.Secondary or metaplastic causes constitute 80% of cases and appear as a consequence of previous lesions. Like cutaneous calcification, they may occur in collagen diseases, including dermatomyositis and scleroderma in panniculitis and in neoplasia, such as basal cell carcinoma and pilomatrichoma –3.The classification of miliary osteoma cutis is controversial. Despite being traditionally described as a primary ossification form, it is associated with previous history of acne in 50% of cases , which wMiliary osteoma cutis was first described in 1864 by Virchow [Hopkins, in 1928, was the first to suggest the role of acne in the development of multiple miliary osteoma cutis . In situ hybridization techniques have already shown that dermal fibroblasts have the ability to differentiate into osteoblasts and even to produce collagen type 1 and osteonectin [Its pathogenesis is not entirely known. There are some hypotheses regarding the origin of the cell forming osteoma. The most accepted theory is fibroblast metaplasia , 2. In sAnother hypothesis is that embryonic mesenchymal cells, erroneously migrated to the dermis, might differentiate into the osteogenic lineage . Gene muThere are several forms of treatment reported in the literature , 5, 6, i


Hepatobiliary cystadenocarcinoma is a rare epithelial malignant neoplasm of the liver or extrahepatic bile ducts. Early diagnosis of hepatobiliary cystadenocarcinoma is difficult because of its asymptomatic features and rarity. Moreover, the molecular pathogenesis of hepatobiliary cystadenocarcinoma remains unclear. Herein, we described a case of hepatobiliary cystadenocarcinoma in female with chronic hepatitis B and repeated hepatolithiasis.A 65-year-old woman with medical history of latent hepatitis B virus infection, repeated choledocholisthiasis, and cholecystitis was admitted due to a heterogeneous cystic mass (5.6 cm × 4 cm) shown on abdominal ultrasonography during regular physical checkup. The patient complained about irregular bowel movements with intermittent diarrhea for two months before presentation. Computed tomography (CT) disclosed a multiloculated cystic lesion in the left hepatic lobe with the presence of intraductal stones and dilatation of intrahepatic ducts. Histological results obtained from left lobectomy specimens showed hepatobiliary cystadenocarcinoma without accompanied mesenchymal stroma.Notably, hepatobiliary cystadenocarcinoma without mesenchymal stroma seldom occurs in women and is usually associated with poor prognosis. We present the rare findings in this patient and suggest that chronic inflammatory insults in the intrahepatic bile ducts might shed light on the cystadenocarcinogenesis. Hepatobiliary cystadenocarcinoma is a rare cystic neoplasm that predominantly occurs in the middle-aged women. Its early detection is challenging because of varied clinical features . The tumA 65-year-old woman was admitted to our hospital for surgical intervention due to one liver tumor identified on the abdominal ultrasonography during regular follow-up for chronic hepatitis B. Before presentation, the patient reported to have irregular bowel movement with loose stool passage and weight gain for about two months. She experienced no abdominal pain, fullness or vomiting. When tracing her past history, the patient had undergone cholecystectomy for cholecystitis and received two times of laparotomy in regard to repeated choledocholisthiasis caused by hepatolithiasis about 30 and 20 years ago, respectively. Besides, four of the patient’s seven siblings were diagnosed with hepatitis B-related hepatocellular carcinoma, which had contributed to their death and severe morbidity.The physical examination was unremarkable. There was no abdominal tenderness, rebounding tenderness and abnormal bowel sounds. Murphy’s sign was also negative. Laboratory studies on admission yield normal blood biochemistry and mild thrombocytopenia (127000 per cubic millimeter). The liver function test showed normal results . No prolonged prothrombin times or partial-thromboplastin times was found. Direct and total bilirubin were not elevated. Enzymes such as gamma-glutamyl transferase or alkaline phosphatase were within normal ranges. The urinalysis was normal, without dark or tea-colored urine. The levels of tumor markers such as AFP, CEA, CA125 and CA19-9 were all within normal limits.Ultrasonography Figure . All secAfter total resection of the tumor, the patient was hospitalized for several days. She had no fever, no distention and other morbidities before discharge. Her incision had healed well 3 weeks after discharge. She showed no adverse event related to hepatobiliary disease or the surgery. The follow up MRI three years after operation confirmed no recurrence of malignancy.Hepatobiliary cystadenocarcinoma is a rare cystic neoplasm that usually presents as a multilocular cystic mass with internal septations, nodules, and irregular, thickened cyst wall ,7,8. It There is currently little evidence describing the genesis of cystadenocarcinoma without OS. More clinical and pathological studies are required to determine whether it is generated directly from biliary epithelium or from other pre-malignant structures. One possible mechanism of the initial cystic formation comes from the hyperplasia of peribiliary glands which are located along the walls of extrahepatic ducts, the neck of the gallbladder and the larger intrahepatic bile ducts ,14. ThesIn addition to the presence of calculi in hepatolithiasis, cholestasis is the common phenomenon of cirrhosis, cholangitis and bile duct cysts. To prove the effect of cholestasis on the biliary epithelium, Yang and colleagues  induced To avoid development of biliary tract malignancies due to surgical intervention, anatomic hepatectomy  and lapaIn conclusion, we strongly suspect that hepatolithiasis and other inflammatory changes contribute to the formation of the patient’s hepatobiliary cystadenocarcinoma, probably through hyperplasia and neoplasia of peribiliary glands and biliary epithelia. It is a possible etiology other than benign cystic lesion or ectopic ovarian tissue during embryonic development, and the absence of ovarian stroma also lowers the possibility of tumorigenesis from pre-existing cystadenoma. Furthermore, since the absence of OS has poorer clinical outcomes, early detection and complete resection of this tumor are the key factors for achieving cure. Regular ultrasonography checkup is warranted for patient with evidence of hepatholithiasis and associated dilatation of intrahepatic ducts for early diagnosis of this malignant disease.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.The authors declared that they have no competing interests.YHD and YHY co-wrote the paper; CBH performed surgical operation; YFL performed pathological analysis; all authors cared for the patient and provided advice on the clinical aspects of the case report. All authors read and approved the manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/14/109/prepub


Gastrinoma is most commonly located in the gastrinoma triangle  or in the adjacent lymph nodes. Due to the low mortality rate, it is often misdiagnosed as other diseases with similar clinical characteristics, such as a solid pseudopapillary tumor of the pancreas (SPTP). Therefore, the current study reports a rare case of gastrinoma located in the tail of the pancreas of a female patient under medical examination, who exhibited no clinical symptoms. The tumor, which was located in the body and tail of the pancreas, was successfully resected and the spleen was preserved. The outcome of surgery combined with the postoperative pathological examination resulted in the patient being misdiagnosed with a SPTP. During the consequent six-year follow-up period, low-density liver lesions and an intractable peptic ulcer gradually appeared. Finally, the patient diagnosis was confirmed as a malignant pancreatic neuroendocrine carcinoma with liver metastases. On June 1, 2011, a liver transplant was successfully performed and the patient has maintained a good overall condition. The underlying clinical and pathological factors that may have resulted in misdiagnosis are investigated in the present study. Through providing our preliminary clinical experiences and lessons, the aim of the present study was to focus the attention of clinicians on this type of cancer in order to improve its diagnosis and treatment. Gastrinoma is a type of neuroendocrine tumor with an incidence of approximately one to three cases per year for every 1,000,000 individuals  and a fiOn December 15, 2006, a 39-year-old female was admitted to the Anhui Provincial Hospital  following the observation of a pancreatic body and a tail lesion in an annual periodic health examination. The patient had not experienced any discomfort and was hemodynamically stable. A benign abdominal examination was conducted and all of the blood and biochemical parameters were within their respective reference ranges. The family and personal medical histories were unremarkable. Written informed consent was obtained from the patient for publication of the present study and any accompanying images. Enhanced computed tomography (CT) revealed a cystic mass in the pancreatic body and tail . ConsideThe current study presents a rare case of a female patient with malignant pancreatic neuroendocrine carcinoma with liver metastases, who was initially misdiagnosed with SPTP. The potential underlying clinical and pathological factors that may have led to misdiagnosis are analyzed and listed in the present study.First, the majority of gastrinomas are located in the gastrinoma triangle (comprising of the duodenum and the pancreas) or in the adjacent lymph nodes , whereasSecondly, it is known that gastrohelcosis and diarrhea are common first signs of a gastrinoma, particularly in non-functioning cases, while SPTP are generally characterized in young females with no clinical symptoms prior to the onset of the illness, negative tumor markers and without a history of peptic ulcers . CombineThirdly, pathological assessment is the most reliable diagnostic basis and considered to be the gold standard. However, it is worth noting that the characteristics of hematoxylin and eosin (H&E) staining with gastrinoma and solid pseudopapillary tumors are similar and may be confused, even when specific typical immunohistochemical indicators, such as Syn or CgA , are obsFinally and most significantly, a monism, rather than pluralism, approach is required to diagnose the disease. The patient in the present case experienced intractable recurrent peptic ulcers and multiple low-density liver lesions developed continuously during the postoperative follow-up period. If clinicians are able to connect these novel conditions with previous surgical history, it may allow a correct revised diagnosis to be obtained promptly following surgery.Therefore, a serious misdiagnosis emphasizes the requirement for a more accurate differential diagnosis of such a rare case of a disease without a typical clinical manifestation. The aim of the present study was to focus the attention of clinicians on this disease in order to improve its diagnosis and treatment to a certain extent.In conclusion, pancreatic gastrinoma should be carefully differentiated from SPTP during diagnosis. Immunostaining of E-cadherin and β-catenin may be necessary to aid with the differential diagnosis.


Torsion of a gravid uterus is a rare obstetric emergency potentially lethal for the fetus and the mother. Some of the cases described in literature are associated with preexisting gynecologic conditions related to pelvic and uterine anatomy, even if most of cases remain unexplained. We report a case of acute 180-degree torsion of uterus at 33 weeks of gestation associated with abruptio placentae in a young Asian woman without apparent risk factors. Torsion of gravid uterus is a rare, potentially serious, unexpected, obstetric emergency and it is almost always diagnosed during caesarean section . UterineWhen the uterus rotates on its longitudinal axis, the blood supply is dramatically altered due to a vascular obstruction involving at first the veins and after the arteries. This sequence is the cause of acute or subacute abdominal pain, fetal distress, and abruptio placentae in the most severe cases.There are no reported cohorts of uterine torsions, but only the description of single cases –9. The mWe report a case of acute uterine torsion at 33 weeks of gestation in a singleton pregnancy without uterine myomas.A 25-year-old Asian woman, gravida 2, para 1, reported to our hospital at 33 weeks of gestation with severe abdominal pain. She had a BMI of 24 and a negative obstetric history in the previous pregnancy. The abdominal pain had started three hours prior to admission, like a vague posterior abdominal colicky pain. The patient and her husband did not relate physical efforts prior the onset of the pain. She did not have abdominal surgery in the past. The course of pregnancy was regular including fetal growth.At the admission in our hospital the patient appeared pale, exhausted, unable to walk, and maintaining an upright position. She had nausea and episodes of vomiting. At triage blood pressure was 90–60 mmHg, heart rate was 120 beats per minute, and temperature was 37.1°C. Abdominal pain was deep, diffuse, with no signs of peritonitis or lateral renal colic. Uterine growth was appropriate for gestational age and the organ was tender on palpation. On vaginal examination cervix was closed and there was no vaginal bleeding or abnormal vaginal discharges.Ultrasonography showed a single fetus in transverse lie, with a normal amniotic fluid. Placental location was clearly “anterior” with vast subchorionic hypoechogenic area interpreted as a sign of abruptio placentae. Placental tissue was hyperechogenic and thicker than normal. Fetal heart rate at ultrasound examination was 90 beats per minute. It was not possible to visualize and interrogate uterine arteries in their typical anatomical sites by Color Doppler and Pulsed Wave Doppler.An emergency caesarean section under general anesthesia was performed. Abdomen was opened by standard Stark incision. Peritoneal cavity contained approximately 100 milliliters of coagulated blood. Hysterotomy was done on “the lower uterine segment.” At amniorrhexis the amniotic fluid was clear. A male fetus in transverse lie was delivered by breech extraction. The fetus was alive but pale and hypotonic. A large quantity of fresh clots leaked from the uterus during the afterbirth. The placenta showed a large subchorionic haematoma. The uterus was ischemic and extremely floppy. At exteriorization of the uterus we observed the anterior crossing over of the proper ovarian ligaments. At that time we realized that the uterus was rotated by 180 degrees. We performed the counterclockwise detorsion of the uterus and confirmed a low transverse incision of the posterior wall of the organ. In a matter of seconds after detorsion the myometrium reverted to a normal colour and consistency. We also observed a superficial bleeding laceration of the left part of the posterior wall that probably was the cause of the hemoperitoneum. There were no myomas or uterine malformations. The posterior uterine incision was closed by a standard two-layer suture. The uterus, after the administration of uterotonic, was properly contracted and it was then replaced into the abdomen. The estimated loss of blood was 1100 milliliters. The placenta was sent for pathologic examination that confirmed the clinical diagnosis of abruptio.Maternal conditions were stable after two hours. Haemoglobin concentration decreased from 10.8 grams/liter at admission to 8.5 grams/liter. White blood cell count was 19.000/milliliter, almost halved from admission values (31.000/milliliter). C-reactive protein had always been negative. Analysing the previous reports and ultrasound images in the patient's pregnancy record, we confirmed that a posterior placental position had been observed at 22 weeks' scan, exactly the opposite of the anterior position we have seen by ultrasound at the time of hospital admission.The newborn at birth weighted 2200 grams, Apgar score was 1, 3, and 5 at 1, 5, and 10 minutes, cord pH was 6.61, and base deficit was 28 millimol/liter.A severe acute cerebral haemorrhage was diagnosed. The newborn was admitted at Neonatal Intensive Care Unit (NICU), never recovered his vital functions, and died sixty days after birth.The woman was discharged three days after caesarian section.Although the torsion of gravid uterus is a real emergency in obstetrics, it is not easy to study the real epidemiology of this disease due to its rarity. Even if it is not possible to find a clear etiology, the most common risk factors are myomas, uterine malformations, pelvic adhesions, ovarian cysts, abnormal fetal presentation, or anomalies and maternal abnormalities of the spine and the pelvis. Other causes like external cephalic version and maternal trauma have been reported , 4. HoweIn our case the only risk factor was the transverse lie of the fetus.Because of its rarity and the emergency of the situation, it is very difficult to diagnose this condition antenatally. The symptoms reported in literature are persistent abdominal pain associated with a tender uterus on palpation, vaginal bleeding in case placental abruptio is also present, shock, and urinary or intestinal symptoms. Sometimes this condition could be even asymptomatic .The modification of placental site compared to the previous scan on ultrasound and the abnormal position of ovarian and uterine vessels across the uterus on Doppler evaluation can help to suspect the torsion.The magnetic resonance imaging could be helpful but it is not feasible in emergency situation when fetal distress is suspected , 4.The management of torsion of gravid uterus consists in emergency laparotomy. As described above, it is frequent to diagnose the condition only after the extraction of the fetus. In unexpected cases posterior low transverse incision is usually performed. In such patient with a posterior Hysterotomy an elective caesarean section is advisable for future pregnancy, because the risk of uterine rupture during labour through the posterior scar is not known.Proposed methods to prevent a recurrence of uterine torsion in future pregnancies, as plication of round ligament or uterosacrals, are not validated .As regards neonatal and maternal complications, Jensen has reported 13% of perinatal mortality .Even if rare, the severity of this pathology requires knowledge of its existence and awareness of how to handle this emergency. This is the only manner to preserve maternal and newborn's health.


To investigate the clinical and pathological features of metanephric adenoma (MA) and the clinical outcome after retroperitoneal laparoscopic nephron-sparing surgery.Six out of 183 partial nephrectomies performed during January 2009 to August 2014 were confirmed to be MA confirmed by postoperative pathological study. Perioperative parameters of the six patients were then retrospectively collected, analyzed and compared with current literature, including warm ischemia time (WIT), total operation time, estimated blood loss (EBL), positive surgical margin (PSM), and complications. Surgical and oncological outcome of all six patients were evaluated based on a mean follow up of 17 months (5–48 months).Tumors in all six cases were all successfully removed by partial nephrectomy. Mean WIT was 24.7 min (19–35 min). Mean operation time was 103.6 min (82–147 min). Mean EBL was 53.5 ml (20–85 ml). No conversion, transfusion or other major complication were observed in all six cases. Postoperative pathology confirmed negative surgical margin in all six cases. During a mean of 17 month follow up (5–48 months), no local recurrence or metastasis were found in all six cases.MA is a rare benign primary kidney epithelial cancer, which could hardly be differentiated from renal malignancies based on preoperative imaging. Our data suggested that retroperitoneal laparoscopic partial nephrectomy can be used for surgical treatment of MA, in terms of tumor control and preservation of renal function. Metanephric adenoma (MA) is a very rare benign tumor derived from kidney epithelial tissue, with less than 100 cases reported in current literature  performed during January 2009 to August 2014 were confirmed to be MA confirmed by postoperative pathological study, including 4 females and 2 males  via an retroperitoneal approach. Surgical procedures:After general anesthesia and routine intubation, patient was placed in 90 degree lateral position with the affected side facing up. Trocar configuration was shown as Fig. All tumors were successfully removed by RLPN without conversion or transfusion. Mean warm ischemia time (WIT) was 24.7 min (19–35 min). Mean operation time (OT) was 103.6 min (82–147 min). Mean estimated blood loss (EBL) was 53.5 ml (20–85 ml). Patients were discharged on day 7 (5–10 days) postoperatively.Mean diameter of tumors matched with preoperative CT scan, with or without capsule. The cross-section of the tumor showed a light yellow or gray soft and homogeneous characteristic Fig. a. Only 1During a mean of 17 month follow up 5–48 months), no local recurrence or metastasis were found in all six cases confirmed by CT (Fig. 8 months,MA is a rare tumor, accounting for the 0.2 % of adult renal epithelial tumors (Kuroda et al. The diagnosis of MA based on history, physical examination and radiological studies remains a difficult task because of the similarity in symptoms and radiological appearance between MA and renal malignancies. Ultrasound is usually able to detect a single and remarkable mass emerging from the kidney surface or inside the renal parenchyma, displaying a hypoechoic, hyperechoic, and uniform echo (Zambrano et al. MA usually appears as a unilateral single mass, and may resides in any part of the kidney, although more prominent in the renal cortex of the kidney surface, with no clear invasion, with or without a capsule. Some studies reported tumor diameter could range from 0.3 to 15.0 cm (Davis et al. Since MA is not characterized by specific and typical clinical symptoms and signs, imaging studies are facing a difficult interpretation. In addition, since MA is a rare tumor, it is easily misdiagnosed as kidney cancer. In the absence of clear pathological findings, clinical treatment and surgical methods are basically equivalent to the treatments for kidney malignancies, such as nephron-sparing surgery or radical nephrectomy (Ebine et al. The lack of sufficient MA clinical data prevents the improvement of its treatment with little literature support. Several studies reported the use of laparoscopic nephrectomy (Ebine et al. In conclusion, MA is a rare primary renal tumor, with no distinct clinical symptoms and radiological characteristics. It can be easily confused with malignancies of the kidney. RLPN is shown to be a safe and feasible approach for treatment of MA both oncologically and functionally.


Introduction. Osteoma is a benign, slowly growing, asymptomatic, osteogenic neoplasm. Osteoma of a bone other than the skull and facial bones is extremely rare. An extremely rare case of parosteal osteoma is reported. Case Presentation. A 51-year-old woman presented with a large mass in the left supraclavicular fossa. Radiographs and computed tomography revealed a well-defined, 9 × 6 cm, lobed mass in the midportion of the clavicle. Magnetic resonance imaging revealed that it had the same density as cortical bone. An open biopsy was performed to rule out malignant bone tumours, and parosteal osteoma was diagnosed. Four years after the biopsy, the patient was asymptomatic. Conclusion. A rare case of parosteal osteoma of the clavicle was described. Open biopsy is required to rule out a malignant bone tumour, even if parosteal osteoma is suspected based on the clinical course and imaging findings. Osteomas arise from cancellous or compact bone and are often referred to as benign osteogenic lesions. Clinically, they appear circumscribed, usually rounded and protuberant, and are characterized by very slow, continuous growth . OsteomaA 51-year-old woman presented with a large mass in the left supraclavicular fossa. The mass was incidentally found on a chest radiograph approximately 30 years earlier. The patient had been asymptomatic but reported that the mass had gradually increased in size.Physical examination revealed an ill-defined, 9 × 6 cm, rough-surfaced, bony-hard mass in the anterosuperior aspect of the left clavicle. Shoulder movement was not limited, and no stigmata of Gardner's syndrome were noted.Radiographs revealed a well-defined, 9 × 6 cm, lobed mass in the midportion of the clavicle bone . A compuAn open biopsy was performed to rule out parosteal osteosarcoma. Histologically, the resected tissue was composed of dense sclerotic lamellar bone, and no evidence of malignancy was found. Thus, a diagnosis of parosteal osteoma was made .Four years after the biopsy, the patient remained asymptomatic, even though the tumour appeared to be growing slightly.Osteoma is a benign osteogenic lesion with very slow growth that is classified into the following three types based on location or clinicopathologic features: (1) skull and facial bones; 2) exostoses or bone islands; and (3) long bone superficial osteomas   exostose.A parosteal osteoma of the long bone may be an asymptomatic incidental finding, or the patient may have noted a mass lesion that enlarged gradually. It is thought that it originates as ossification in intramembranous bone and can be seen at almost any age, with a focus on the 4th and 5th decades.Radiographs reveal homogeneous, well-defined, smooth round, or lobulated margins. Typically, a CT scan shows that the heavily ossified mass is attached to the cortex, with no areas of lucency.Primary extracranial osteomas are extremely rare, representing only 0.03% of biopsied primary bone tumours . To the The differential diagnosis of parosteal osteoma should include tumoural calcinosis, osteochondroma, myositis ossificans, parosteal osteosarcoma and parosteal chondrosarcoma. It is especially important to rule out a malignant bone tumour. Unlike conventional osteosarcoma, this type of tumour most commonly occurs in patients in the 3–5th decades of life. Although low-grade, the lesion can infiltrate the underlying cortex and extend into the medullary cavity; it also has a propensity to recur . The appThe diagnosis may be difficult and risky with a small biopsy sample; however, in conventional osteoma, surgical intervention is usually only warranted in symptomatic patients. On the other hand, it is not unusual for parosteal osteosarcoma to be treated with definitive operative resection without a previous biopsy .Osteoma is a benign disease. If areas of lucency are not seen on radiography, and histological examination does not reveal spindle cell proliferation, a diagnosis of osteoma can be considered. Once the diagnosis of parosteal osteoma has been made, wide resection is no longer necessary because recurrence is rare  and maliA rare case of parosteal osteoma of the clavicle was described. Open biopsy is required to rule out a malignant bone tumour, even if parosteal osteoma is suspected based on the clinical course and imaging findings. The patient was followed up for four years and remained asymptomatic throughout. The diameter of the tumour increased slightly over the 4-year followup. Since the growth potential of osteoma remains unclear, the patient requires close clinical and radiographic followup.


We present a 2-day-old female neonate with cleft of the upper sternum, patent ductus arteriosus (PDA), atrial septal defect (ASD), and subglottic hemangioma. Dimensional and Doppler echocardiography, abdominal ultrasonography, and imaging were performed. She underwent a surgical repair of sternal cleft in neonatal life. After 8 months, she developed respiratory distress, apnea due to subglottic hemangioma. She underwent urgent tracheostomy. Subglottic hemangioma was treated with the KTP532 laser.  Sternal cleft is a rare congenital anomaly which results from failure of fusion of the 2 lateral mesodermal sternal bars during 8th week of gestation.[1] It is often associated with aplasia cutis congenita (ACC) at sternum. Subglottic hemangioma is also a rare entity.[2] Numerous treatment modalities have been advocated like systemic corticosteroids, tracheostomy, carbon dioxide laser, or interferon alfa-2a. The coexistence of subglottic hemangioma and sternal cleft in presence of cardiac anomalies may pose diagnostic and treatment challenges especially when subglottic hemangioma is revealed on subsequent presentation. We herein report a rare association of sternal cleft and ACC with subglottic hemangioma, PDA, and ASD.A 2-day-old, term female neonate, presented with a U-shaped cleft of the upper half of the sternum, with central area of ulcerated area of overlying skin through which pulsating heart was visible . There was no history of maternal infection or medication during early pregnancy. Dimensional and Doppler echocardiography showed a sternal cleft, PDA, ASD, and a normal aortic arch. Abdominal ultrasonography was normal. The patient underwent surgery at 3rd day of life. The necrotic fibrous tissue (ACC) on the sternal cleft area was completely resected without opening the pericardium. Fasciocutaneous flaps were raised on either sides and both sternal bars were approximated with 2/0 PDS and covered by fasciocutaneous flaps.After 8 months, she was readmitted because of the severe respiratory distress and stridor. On examination, there was a hemangioma involving the lower lip, oral mucosa, and right parotid region. Urgent spiral CT-scan of head and neck revealed subglottic hemangioma. A tracheostomy was performed to relieve the respiratory distress. She underwent laryngoscopy and bronchoscopy a week later and subglottic hemangioma was treated by KTP532 laser. Tracheostomy tube was removed subsequently. She had no respiratory distress or subglottic hemangioma at four year follow-up .A cleft sternum  is a rare congenital anomaly with unknown etiology.[1] Majority of the cases of sternal clefts are reported in female patients. Jose et al have reported 8 cases of congenital sternal clefts including six female and two male patients.[2] Sternal clefts can be superior, inferior, and complete. The superior cleft, as seen in the index case, is the frequent one. Superior defects are usually U-shaped as compared to inferior ones which are V-shaped. Sternal cleft is also a part of PHACES  syndrome. Mazzie et al reported a female neonate with upper sternal cleft and ACC, associated with cardiovascular anomalies, and hemangioma involving face, mouth, and upper trachea causing stenosis of glottis.[3] They considered their patient a forme fruste of PHACES syndrome. Our patient also had upper sternal cleft, with cardiac anomalies, and hemangioma involving the same pattern as the case of Mazzie et al. Thus our case can also be considered as forme fruste of PHACES syndrome.Treatment of sternal cleft is surgical apposition of sternal bars. The surgery is usually performed in neonatal period when sternal bars and chest wall are most compliant. Treatment is neonatal life is associated with good functional and cosmetic outcome as found in the index case. To conclude, any patient with sternal cleft anomaly should carefully be evaluated for cardiac anomalies, and airway hemangioma. PHACES syndrome should be considered in any infant of sternal cleft presenting with a large and segmental facial hemangioma.Source of Support: NilConflict of Interest: None declared


Primary extra-gastrointestinal stromal tumor (EGISTs) arising in the pancreas is extremely rare: only 20 cases have previously been reported in the English literature from 2000 to 2013. We reported a case of EGIST of the pancreas in a 69-year-old woman who presented with abdominal pain and with a solid, heterogeneously enhancing neoplasm in the uncinate process of the pancreas, revealed preoperatively by an abdominal computed tomography scan. A diagnosis of neuroendocrine tumor was suggested. Positron emission tomography with 68Ga-DOTATOC did not show pathological accumulation of the tracer in the pancreas. The patient underwent enucleation, under ultrasonic guidance, of the pancreatic tumor that emerged to the surface of the pancreas. Histopathology and immunohistochemical examination confirmed the final diagnosis of EGIST of the pancreas (CD117+), with one mitosis per 50 high-power fields. Although rarely, GIST can involve the pancreas as a primary site, and this tumor should be considered in the differential diagnosis of pancreatic neoplasms. Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract, with an annual incidence of 10 to 20 per million . ICCs foA 69-year-old woman presented in March 2013 with abdominal pain localized in the right hyphocondrium. There was no history of vomiting, gastrointestinal bleeding, jaundice, anorexia, or weight loss. Abdominal ultrasonography did not show pathologic features, but the pancreas was not clearly visualized. Contrast-enhanced computed tomography (CT) of the abdomen Figure  and smooWe reported a rare case of a primary pancreatic GIST: the small size, the well circumscribed margins of the lesion, and the contrast enhancement at CT examination suggested a diagnosis of neuroendocrine tumor of the pancreas. GISTs reported outside the gastrointestinal tract as apparent primary tumors are defined as ‘extra-gastrointestinal stromal tumors’ (EGISTs). The concept of GIST has recently been established, due to the progress in immunohistochemical analyses. It is presumed that these tumors originate from the interstitial cells of Cajal (ICCs), pacemaker cells, which are present throughout the wall of the gastrointestinal tract and which regulate the motility. ICCs share many characteristics with EGISTs, including expression of CD117 and CD34 . In factn = 1.5%) involved the entire pancreas [Pancreatic GISTs are extremely rare. We collected only 21 cases (including our patient) from the English literature: their clinicopathologic features and outcomes are summarized in Table pancreas . There ipancreas ,22 and iThe clinical presentation of EGISTs is variable, depending on the location and size of the tumors. The tumor may even be found incidentally. The most frequent clinical symptoms are: abdominal pain, ileus, bleeding, anemia, and weight loss. Our patient presented with a small, solid, hyperintense lesion resembling a neuroendocrine tumor. A similar finding of small, solid tumor in the uncinate process of the pancreas was reported by Yan et al.  in a casIn conclusion, we report a new case of EGIST arising from the pancreas, misinterpreted as neuroendocrine tumor. Despite its rarity, GIST should be kept in mind in the differential diagnosis of primary tumors of the pancreas, especially when a hypervascular lesion, solid or cystic, is lacking somatostatin receptors at PET examination. Radical resection of pancreatic GISTs is the treatment of choice, and repeat surgery for recurrence may offer a prolonged survival.Written informed consent was obtained from the patient for publication of this case report and any accompanying image. A copy of the written consent is available for review by the Editor-in-Chief of this Journal.The authors declare that they have no competing interests.VB and CS conceived the study, carried out the literature search, and drafted the manuscript; MG and DP helped in management of the patient; SP carried out the pathologic diagnosis and immunoassays; SM made critical revisions and supervision. All authors read and approved the final manuscript.


Pneumatosis intestinalis (PI) is a condition in which multiple gas-filled cysts are located in the bowel wall; it can represent a wide spectrum of diseases and a variety of underlying diagnoses. The present report describes the case of an 86-year-old man with symptomatic primary PI of small bowel treated with surgical approach after periodic episodes of cysts rupture and superimposed inflammation revealed on the basis of a clinical suspicion thanks to abdominal computed tomography. Moreover, after one year of followup, there has been no recurrence of digestive symptoms. Pneumatosis intestinalis (PI) is a finding characterized by the presence of gas within the bowel wall , 2. Two PI has been classified as primary (idiopathic) and secondary. Primary PI 15%) is generally nonsymptomatic and diagnosis is often occasional. This form is usually located in the colon. Males are more often affected and the age group most affected is between the fourth and sixth decades of life [% is geneSecondary PI (85%) has been associated with numerous coexisting disorders of the gastrointestinal tract or the respiratory system, such as chronic obstructive pulmonary disease, intestinal obstruction, ischemic bowel disease, necrotizing enterocolitis in premature infants, immunodeficiency such as AIDS, bacterial and/or viral infection, and drug therapy –13. MorpWe present a rare case of idiopathic bubble like (cystoides) PI of the small bowel.An 86-year-old man was admitted to the emergency department presenting with abdominal pain, subobstructive episode, and constipation alternately to diarrhea. His previous medical history revealed a cholecystectomy.Abdominal examination at admission revealed a diffuse distention and mild rebound tenderness. Laboratory studies revealed unremarkable serum blood count, electrolytes, and liver biochemistry.Abdominal computed tomography (CT) without intravenous contrast demonstrated some gas-filled cysts within the small bowel wall, not associated with free intraperitoneal air. Based on the CT finding of air within the bowel wall, the patient was diagnosed with small bowel PI. The patient was not treated and he was discharged at his own request.One month later, presenting with a new episode of generalized abdominal pain, the patient underwent abdominal ultrasound (US) and plain abdominal film. The latter showed a large amount of intramural gas within some small bowel loops ; US was The onset of subobstructive symptoms over the following first month led the patient to repeat abdominal CT examination with intravenous contrast. It demonstrated the presence of small bowel PI with intraperitoneal free air near the liver and in the mesenteric fat due to the rupture of intramural gaseous cysts into the peritoneal cavity . A laparThe most prominent affected segment of around 50 cm in length was resected  and lysiHistological study revealed cysts located within the subserosa, with giant cells around the cystic wall.After one year of followup, the patient was still asymptomatic.PI is radiologically characterized by cystic or linear collections of gas in the subserosa or submucosa of the gastrointestinal tract , 16. It The first description of pneumatosis cystoides intestinalis was made by Du Vernoin as a postmortem observation .In most cases, PI is an incidental finding, whereas in others PI is secondary to a wide variety of gastrointestinal and nongastrointestinal diseases , 19.Clinically, PI ranges from benign disease, which does not require treatment, to more severe conditions needing oxygen, intravenous hydration, and antibiotics, to a life-threatening entity requiring immediate surgery.In cases of PI due to benign causes, especially PI associated with pulmonary disease, the patients are usually asymptomatic and often only necessitate conservative therapy. Some patients may have mild abdominal discomfort, which is usually related to the underlying associated medical condition. Physical examination is rarely abnormal unless there are peritoneal signs from intestinal perforation in cases of PI due to life-threatening causes , 20, 21.In our case, the persistence of symptoms, due to inflammatory adhesions subsequent to episodes of asymptomatic rupture of the cysts, has made it necessary for lysis of adhesions. Since the bubbles were limited only to the small bowel, it was possible to perform a preventive partial jejunal and ileal resection in order to prevent further acute episodes.Endoscopy, barium enema, US, plain abdominal film, and abdominal CT can be supportive in diagnosing PI.In our case endoscopy was not performed due to the location of cysts into the small intestine, such as for barium enema. However, regarding the latter some authors have warned that PI could be confused with intestinal polyposis on barium enema, as they have very similar appearance , 22–26.US can also be used to detect PI –29. ThisPlain abdominal film can be useful in identification of PI but it is more recommended for the study of complications, such as the pneumoperitoneum that is detectable on erect chest radiography by the presence of subdiaphragmatic air or by the evidence of air pockets on supine plain abdominal film .CT has greater sensitivity in diagnosing PI than plain abdominal film or US since it can distinguish PI from intraluminal air or submucosal fat , 22–26.The ability to study the bowel wall in the coronal, sagittal, and axial planes may allow a more confident diagnosis of PI and portal venous gas. Because CT is more sensitive than plain abdominal film in detecting PI, it can be used to clarify ambiguous radiographic findings and also to search for potential causes. On both plain abdominal film and CT, PI usually appears as a low-density linear or bubbly pattern of gas in the bowel wall. It can be a combination of both linear and bubbly bowel-wall gas. There also may be circular collections of gas in the bowel wall. CT has also been shown to be more sensitive than plain abdominal film in detection of some complications like portal and portomesenteric gas or the presence of pneumoperitoneum detected even with 1-2 mL of free intraperitoneal air .In our case, CT allowed to identify complications consisting of intraperitoneal free air near the liver and in the mesenteric fat, without signs of portomesenteric air. On CT scans we recognized a typical primary cystoids pattern characterized from segmental distribution of the radiolucent clusters along the bowel wall and cysts with a variable diameter between 5 and 30 mm and margins clearly identifiable  but withCT is the most appropriate tool to localize the PI and to make differential diagnosis between primary PI and secondary PI basing on the shape of lesions.The secondary forms usually have a different feature than idiopathic, showing a linear radiolucency within the wall of the gastrointestinal tract, parallel to the intraluminal gas. Sometimes the aspect can be fine bubble or mist .Currently, there is no consensus on the appropriate management of PI. Since PI can represent a wide range of pathology, it is in itself not diagnostic of any certain condition. The variety of presentations of PI highlights the fact that clinicians should interpret radiographic findings in concert with the current clinical scenario in order to ensure a correct diagnosis and to guide one toward a suitable management. Around 50% of patients with PI can be successfully managed nonoperatively .The idiopathic PI does not require surgical intervention in itself since some lesions disappear spontaneously within months or years , unless Identification of cases in which laparotomy can be avoided, or deferred, is important in order to prevent unnecessary surgery with its associated morbidity and financial costs.In conclusion the authors emphasize the role of TC in the localization of lesions and identification of PI pattern; the meticulous integration of the appearance on abdominal CT scan, the laboratory data, and clinical presentations permit clinicians to suspect the onset of complications and to distinguish benign from life-threatening PI and to decide whether or not urgent surgical intervention is necessary.


Lumbar hernias are rare conditions and about 300 cases have been reported since the first description by Barbette in 1672. Therefore strangulation or incarceration are also exceptionally encountered. We present a 62 -year-old-man who had strangulated left lumbar hernia and consequent mechanical small-bowel obstruction, alongside with a non strangulated right lumbar hernia. Through a median laparotomy, an intestinal necrosis was found. A bowel resection with end to end anastomosis was performed and the lumbar hernias were repaired on both sides. The recovery was uneventfull. To the best of our knowlwdge thanks to the litterature review presented here, this is the 19th case of incarcerated or strangulated spontaneous lumbar hernia described in the surgical litterature since 1889. Lumbar hernia though well described, is a rare condition with approximately 300 cases reported in the literature since it was first described by Barbette in 1672. Twenty percent of lumbar hernias are congenital and the other 80% are acquired; the acquired lumbar hernias can be further classified into either primary (spontaneous) or secondary (either iatrogenic or traumatic)[A 62-year-old man presented to our emergency department with nausea, vomiting and abdominal pain together with swelling and pain of the left lumbar region for 4 days. His medical history was not consistent he was a farmer. On physical examination, the abdomen was distended and tympanic. There was tenderness, especially in the left lumbar regiont. A small painfull irreductible mass (about 6-cm in diameter) was palpated above the left iliac crest. Another mass, instead reductible was found on the right lumbar region above the iliac crest Figure . AbdominA preoperative work-up was normal except the ESR CRP and leukocyte count that were increased. Electrolyte and other biochemical studies were within normal limits.The patient was taken to the operating room for urgent surgery with the diagnosis of intestinal obstruction due to incarcerated lumbar hernia. An abdominal exploration was performed through a midline incision. During the exploration, at approximately 200 cm from the Treitz ligament, a loop of small bowel was found incarcerated within the left lumbar triangle of Petit. A 40-cm necrotic small-intestinal loop was resected and continuity was re-established. During evaluation of the hernial areas, there was no other herniation except the right lumbar hernia already mentioned. The lumbar hernias were repaired with a 2(USP) resorbable suture.The post-operative period was uneventfull. The patient was discharged without any complication on the thirteen postoperative day. As of date more than 2 years after the operation, the patient is doing well. No recurrence has been observed.Lumbar hernia is a well documented but extremely rare condition. Men in their sixth decades and above are more proned than women. Complications such as strangulation is rarely encountered and since 1889 with the excellent description of a patient having a strangulation by Hume; surgeon at the Royal Infirmary in Newcastel on Tyne, about 1Lumbar hernia is seen mostly in association with other abdominal wall hernias in elderly patients . They can also be bilateral as seen in this case. It was reported that coexistence of lumbar hernia and other abdominaal wall Hernia is observed in 13% of patients. These reports suggest that a patient presenting with a lumbar hernia should be explored for the presence of a coexisting hernia, such as inguinal, femoral or obturator hernia[Preoperative diagnosis of lumbar hernia is common. Because specific physical findings are obvious, They are usually confused with lipoma or other superficial swelling of the flank. Unfortunately the diagnosis can be delayed and done after bowel obstruction. This was the case in our patient who was presenting signs of bowell obstruction before the lumbar hernia was identified. In some cases it is during diagnostic laparotomy for bowel obstruction that the diagnosis is done as also for abdominal wall hernias,2.Modern radiological modalities such as CT Scan, ultrasonography (US) and magnetic resonance imaging (MRI) can reliably make the early diagnosis of lumbar hernia, especially in elderly and frail patients having other abdominal wall hernias. X-ray fModern hernia repair using synthetic graft is recommended in lumbar hernia. But in case of strangulation, an incision for exploration or diagnostic laparoscopy should be preferred. In this patient, we perfomed a laparotomy since the patient presented late. Actually there are enough evidence that in abdominal wall hernias mortality is most often associated with delay in presentation and diagnosis. This caIntestinal obstruction and bowel necrosis, require emergency laparotomy with a midline incision. This approach gives the best exposure, allows reduction of the hernial content and facilitates bowel resection and abdominal toilet, if necessary. Other herniation sites can also be evaluated with this incision. Other types of approaches, such as preperitoneal, lumbar, can be applied when early diagnosis is made or in not strangulated cases.A laparoscopic approach was also envisaged. It is currently encouraged in emergency repair of complicated abdominal wall hernias. HoweverVarious repairs include primary suture of the orifice, muscle flaps, omentum, broad ligament, uterine fundus, prosthetic material and mesh plug. Without repair, compications rates of approximately 25% are reported.The use of mesh for repair of the strangulated hernias in which resection was performed is controversial. Some auMortality is reported to be between 10% and 50% in lumbar hernia. Unfavorable outcomes are commonly associated with delay in diagnosis and therapy, poor condition, elderly patients having coexistent diseases and strangulation with intestinal gangrene.Although lumbar hernias are rare, they should be considered when an elderly, thin patient presents with a bowel obstruction. Early diagnosis and treatment are the most important factors in decreasing mortality and morbidity; therefore, rapid action for diagnosis and therapy is essential.Written informed consent was obtained from the patient for the publication of this report and any accompanying images.The authors declare that they have no competing interests.MF, Conceived and wrote the manuscript. PF, Collected the data. AE, MNN, MS critically revised the manuscript. Overall responsibility MF. All authors read and approved the final manuscript.


Schizencephaly is an uncommon congenital disorder of cerebral cortical development. The defect is characterized by the presence of a cleft in the brain extending from the surface of the pia mater to the cerebral ventricles. The margins of the cleft are lined with heterotropic, dysplastic gray matter. The causes of schizencephaly are heterogeneous and can include teratogens, prenatal infection, maternal trauma, or EMX2 mutations.In the present paper, the authors described difficulties in employing diagnostic imaging in differentiating between type II (open-lip) schizencephaly and much more common intracranial fluid spaces of a different origin .In all the three cases, the treatment consisted in implantation of a shunt system; nevertheless, it should be emphasized that a surgical intervention in the third presented case  aimed at relieving the symptoms of intracranial hypertension—a directly life-threatening condition—since shunting is not a method of treating schizencephaly itself.Although proper interpretation of the character of intracranial fluid spaces is of significance for further therapeutic management, yet, the key decision as to the surgical intervention is made based on clinical presentation, predominantly on symptoms of intracranial hypertension. Schizencephaly is a rare congenital CNS malformation belonging to the group of cell migration defects, which develop between 2 and 5 months of gestation. The defect is characterized by the presence of a cleft in the brain extending from the surface of the pia mater to the cerebral ventricles. The margins of the cleft are lined with heterotrophic, dysplastic gray matter. The most common location of the anomaly is the frontal lobe and the region of the lateral sulcus –4.The malformation was first described by Wilmarth in 1887, while in 1946, Yakovlev and Wadsworth, basing their observations on five patients with neurological deficits and brain deformations, described two types of schizencephaly—type I (closed-lip), a form that does not communicate with the ventricular system, and type II (open-lip) that presents with communication with the ventricular system .The incidence of the defect is reported as 1/1650 patients with epileptic seizures and/or psychomotor retardation or 1.54/100,000 births . This maThe etiopathogenesis of schizencephaly has not been fully elucidated. One concept postulates the effect of external factors, which damage the developing fetal brain. In keeping with this idea, the cause of schizencephaly is middle cerebral artery stroke in consequence of an inflammatory process occurring in utero, e.g., as an effect of cytomegalovirus infection. The hypothesis is supported by the fact that the majority of schizencephaly clefts are seen in the lateral sulcus region and thus in the middle cerebral artery vasculature –14.Other risk factors of schizencephaly development include also maternal young age (below 20 years of life), no prenatal medical care , and abuse of alcohol and narcotic substances as well as using some medications by pregnant women . Cases of bilateral schizencephaly corpus callosum hypoplasia have been described in children with fetal alcohol syndrome (FAS) . AnotherIn keeping with the above theory, children with schizencephaly have a heterozygotic mutation of the EMX2 gene, which is a regulating gene for structural development of the prosencephalon . The advThe method of choice in diagnostic imaging of schizencephaly is MRI. CT is also useful, but to a lesser degree, since it provides poorer images of the gray matter, which are the key factor in differentiating between the malformation and other fluid-associated CNS abnormalities. Schizencephaly may be also diagnosed in prenatal or postnatal ultrasonography, but this is true for type II (open-lip) only.Medical imaging shows schizencephaly as a linear cleft lined with heterotrophic gray matter and extending from the cortical surface to the ventricular system. The gray matter within the cleft is dysplastic (polymicrogyria) , 17. CluOther concomitant pathologies that may be observed in patients with schizencephaly include hydrocephalus   and the Schizencephaly, especially its type II, is a severe irreversible CNS malformation, which is manifested by epilepsy, often refractory, and varying degrees of paralysis—hemiparesis in case of unilateral schizencephaly and quadriparesis in bilateral schizencephaly. The malformation is additionally associated with mental retardation and, in case of severe forms of schizencephaly located in the frontal lobe or in the lateral sulcus region, with varied forms of characteropathy. Type I schizencephaly (closed-lip) is characterized by a markedly milder course. The defect may be asymptomatic or diagnosed only in adult patients. It presents with epileptic seizures and mild motor deficits , 19, 20.As a rule, therapeutic management of both types of schizencephaly is conservative and predominantly consists in rehabilitation of motor deficits and mental retardation and treatment of epilepsy. Surgical treatment is undertaken only in some cases with concomitant hydrocephaly or intracranial hypertension , 23.A 3-year-old boy admitted to the University Children’s Hospital of Krakow following the first epileptic seizure. The patient complained of headaches and vomiting. Based on clinical presentation and medical history, intracranial hypertension was suspected. CT scan demonstrated bilateral fluid-filled spaces in the middle cranial fossa (the larger being situated on the left side), with hypotrophy of the adjacent cerebral structures. In addition, CT showed a minimal mass effect manifested as displacement of the longitudinal fissure of the brain to the right and compression and stenosis of the left lateral cerebral ventricle, as well as mild thinning of the squama of the temporal bone at the level of the malformation Figs.  and 2.FiIn differential diagnosis, consideration was given to the open type of bilateral schizencephaly or bilateral arachnoid cysts of the lateral sulcus, with type III on the left and type II on the right side .The CT scan favored the arachnoid cyst , yet schizencephaly could not have been ruled out based on a single CT scan and medical history of the patient. The scan was performed as an emergency procedure in a patient with severe and growing in intensity symptoms of intracranial hypertension, what did not allow for extensive, time-consuming diagnostic management.A decision was made on a surgical intervention consisting in implantation of a cysto-peritoneal shunt on the left side. Following shunting, the patient developed complications consisting in small intracerebral and paracerebral hematomas Figs.  and 4.FiIn subsequent follow-up scans, gradual resorption of hemorrhagic lesions was seen. The fluid-filled spaces were still present; albeit markedly smaller as compared to the initial scan, discrete asymmetry of the lateral ventricles persisted, but no displacement of the lateral fissure of the brain was detected. The patient improved clinically Fig. .Fig. 5A Following implantation of a drain system and clinical improvement of the patient, a MRI scan of the head was performed, which ultimately confirmed that the fluid-filled spaces in the two middle cranial fossae corresponded to arachnoid cysts.A 1-month-old girl was admitted to the University Children’s Hospital of Krakow due to suspected intracranial hypertension. She had been previously diagnosed and treated elsewhere due to epilepsy and extensive bilateral intracranial fluid-filled spaces. As it followed from her medical history, the mother had had a toxoplasma infection in pregnancy.A CT scan performed immediately upon admission demonstrated bilateral extensive fluid-filled spaces in the temporal and occipital lobes, which corresponded to markedly distended temporal and occipital horns of the lateral ventricles of the brain. Additionally, the scan showed agenesis of the corpus callosum and periventricular calcifications Fig. .Fig. 6Ma2O fixed-pressure ventriculoperitoneal shunt on the right side. No postoperative complications were noted  or in implanting a shunt system . Both methods are associated with a risk of such complications as postoperative bleeding, distension of the paracerebral fluid spaces forming subdural hygromas or hygro-hematomas, empyemas, meningitis, and hydrocephalus. In case when shunting is employed as the therapeutic modality . In such cases, a MRI scan shows areas of gliosis, i.e., glial scars, surrounding the fluid-filled lesions; medical history is also of assistance. Open-type schizencephaly should be also differentiated from hydrocephalus (a case discussed in the present paper) and from holoprosencephaly.Three presented above cases of extensive intracranial fluid-filled spaces were qualified for surgical treatment in view of clinical symptoms of acute intracranial hypertension. Inasmuch as surgical treatment of arachnoid cysts and hydrocephalus is a commonly employed and accepted therapeutic modality, implantation of a shunt system in the third presented case deserves a separate discussion. In this case, the employed modality addressed acute intracranial hypertension rather than schizencephaly itself. The effect of the treatment was a resolution of symptoms of intracranial hypertension, which is a direct life-threatening condition and requires a prompt intervention.On the other hand, schizencephaly itself and its associated symptoms are treated conservatively.


Eruption of tooth into extraosseous locations is an extremely rare condition. We report a case of a six-year-old girl child with tooth-like structure erupting from the right buccal mucosa. Clinical, radiographic, and histopathologic examination suggested the diagnosis of compound odontoma. Very few cases have been reported so far, where tooth has been located completely in the soft tissue and a variety of names have been used for that condition. A brief review of the literature and the ambiguity in naming the situation is discussed. Odontoma is the most common benign odontogenic tumour showing slow-growth and nonaggressive behaviour , 2. The Although the etiology is unknown, cases have been found to be related to local trauma, infection, from an exuberant proliferation of the dental lamina or its remnants . HitchinA six-year-old girl child was brought by her mother to the outpatient department with a chief complaint of tooth-like structure erupting from the right cheek region which started 2 months ago. The patient gave a history of mild pain and slight difficulty in mouth opening for the past two weeks. Her past medical and dental histories were noncontributory and she was otherwise a healthy child with a normal delivery. There was no prior history of trauma and no specific abnormality was noticed extraorally. On intraoral examination, a creamish-white, hard, 1 × 2 cm, molariform tooth-like structure was seen opposite to maxillary right first molar along the occlusal plane in the buccal mucosa . It was Radiologic examination revealed a radiopaque mass consisting of three tooth-like structures surrounded by a narrow radiolucent rim exclusively located in the posterior aspect of right buccal mucosa and no other ossifications were evident Figures  and 3. SOdontoma by definition refers to any tumour of odontogenic origin; however, it means a growth where both the epithelial and mesenchymal cells exhibit complete differentiation . OdontomNumerous reports have been reported where teeth are located in unusual locations  with mos “supernumerary or accessory teeth” if normal dentition is confirmed. The third case was by Liu et al. in a 4-year-old nonsyndromic girl child who found 2 mature molariform teeth in left buccal mucosa which was similar to the our case [A search of literature revealed only three cases with eruption of tooth from the cheek region. The first case of odontoma in cheek was reported by El sefdy Bakry in 1977 in a 9-year-old girl with Klippel-Feil syndrome . A seconour case . The autour case  also agrour case .In the present case, the orthopantomograph of the child revealed the entire primary dentition and the developing permanent molar, incisor, and canine tooth buds. No bony relation of the molariform tooth was noted. The radiolucent rim seen is due to the follicular tissue  which waIn our case, no evidence of osseous tissue or cartilage is seen. So, the term choristoma cannot be applied in the current scenario. Though “supernumerary” is the term suggested by various authors, we found three tooth-like structures (denticles) attached together rather than a fully formed separate tooth structure which was confirmed both macroscopically and microscopically. Henceforth, after careful evaluation and compiling all the terms, we confer the present case as ectopic compound odontoma of denticular type.


Neuroblastomas and Wilms’ tumors are frequent pediatric solid tumors. The first is frequently detected in the adrenal gland and the second develops in the kidneys. The extension through the vena cava and the lung metastases are frequent in Wilms’ tumors and are rarely seen in neuroblastoma. We present the cases of three children with abdominal tumors with thrombus in the inferior vena cava and pulmonary metastases demonstrating a stage 4 neuroblastoma. The three male patients were between 23 to 48 months old. They presented an abdominal mass, near the superior pole of the kidney. Thrombus of the vena cava was showed on imaging studies in all cases and pulmonary metastases were always found. Catecholamine metabolites were present in the first case and negative in the two others. Two out of three patients had a radical nephrectomy. The pathological analysis always found a poorly differentiated or undifferentiated neuroblastoma without MYCN amplification and confirmed the vein tumoral thrombus in the second case. The evolution of the first two patients was unfavorable and the third is alive. Invasion of the inferior vena cava and pulmonary metastases in children with neuroblastoma is uncommon and can modify the surgical management. The primary diagnostic considerations of a large palpable right or left upper quadrant mass in an infant or young child include neuroblastoma and Wilms tumor. Commonly, the evaluation of a child with an abdominal mass includes imaging and biological analysis to aid in the diagnosis of possible neoplastic disease and to facilitate the distinction between the two tumors. In rare cases, the diagnosis is not so simple. Many Wilms tumors present as large abdominal masses with invasion of the renal vein and extension into the inferior vena cava, and many clinicians consider the finding of tumor thrombus in the inferior vena cava suggestive of Wilms tumor . Eight months after his initial visit, the patient was admitted for severe cephalalgia with vomiting. A cerebral scan showed extensive metastases with important mass effect. The clinical state went on deteriorating with evidence of brain herniation and focal neurologic signs. The patient died two days after his admission.The patient was a boy who presented at four years of age with fever, diffuse abdominal pain, vomiting and loss of appetite. He was admitted to hospital where blood tests showed anaemia with neutropenia. The chest x-ray revealed an important pleural effusion, and an abdominal ultrasonography demonstrated a left 9 × 7.4 × 8.3 cm solid, well vascularized mass in the upper left quadrant. The physical examination done at that time described a palpable and painful mass in the upper left quadrant. An abdominal CT-scan showing a mass originating from the superior aspect of the left kidney, encapsulated, of 8.2 × 8.7 × 8 cm of size. Its centre was necrotic without calcifications  followed by intensification therapy with autologous bone marrow transplantation, abdominal radiotherapy and immunotherapy. Twenty one months after the end of the treatment and forty two months after the diagnosis, the patient is actually in complete remission.A boy presented to his local physician for fatigue at the age of forty eight months. The physical examination revealed an abdominal mass for which the boy was sent to hospital. At arrival, the physical examination showed an easily palpable mass in the right abdomen extending from the costal margin to below the umbilicus and crossing the mid-line. The neurological examination was normal. Blood tests demonstrated an anemia with normal values for HVA-VMA. An abdominal ultrasonography showed a right renal mass with tumour in the right renal vein with extension to the inferior vena cava which was confirmed on the CT-scan Figure a. Bone mWilms tumor and neuroblastoma are the two most common abdominal neoplasms in young children. Both tumors could be localized in the upper abdominal quadrant and to discriminate between them could be difficult. It is essential to establish the correct diagnosis before surgery because it will change the surgical management. With a neuroblastoma, the surgeon will then determine during surgery if it is possible to separate or not the tumor of the adjacent kidney because tumors with thrombus in vena cava and pulmonary metastasis could directly invade the kidney.However, each disease has specific characteristic features that may suggest a working diagnosis to guide evaluation and the initial surgical approach before histologic tissue analysis. The first clinical difference between the two tumors is the age at diagnosis. The peak incidence of neuroblastoma is at age 19 months and the distribution of cases by age clearly shows that this is a disease of infancy and early childhood  in urine is one of the most sensitive methods to diagnose neuroblastoma. More than 90% of the neuroblastomas are positive for one of the three parameters (Strenger et al. The histological analysis of the neuroblastoma with thrombus in inferior vena cava revealed an aggressive tumor poorly or undifferentiated with intermediate or high MKI and unfavorable histology (Mehta and Lim-Dunham In conclusion, these three cases illustrate the importance of complete staging of an abdominal mass in view of treatment. If these cases are very infrequent, they typically demonstrated that in patient with invasion of the inferior vena cava and renal vein associated with pulmonary metastases, neuroblastoma must be eliminated even if it was a non-secretory tumor, in particular if surgery is considered. Not only does it complicate an intervention, it also dictates the prognosis and following treatment.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.


Patients that present with multiple primary malignant neoplasms are increasingly encountered, but the treatment of such patients presents specific challenges and long-term survival is rare. The present study reports the case of a 45-year-old female diagnosed with three rare, distinct primary malignant neoplasms, including epithelioid hemangioendothelioma (EHE) of the brain, Ewing’s sarcoma of the lumbar 2 vertebra and a malignant solitary fibrous tumour (SFT) of the liver, at different time points. The patient underwent multidisciplinary treatment according to the diagnoses, including radial resection of all primary lesions, chemotherapy  and radiotherapy, to treat Ewing’s sarcoma and metastases of EHE and malignant SFT. Following these treatments, the patient survived for >14 years. Multidisciplinary treatment regimens based on surgery can lead to long-term survival of patients with multiple asynchronous rare primary malignant neoplasms. The present study reported that multidisciplinary treatment regimens based on surgery can lead to the long-term survival of patients with multiple asynchronous rare primary malignant neoplasms. The occurrence of multiple primary malignant neoplasms (MPMNs) is increasing, with the reported incidence ranging between 0.734 and 11.3% in India . FurtherStudies on the survival of patients with MPMNs are limited, possibly due to the particularly heterogeneous nature of this group . It has In the current study, a patient presented with three rare distinct primary malignant neoplasms, consisting of epithelioid hemangioendothelioma (EHE) of the brain, Ewing’s sarcoma of the lumbar vertebrae and malignant solitary fibrous tumour (SFT) of the liver. The primary Ewing’s sarcoma affected the lumbar 2 (L2) vertebra, as did metastases of the EHE and malignant SFT. The patient received multidisciplinary treatment and survived for >14 years. Written informed consent was obtained from the patient’s family.A 31-year-old female presented with severe headache upon wakening for 6 months and was referred to The First Affiliated Hospital of Sun Yat-sen University  in May 1999. No other symptoms were noted during a physical examination. Head computed tomography (CT) scans revealed a slightly hyperintense mass in the left parietal region. Radionuclide bone scanning, chest radiography and abdominal ultrasonography revealed no evidence of malignant lesions in other organs. The patient underwent surgical excision of the left parietal lesion. Histopathological analysis of the resected tumour revealed an EHE with a cluster of differentiation (CD)34-positive and factor VIII-associated antigen-positive immunophenotype. A post-operative follow-up, which lasted for five years, revealed no evidence of recurrence.In November 2004, the patient reported lower back and left leg pain. A magnetic resonance imaging (MRI) scan revealed a compression fracture and slightly hyperintense mass in the L2 vertebra, as well as compression of the spinal cord . FollowiIn April 2007, the patient presented with recurrent lower back and left leg pain. An MRI scan revealed a posterior enhancing mass and spinal cord compression at the L2 vertebra level, with involvement of the bilateral vertebral lamina and superior processes . The invIn May 2009, the patient presented with an abdominal mass. An abdominal CT scan revealed a hyperintense mass in the S5 hepatic segment, with a maximum diameter of 10 cm . The invIn March 2010, the patient presented again with recurrent lower back and left leg pain. MRI and PET-CT scans revealed a posterior enhancing mass and spinal cord compression at the L2 vertebral level . RecurreIn April 2013, the patient experienced fever, dyspnoea and bone pain throughout the body. Radionuclide bone scanning and chest radiography revealed multiple bone metastases and a pulmonary infection. Laboratory examinations revealed pancytopenia. The patient was treated with palliative care and symptomatic therapy, but succumbed to respiratory-circulatory failure in June 2013.Multiple primary neoplasms in a single patient were first reported at the end of the 19th century , who speEwing’s sarcoma of the bone most frequently involves the pelvic bones and femur , and altHepatic SFT is rare  and exhiIn the present study, the interval between the occurrences of the first two primary neoplasms was eight years, and the subsequent intervals were two years. Similar to other reported cases, the mean interval between the diagnoses of the first two primary tumours is always greater than the interval between the detections of subsequent neoplasms, the cause of which has yet to be elucidated . There aTreatment strategies for patients with MPMNs differ from the strategies used for patients with metastatic or recurrent cancer. Therefore, a novel tumour in a patient with cancer should be considered not only as recurrence or metastasis but also as a different primary malignancy. For asynchronous primary malignant neoplasms, it has been recommended for each tumour to be evaluated and staged as an independent tumour and treated aggressively with curative intent to achieve the maximum therapeutic benefit . If surgSurgical removal is the recommended treatment for EHE , and preCurrent protocols for the treatment of Ewing’s sarcoma include radical excision, which generally exhibits a good prognosis , followeThe recommended treatment for hepatic SFT is radical removal of the tumour with clear margins of resection, with post-operative adjuvant therapy reserved for cases of incomplete resection or cases exhibiting histopathological features of malignancy . In the et al reported the mean overall survival of patients with MPMN to be 97.2±15.0 months (Depending upon the site, pathological pattern, tumour variant, type of treatment and clinical series, the five-year survival rate of patients with MPMN can range between 33 and 65% ,38. Gurs0 months . The patThe findings of the present study have the following clinical implications. Firstly, if pre-operative biopsy is not performed, a correct diagnosis may not be obtained, thus delaying treatment for MPMNs. A more standardized diagnosis procedure, including pre-operative biopsy, would be required in future for an improved outcome. Secondly, with the exception of recurrence of primary lesions or metastases, the occurrence of novel MPMNs should be considered during follow-up. Thirdly, multidisciplinary treatment can substantially increase the survival of patients with MPMNs.In the present study, multidisciplinary treatment based on surgery exhibited a significant survival advantage for a patient with a rare case of MPMN. However, further research with additional cases, particularly including patients with more than two MPMNs, is required to definitively prove the clinical benefit of multidisciplinary treatment for the overall survival of patients with MPMNs.