Lymphangiomas are rare benign tumors. They are preferentially located in the head, neck, and axilla in children.However, lymphangiomas in the peritoneal cavity are extremely rare, particularly in adults. In the abdomen, lymphangiomas occur most commonly in the mesentery, followed by the omentum, mesocolon, and retroperitoneum. The etiology is unclear, but they are considered primarily to congenital in origin. Preoperative diagnosis is often difficult due to the frequent silent clinical course. Radiological investigations are a useful diagnostic tool, but definitive diagnosis is confirmed by histopathology after a complete surgical resection. The report describes five cases of adult patients with mesenteric lymphangioma.A 67-year-old female presented with a dull abdominal pain in the right hypochondrium and flank of a 1-month duration and presence of constipation.An abdominal examination revealed distension, hyperactive bowel sounds and tenderness during palpation in the central abdominal quadrant and right flank.An ultrasonographic abdominal scan revealed the presence of a cystic mass (max diameter 32 mm) in the retrocecal adipose tissue.A computed tomography (CT) abdomen scan confirmed the presence of the cystic lesion and its dissociability from the cecum, appendix and ileum. In addition, the CT scan revealed the presence of multiple ipodense formations in the body and tail of pancreas.Neoplastic markers  were negative.During the hospital stay an abdominal MR was performed. The exam revealed in the retrocecal adipose tissue the presence of a fluid-filled formation with a diameter of 35 mm that appeared indissociable from the cecum for the presence of a tissutal connection Figure . Other cThe patient underwent exploratory surgery: at laparotomy a yellowish ovalar formation with a diameter of 6 cm was found arising from the mesentery of ileum with partial adhesion of the appendix. The lesion had a cystic aspect and was fluid-filled. We performed a total excision of the lesion and appendicectomy. Histopathological examination was consistent with the diagnosis of mesenteric lymphangioma. The patient has been followed-up for 6 months and no recurrence occurred.A 68-year-old male patient presented with a painless abdominal swelling of two years duration. A clinical examination revealed the presence of an abdominal mass extending from the right hypocondrium to the right iliac fossa.Abdominal ultrasonography and CT scans revealed the presence of an ipodense and fluid – filled formation, 10 cm in diameter which extended from the right hepatic lobe to the ascending colon. Posteriorly the mass bordered the anterior aspect of the right kidney. In addition, a left kidney agenesia was present.An abdomen MR was also performed: presence of a 10-cm fuid – filled mass with internal septa that extends from the hepatic hilum to the right colon, displacing medially the second duodenal portion and anteriorly the right colonic flexure. At MR this formation presented a strict contiguity with the common bile duct Figure .In consideration of the clinical symptoms, we performed a laparotomic exploration.At laparotomy we found an ovalar formation of cystic aspect, 10 cm in diameter, which was closely adherent to the biliary tree. The dissection was conducted closely the CBD and the mass appeared originating from the cranial extremity of the common hepatic duct. The complete excision determined a microperforation of the biliary tree that was repaired with a reabsorbable suture. An abdominal drainage was positioned and it was removed in the 7 th postoperative day.A citologic examination was conducted on the fluid aspirated from the cyst and it revealed the presence of chronic inflammatory cells. The histopathological examination showed the presence of dilated lymphatic vessels and endothelial proliferation: this pattern was consistent with the diagnosis of cystic emolymphangioma.A 80 – year – old male patient complained of diffuse abdominal pain. A clinical examination revealed an occlusive state. We performed an abdominal ultrasonography and CT that revealed in left hypocondrium the presence of an anechogenic ovalar formation of 8,6 x 4 x 5 cm, with internal septa and a thin wall, with polycyclic edge . This formation appeared adherent to the gastric body and the jejunal loops.Neoplastic markers  were negative.The patient underwent a laparotomic exploration and the cystic formation was completely excised. Figure The histopathological examination was consistent with the diagnosis of mesenteric cystic lymphangioma. Figure The patient has been followed-up for 4 months and no recurrence occurred.A 77 – year – old male patient complained of a dull abdominal pain of one year duration localized in the periumbilical region. This pain was associated to abdominal swelling and early satiety after meals.An abdominal examination revealed the presence of a solid mass that occupied the epigastric and mid-epigastric region. An abdomen CT showed a huge mass composed by multiple confluent cystic lesions displacing inferiorly the small bowel. Figure At laparotomic exploration, the mass appeared composed by multiple cysts containing a citrine fluid. A careful dissection was conducted to excise the mass without spillage of fluid in peritoneal cavity.The histopathological examination confirmed the diagnosis of mesenteric cystic lymphangioma.The post-operative course showed no complications. The patient has been followed up for 1 year and no recurrence was observed.A 65-year-old male patient was diagnosed ten years before the presence of an intra-abdominal limphangyoma. The mass was followed-up with annual abdominal – MR and ecotomography.At abdominal – RM the formation was fluid- filled with internal septa and measured 18x12 cm : it was placed between the posterior aspect of the stomach and the pancreas with lateral extension in the hepatorenal space.Figure  He had nIntra-abdominal mesenteric lymphangioma is an uncommon tumor that is usually manifesting in early adulthood. This tumor has an incidence of approximately 1/20,000 and 1/250,000 hospital admissions, and it is more frequent in men than women with a M/F ratio of 3:1. Intra-abdominal lymphangioma are almost always found in the retroperitoneum, followed by mesentery. In intraperitoneal sites, the small bowel mesentery (70%) is the commonest site, with 50–60% of all cysts located in the ileal mesentery. 8]The ultrasonographic presentation of a mesenteric lymphangioma is described as a cystic lesion with multiple thin septa (honeycomb or cobweb pattern). Ultrasound-guided diagnostic aspiration usually yields a chylous aspirate. On CT imaging, mesenteric lymphangiomas appear as a uni- or multilocular masses with enhancement of the wall and septum by contrast medium . HoweverHowever, the definitive diagnosis of lymphangioma is based on histopathology and immunochemistry : the lining mesothelial cells are immunoreactive for cytokeratin and negative for factor VIIIIs. Double staining with Prox1 and CD31 is the most reliable method for characterizing lymphangioma endothelial cells.In our patients we performed a complete surgical excision of the mass, that is the treatment of choice for cystic lymphangioma, even if asymptomatic. Surgical enucleation without damage to the blood supply of the bowel is also suggested, with the prognosis being generally favorable, but with increasing tumor size radical resection becomes more difficult and local recurrence more probable ,13. InfiThe prognosis after adequate excision of the cystic tumors of the mesentery is considered to be excellent.Drainage has been suggested as a modality of treatment in high – risk patients but is often unsuccessful because of recurrence and for the risk of perforation of the mesentery during the drainage of the lymphangioma . InstillAdjuvant treatment with OK-432, a biological response modifier with antitumor effects, has been shown to prevent further enlargement of small localized remnant cysts after surgery .In our experience, we follow up the patients with a clinical examination and an abdominal ultrasound at 3 , 6 and 12 months after the operation , then annually. During a median follow up period of 11,5 months (4 – 48 months) no recurrence occurred (Table Mesenteric lymphangioma in adult is a rare disease: it may present as an abdominal swelling or acute intestinal obstruction. Preoperative diagnostic tools are ultrasound and abdomen CT or MR. Complete surgical resection is the ideal modality of treatment for mesenteric lymphangioma. Definitive diagnosis is confirmed by histopathology.


Phyllodes tumors (PTs) of the breast are a rare type of tumor that account for <1% of breast tumors in females and usually present as a large lump of 3–5 cm in size. Surgery is the first line of treatment for PTs, and chemotherapy and irradiation may be useful in certain patients but not all. In the present study, the case of a 63-year-old female patient with a huge PT in the left breast is described. On physical examination, the patient presented with a huge mass of ∼45 cm in diameter, weighing ∼11 kg, and a composite of multiple chronic diseases. The breast and pectoris major and minor were excised. Post-operatively, the patient recovered well and to date there has been no evidence of local recurrence or distant metastasis. Phyllodes tumors (PTs) of the breast are rare biphasic fibroepithelial tumors, characterized by the proliferation of stromal and epithelial cells ,2. They A 63-year-old female patient was admitted to the Galactophore Department, Bao Ji Central Hospital with a huge lump in the left breast. The patient stated that they had identified a thumb-sized lump two years earlier, but that they had ignored it. From July 2011, the patient observed that the lump was growing rapidly; in February 2012 the lump had become so large that the patient’s left chest was entirely covered with a solid, irregularly-shaped, somewhat moveable mass of tremendous size and the patient was barely able to lie in the prone position. Family members then finally convinced the patient to seek medical attention. Upon physical examination, the patient presented with an elastic hard mass in the left breast that was 45 cm in diameter, with a circumference of 97 cm . Rapid gDue to the non-compressible nature of the mass and the size of the tumor, regular breast examinations, including mammography and ultrasound studies, could not be used, while a computed tomography (CT) scan of the chest revealed a solid mass. Total body scintigraphy was negative. Comprehensive examinations and detailed assessments were additionally performed on the patient due to the presence of high blood pressure, diabetes, atrial fibrillation and cerebral infarction. In order to ensure the long-term success of the treatment, a consultation with the Respiratory Medicine, Vasculocardiology, Neurology and Anesthesiology departments was organized. Antihypertensive, glycemic control, oxygen uptake and antiarrhythmic therapies were administered prior to surgery. The pectoris major and minor had atrophied and were tightly adhered to the tumor due to long-term compression, so the surgeon decided to resect the mass along with these muscles. The mass was resected subsequent to 3 hours of surgery . The posIn view of the benign nature of this tumor, no chemotherapy or radiotherapy was administered following surgery. The patient recovered well and to date there has been no evidence of local recurrence or distant metastasis.PTs, also known as cystosarcoma phyllodes, were first named by Johannes Muller ,5. MullePTs are rare tumors of the breast that account for 0.4–1% of all breast tumors, and the average age of the affected patients is 45 . At preset al  are common in elderly females and exhibit frequent metastasis (lymphatic and blood-borne) with a high mortality rate . In certain cases, there may be a requirement to resect the pectoris major and minor. If the mass is >5 cm or malignant, wide local excision is recommended. Although chemotherapy, radiotherapy and local lymph node dissection are not used in conventional treatment modalities for PTs, they may be useful for patients with aggressive tumors, positive margins and high mitotic rates, as well as for those with recurrence . The recIn summary, PTs of the breast are rare and the present malignant PT is notable due to its large size and weight of ∼36×40×18 cm and 11 kg, respectively, in combination with the presence of other chronic diseases. We suggest that this report may be useful for the diagnosis and treatment of future PT patients.


Introduction. Pseudomyxoma peritonei (PMP) is an uncommon surgical entity. We report a case of aggressive disease with an unusual clinical presentation and we analyze current data on diagnosis and management of PMP. Case Presentation. A 71-year-old male patient presented with intermittent diarrhea and loss of appetite during the last two months, without any other classic symptoms of PMP. The clinical examination was misleading due to patient's obesity. The radiological evaluation revealed ascites of the abdomen and possible mucocele of the appendix, whereas the laboratory exams showed high values of specific tumour markers. The patient underwent an exploratory laparotomy for definite diagnosis. Biopsies and immunohistochemical examination confirmed the diagnosis of an aggressive and extended peritoneal mucinous carcinomatosis (PMCA). The patient was programmed for adjuvant systematic chemotherapy, which was not completed due to progression of the disease. Conclusions. Progressed PMP can present with unspecific symptoms that mislead diagnosis. Cytoreductive surgery in combination with systematic chemotherapy could be appropriate for aggressive PMCA, even with an unfavourable prognosis. Pseudomyxoma peritonei (PMP) is an uncommon tumor known for its production of mucin in the abdominal cavity. If left untreated, mucin will eventually build up to the point where it compresses vital structures, such as the colon, the liver, kidneys, stomach, spleen, and pancreas . For yeaRegarding its classification, PMP is a broad descriptive term embracing a wide spectrum of biological behaviour of neoplasms, from the benign to the frankly malignant lesion. Ronnett and colleagues proposed a classification distinguishing “disseminated peritoneal adenomucinosis” (DPAM) from “peritoneal mucinous carcinomatosis” (PMCA) . FurtherRegarding the clinical presentation, pseudomyxoma peritonei is a disease more commonly seen in women , with an average age of 53 years who usually present with increasing abdominal girth and a primary ovarian lesion . Other pThe usual clinical features of this tumor are increasing abdominal girth (40%), bilateral or unilateral ovarian tumors 20%), hernia sac tumors (20%), appendicitis-like syndrome 10%), and infertility (10%) 0%, and i, 6. Narr0%, herniProper diagnostic investigations include an ultrasonographic examination of the abdomen initially, whereas computed tomography scans will reveal further information about the extent of the disease . AdditioWe report a case of PMCA in a male patient with an unusual clinical picture, who underwent an exploratory laparotomy and was programmed for systemic chemotherapy afterwards.Male patient, 71 years old, presented to the emergency department complaining of episodes of intermittent diarrhea without any fever, vomiting, or abdominal pain. The symptoms had appeared, for the first time, two months prior to the patient's presentation. During the clinical examination, there were no palpable masses of the abdomen and an abdominal distention could not be clearly diagnosed, due to patient's obesity. The patient complained also about loss of appetite during the last two months. Regarding the standard serum investigations, we observed anemia (HCT 30%), without any other abnormal values of biochemical tests. There was an ultrasound requested, if there was a significant amount of intraperitoneal ascites discovered. Tumor markers were also measured in serum, where Ca 19-9 and CEA serum levels were higher than normal values (Ca 19-9 = 204.4 IU/mL and CEA = 83.5 ng/mL). The computed tomography (CT) control of the abdomen showed that the peritoneal cavity included a significant amount of ascites, and a cystic mass (8 cm in diameter), with small calcifications within its wall, was found in the right iliac fossa, possibly a mucocele of the appendix. Moreover, no enlarged lymphnodes of the abdomen were observed. Colonoscopy and gastroscopy findings were negative for pathology. A small amount of ascitic collection was drawn percutaneously for cytologic examination. The result was a small number of observed lymphocytes in the setting of large amount of mucous matrix. There were no malignant cells observed. The patient underwent an exploratory laparotomy for definite diagnosis. The abdomen was full of gelatinous ascitic liquid and diffuse implantations of the peritoneum. Extended adhesions of the small intestine to the abdominal wall due to neoplastic implantation were found as well. A large mucocele was identified arising from the appendix. The disseminated picture of the disease indicated a cytoreductive surgery procedure. Biopsies were taken from the abdominal wall and the mesenterium. They revealed elements of peritoneal mucinous neoplasm of high malignant grade and microscopic lesions of low differentiated character. The histopathology and further immunoassays (CK7 negative and CK20 positive) confirmed the diagnosis of a disseminated PMCA of appendiceal origin. The patient underwent 4 cycles of systematic chemotherapy (5-fluorouracil based) as well, which was interrupted due to secondary toxic effects and progression of the disease in the abdomen.In the past, pseudomyxoma peritonei (PMP) has been attributed to a variety of primary tumors , 2. ThisAs mentioned above, PMP has multiple clinical manifestations that lead to difficulties in definitive diagnosis and timely treatment . As sympRegarding the preoperative evaluation of possible biomarkers, PMP patients with preoperative elevated tumor markers such as CEA and Ca 19-9 are at increased risk of developing recurrent disease despite aggressive therapy . LikewisTreatment of PMP is variable, both due to the rarity of the disease and to its frequently slow-growing nature . CurrentLikewise, regarding the proper management of aggressive forms of PMP, there is still a controversy among the researchers. Recent studies support that cytoreduction with peritonectomy plus HIPEC is a safe procedure that suggests an improvement to the survival rates, even in aggressive cases , 22. HowFinally, pseudomyxoma peritonei may recur following cytoreductive surgery and systemic chemotherapy, as seen in our case, especially when the disease is diagnosed in an advanced stage . PeriodiWe have described an unusual case of aggressive and progressed PMCA, with a misleading clinical presentation. There is no consensus regarding the proper management of aggressive cases. Cytoreductive surgery in combination with systematic chemotherapy could be appropriate for aggressive PMCA, even with an unfavourable prognosis.


Authors report the case of a 51-year-old man, presenting with epigastralgia of recent onset. Physical exam was unremarkable. Endoscopy revealed a large, ulcerated, submucosal, antral tumor. CT scan reveals an antral mass with fat attenuation. The patient underwent a total gastrectomy. Macroscopic examination identified in the antral wall a 9-cm, well-circumscribed, nodular lesion, with a yellow, greasy cut surface. On histological examination, the tumor was composed of a mature adipocytes proliferation, showing significant variation in cell size, associated to some lipoblasts. Nuclei were sometimes large, slightly irregular, but without hyperchromasia nor mitosis. Diagnosis of a well-differentiated liposarcoma was suspected and molecular cytogenetic analyses showed no MDM2 nor CDK4 gene amplification on fluorescent in situ hybridization. The diagnosis of lipoma was made. Twelve months following surgery, the patient is doing well. Lipomas are relatively uncommon tumors in the gastointestinal tract, often located in the right colon . GastricA 51-year-old man, in otherwise excellent general health, presented with epigastralgia of recent onset. The patient denied any history of gastrointestinal hemorrhage, nausea, vomiting, and change in bowel habits, fevers, or melena. Physical examination was unremarkable. Endoscopy revealed a soft, large, ulcerated, submucosal mass in the gastric antrum. Multiple biopsies were obtained but were all superficial, showing unspecific inflammation of the gastric mucosa. The abdominal CT scan revealed a round, well circumscribed, low-attenuation, gastric antral mass, measuring approximately 9 cm in diameter . The patGross examination of the surgical specimen identified in the antral submucosa, a well-circumscribed, smooth, nodular lesion, measuring 9x7.5x5 cm, with a homogeneous, yellow, greasy cut surface. Overlying mucosa was partially ulcerated .Histological examination revealed a submucosal tumor , composeGastric lipoma is a rare, benign lesion, accounting for only 5% of gastrointestinal tract lipomas and fewer than 1% of all gastric tumors , 4. TheyClinical symptoms depend on the size of the lesion. When small (< 2cm), lipomas are usually asymptomatic and they are often discovered fortuitously , 3, 4. WEndoscopically, gastric lipomas typically appear as a soft, sharply defined, submucosal mass, often yellow in color as opposed to the surrounding pink mucosa. Typically, 3 clues help to identify these lesions as lipomas on endoscopic examination: the “tenting sign”, in which the overlying mucosa is easily retracted with the biopsy forceps; the “cushion sign” which occurs when the forceps produces a soft, cushioning indentation when applied to the lipoma; and the “naked fat” sign, which refers to exposed adipose tissue on the surface of the lipoma that pokes through the normal overlying mucosa after multiple biopsies of the normal mucosa are performed . OccasioDiagnosis is confirmed only after histopathological examination of surgical specimen. Standard biopsies are often inadequate because of the submucosal location of the tumor. On gross examination, gastric lipomas appear as solitary, smooth, soft masses, most often more than 2 cm in diameter, with an average size of 6.5 cm. On cut surface, they appear bright yellow, round, greasy, and encapsulated unless they have become infracted. Large lesions may be ulcerated, like in our patient , 2, 4. HWhen the tumor is large, there is a progressive tendency for the submucosal mass to extrude into the lumen, leading to traumatic and inflammatory changes and resulting in necrosis, ulcération, and hemorrhage. Secondary changes including nuclear hypertrophy, hyperchromasia, fat necrosis, fatty cysts, and foamy macrophages may be observed. Lipoblasts and variation in cells size could, in addition, be present. The tumor can, in this situation, mimic a WDLS , 7, 8. IThe treatment of choice for a symptomatic gastric lipoma is circumferential excision with a clear margin of normal tissue. Due to the benign nature of this lesion an extensive surgical procedure such as an extended gastrectomy is not necessary. Simple local enucleation or partial gastric resection is sufficient to remove the lipoma without fear of relapses or malignant degeneration . The proFatty tumors are rare in the gastrointestinal tract. Differentiating benign from malignant neoplasms is sometimes difficult in morphologic features. Cytogenetic procedures are, in these cases, the only means for an accurate diagnosis.


Adenofibroma is an extremely rare benign biphasic neoplasm that is classified into the mixed epithelial and mesenchymal tumor group. These tumors tend to occur in postmenopausal and elderly women. We report the case of a large polypoidal mass per vagina occupying the whole pelvis in a young woman. Preoperative biopsy showed benign epithelial and mullerian mesenchymal components suggestive of mullerian adenofibroma. Total hysterectomy with bilateral salpingectomy was done. The diagnosis of papillary adenofibroma of cervix was made. The total surgery assured complete excision and permitted adequate sampling to exclude malignancy. Uterine adenofibroma was first described by Ober in 1959 as a form of mixed mesodermal tumor . CervicaA 21-year-old unmarried lady with history of brownish discharge per vagina and lower abdominal pain for the past 2 months presented with acute retention of urine. There was no history of mass per abdomen or mass per vagina. Her last menstrual period was 2 months back. The previous menstrual cycles were regular. There was mild pallor on examination. The abdomen was soft and no mass palpable. Ultrasonography showed large soft tissue lesion embracing the uterus all around. Bilateral ovaries were normal. Computed tomography scan showed a large well-defined heterogeneously enhancing mass lesion measuring 10.4 × 11 × 14 cms. This appeared to arise from the vagina extending superiorly up to the level of the pelvic inlet . The lesMixed epithelial-mesenchymal tumors contain both epithelial and mesenchymal elements as active participants in the neoplastic process. This tumor group includes adenofibroma and adenosarcoma. Women with uterine adenofibroma tend to be elderly and present with either abnormal uterine bleeding or postmenopausal bleeding . There aIt is important to distinguish adenofibroma from adenosarcoma. Adenofibroma contains a mixture of histologically bland epithelium and mesenchyme. It has broad papillary fronds covered by epithelium projecting from the surface of the neoplasm and extending into cystic spaces within it. Epithelium may be cuboidal as in this case. A mixture of various types of epithelia, including endocervical, tubal, and squamous often occur within the same neoplasm . The mesTotal hysterectomy is the preferred treatment for an adenofibroma because the neoplasm may recur if it is incompletely curetted or excised . Total hAdenofibroma should be considered in the differential diagnosis of uterine polyps even in young women. Thorough preoperative evaluation and complete surgical management is important to rule out malignancy and to prevent recurrences.


Background. Although some mature cases of teratomas have recently been described in the cervix, they are not commonly found in the uterus, especially in immature forms. An immature uterine teratoma relapsing after surgery as malignant neuroepithelioma has never been reported in the literature. Case Presentation. We describe a case of immature teratoma which occurred primarily in the uterus in a 56-year-old female. Treatment consisted of total simple hysterectomy. Three months after surgery, the patient relapsed with voluminous pelvic mass and was treated with bleomycin, etoposide, and cisplatin-containing chemotherapy regimen. Conclusion. In this report and according to the pertinent literature, clinical and pathological features and management of uterine immature teratomas are discussed. The mainstay of treatment is surgery. The prognosis of this unusual disease remains uncertain. Teratomas are tumors commonly composed of multiple cell types derived from one or more of the three embryonic germ layers: ectoderm, endoderm, and mesoderm. These tissues are foreign to the location in which they are found. Teratomas may be classified as mature or immature on the basis of the presence of immature neuroectodermal elements within the tumor. Mature tumors have no immature elements. Teratomas are the most common germ cell tumors. They usually arise in the gonads and often occur in infancy and childhood. Extragonadal teratomas are rare and mainly develop in midline structures . Primary teratomas of the uterus have rarely been reported since Mann's first description of this entity in 1929. Here we report a case of uterine immature teratoma in a 56-year-old woman which is an exceptional presentation of this tumor and review the relevant literature.A 56-year-old Moroccan woman with no significant past medical history presented to gynecological consultation in June 2009 with a one-month history of dysuria, burning urination, and progressive lower abdominal distention. She had previously given birth to one child. The patient was menopausal since ten years ago.Clinical examination revealed a painful distended abdomen with bulky uterus. The adnexa were normal on palpation.Transabdominal ultrasonography demonstrated a solid uterine mass. The ovaries were not visualized and there was no ascite. Magnetic resonance imaging (MRI) of the pelvis showed a voluminous and heterogeneous uterine mass measuring 12 × 10 cm. The lesion was located at the uterine corpus and was displacing bladder and rectum. The ovaries appeared normal and there was no lymphadenopathy. The tumor appearance suggests a calcified fibroma.The patient underwent a total simple hysterectomy. During surgery, a 12 cm mass was attached to the uterine corpus. The en bloc resection of this voluminous mass was impossible and the tumor was fragmented. Up to 20 pieces were removed. On sectioning, the fragments were macerated and showed various tissue consistencies.Microscopically, the tumor was an immature teratoma, composed of a mixture of embryonic mature and immature elements. The tissue types found included nerve ovoid cells involved in a fibrillar background, active cartilaginous foci, and glandular formations. The immature component was dedifferentiated cells around epithelial tubes with the presence of mitoses . Our patient recovered well from surgery and a surveillance approach was taken. Three months later, the patient presented with vaginal bleeding. On digital examination of the vagina, she was found to have friable mass filling the upper half of the vagina with severe bleeding.Pelvic and abdominal computer tomography (CT) scan revealed a bulky mass of 14 × 8 cm filling the pelvic cavity. The tumor was involving the bladder and the rectosigmoid with left ureterohydronephrosis. The scan of the thorax showed no evidence of extended disease.βhCG) and lactate dehydrogenase (LDH) were within normal limits. Alpha-fetoprotein (AFP) was raised at 556 ng/mL.Tumor markers including beta human chorionic gonadotropin  based on bleomycin , etoposide , and cisplatin  every 3 weeks, followed by surgical removing of residual mass. Unfortunately, our patient died when she returned home few days after the first course of chemotherapy.Extragonadal germ cell tumors are rare, comprising 1%-2% of all germ cell tumors. However, they can arise anywhere along the migration route of germ cells . Also, tTeratomas are the most common germ cell tumors. The occurrence of primary uterine teratoma is exceptional. In 1929, Mann was the first to describe a case of primary mature teratoma in the uterus . Since tSome interesting theories about the histogenesis of these tumors can be discussed. The first one suggests that uterine teratomas arise from primordial fetal germ cells that had abnormal migration from the fetal yolk sac endoderm to the gonadal ridge during early embryogenesis. As uterus is a midline organ, this supposes that primordial cells had a midline passage . AnotherUterine teratomas occur typically in the second to fourth decades of life. They may rarely occur in the case of postmenopausal women such as our patient. Clinically, there are no specific symptoms, and patients may present with abnormal vaginal bleeding, pelvic pain, or lower abdominal distention . PressurA diagnosis of teratoma in the uterus cannot be made when imaging techniques do not display the characteristic findings. In CT or MRI, the appearance is variable with heterogonous, predominantly solid lesions or cystic or mixed solid and cystic lesions with small foci of fat or calcifications. The differential diagnosis might include uterine fibroid and lipoleiomyoma . In prevDue to the extreme rarity of the immature uterine teratoma, there is no uniform consensus regarding treatment. However, the mainstay of treatment is surgery consisting of a complete tumor excision or radical hysterectomy with or without pelvic and para-aortic lymphadenectomy . Also, tImmature uterine teratoma is an uncommon presentation for a uterine mass. Because of the extreme rarity of this disease, there is no therapeutic standard at the moment and the prognosis remains unknown.


Intracranial germ cell tumors are uncommon and account for only 0.3–3.4% of all intracranial tumors. Teratomas are a subset of these neoplasms, and their finding in brain structures is exceptionally rare, and occurrence within the skull base is quite novel. The authors report the case of a 57-year-old male patient who presented with vision changes, incontinence, ataxia, and altered mental status of 1 week's duration. Imaging revealed a large intrasellar mass with suprasellar extension, involvement of the ventricular system, and marked hydrocephalus with the enlargement of the lateral and third ventricles. The patient underwent a pterional craniotomy/transsylvian approach for resection of the mass. Postoperative histological examination of the resected mass was confirmatory for a mature cystic teratoma. This was followed by radiotherapy, stereotactic radiosurgery, and adjuvant radiotherapy. At the most recent followup, approximately 4 years later, the patient is doing well with improved vision since the operation. This report highlights our experience with a teratoma in a very unusual location, and we review the relevant literature. Intracranial germ cell tumors of the central nervous system are rare and usually occur in the midline of the brain, particularly at the pineal region and neurohypophysis , 2. WhilTeratomas comprise only 0.5% of all intracranial tumors . One stuThe patient is a 57-year-old man who presented in July 2009 with decreased vision bilaterally, left sided facial weakness, ataxia, and short-term memory loss. The patient had also presented previously with a history of seizures, most recently a few months prior to the current presentation. A craniopharyngioma or another parasellar mass was suspected, and an ophthalmology consult was obtained.MRI showed a large intrasellar mass with considerable suprasellar extension and chiasmatic compression with the displacement of the inferior frontal lobe. There was also hydrocephalus with gross enlargement of the lateral and third ventricles . A hormoThe patient was determined to be an excellent candidate for surgery, the only other comorbidity being glaucoma treated with Lumigon. Because of the location of the tumor, it was decided that the patient would undergo a right pterional craniotomy/transsylvian approach for the resection of the tumor and the placement of a ventriculostomy catheter. After placement of a ventriculostomy catheter, the major part of the craniotomy followed. The first line of incision was made in a curvilinear fashion that began at the area 1 centimeter anterior to the tragus of the ear near the root of the zygoma. It curved superiorly up around the temporal bone and parietal bone and then extended toward the midline frontal bone. Dissection proceeded to expose the appropriate amount of cranial anatomy. The tumor was eventually exposed by opening up the sylvian fissure to separate the frontal lobe from the temporal lobe. The tumor was noted to have both cystic and solid components. Fluid aspirated from the cystic component was thick and yellow, with the appearance of oil or cholesterol. There were odd appearing components to the tumor, including what appeared to be yellow clumps of hair embedded within fatty deposits. There were also several firm calcified structures that appeared to be compressing the left optic nerve. The optic nerve itself appeared to be blanched in color without a very good blood supply. There were several firm calcified deposits of tumor that were tightly adhered to the optic chiasm, right carotid artery, and cranial nerve III complex. Though these were tightly adhered and it was decided to be too dangerous to completely resect, the high quality of decompression performed made it so that it was not felt that complete removal of these structures was clinically indicated. Specimens taken during the operation were sent to pathology and revealed portions of skin with skin appendages, innumerable hairs, and keratinous debris . Fluid fUpon closure of the procedure, there appeared to be no complications, and postoperative condition was deemed stable. Due to incomplete resection of tumor and risk of recurrence, external beam radiotherapy and eventually stereotactic radiosurgery were used as well as postoperative adjuvant therapy. The patient had a seemingly uncomplicated subsequent recovery and was discharged home. Though over the next month he had a slowly progressive decline in mental status and was admitted for observation. He was discharged soon after following close monitoring and recovery of his status. Since then, he has been watched carefully with frequent clinic appointments and imaging studies. At the most recent followup, approximately 4 years later, he is doing well with significantly improved neurological status and vision.Mature teratomas are well-differentiated tumors that are usually lobulated and often firmly adhere to neighboring structures . They coIntracranial germ cell tumors are rare comprising about 0.3% to 3.4% of all intracranial tumors . The preTeratomas are a type of germ cell tumors containing tissue elements of each of the three germ cell layers: endoderm, mesoderm, and ectoderm , 12. TheClinical and radiographic findings can make it difficult to distinguish a mature teratoma from a craniopharyngioma . In thisThe typical treatment for mature teratomas is surgical resection, which was successfully done in this case. The deterioration of the patient after a period of improvement in mental status and ambulation may be due to hydrocephalus or due to chemical encephalitis from by-products of the tumor. Mature teratomas are benign and have been reported to have survival rates up to 93% at 10 years . HoweverSuprasellar mature teratomas are exceptionally rare. However, because they mimic many other lesions, it is important to recognize this entity in the differential diagnosis and to obtain a thorough histological diagnosis. A multimodal approach for treatment is recommended with complete surgical resection, radiotherapy, and chemotherapy. Future reports on this rare location for a teratoma can help ascertain long-term outcomes.


A pseudoaneurysm is a haematoma which is surrounded by connective tissue and communicates with the lumen of a ruptured blood vessel. It has no true defined capsule. We describe a case of tuberculous pseudoaneurysm. This is a rare complication of tuberculosis. The clinical presentation of these lesions is highly variable. Definitive diagnosis should consist of contrast-enhanced CT and arteriography. Treatment should include repair of the arterial wall by surgery, endovascular stent-graft insertion, or embolization followed by a full course of antituberculous chemotherapy. Our case is highly unusual in that the pseudoaneurysm arose from the subclavian vasculature in a patient with extrapulmonary tuberculosis only. A pseudoaneurysm is a haematoma which is surrounded by connective tissue and communicates with the lumen of a ruptured blood vessel. It has no true defined capsule. These lesions are a rare complication of tuberculosis. Nevertheless they must be recognised promptly as perforation or rupture may lead to haemodynamic shock and death. Previous case reports predominantly describe pseudoaneurysms of the thoracic or abdominal aorta occurring in patients with pulmonary tuberculosis. Our case is highly unusual in that it describes a pseudoaneurysm of the subclavian vasculature in a patient with extrapulmonary tuberculosis only. A forty-year-old gentleman was referred with a painful right supraclavicular mass. The mass was first noticed two months prior to presentation and had enlarged considerably over this period. There was associated weight loss and malaise. There was no history of fever, cough, or dyspnoea. The patient was a nonsmoker who drank alcohol occasionally. Physical examination revealed a mildly tender, firm, and mobile mass in the right supraclavicular fossa . No pulsChest radiograph and routine blood tests were normal. Mantoux test was positive at 19 mm. White caseous material was aspirated from the mass. Microscopy revealed caseating granulomatous lymphadenitis suggestive of tuberculosis (TB). No acid-fast bacilli were seen on smear and no organisms were grown from culture. Screening tests for HIV and Hepatitis B and C were negative. CT neck and thorax were performed to assess for other sites of lymphadenopathy; right upper and lower paratracheal and right hilar nodes were also found to be enlarged. The right supraclavicular mass measured 5.7 × 5.4 cm . This arThe patient was placed on isoniazid, rifampicin, ethambutol, and pyrazinamide. The patient received six months of antituberculous chemotherapy. Three months after treatment was completed, the patient was reassessed. He remained clinically well; there was no evidence of the mass on examination. CT neck was also performed at time of followup. This demonstrated complete resolution of the supraclavicular mass . Blood then leaks through the defect into the extravascular space where it is encapsulated by connective tissue and becomes organised. Pseudoaneurysms differ from true aneurysms which are defined as a localised dilatation of all layers of the arterial wall. Mycotic aneurysm secondary to TB is a rare event. In a study of 22,792 necropsies between 1902 and 1955, Parkhurst reported only a single tuberculous aneurysm . In receA literature search revealed only two previous reports of tuberculous aneurysms involving the subclavian artery; one of these aneurysms appeared to arise from miliary TB  while thRecognition of tuberculous aneurysms remains difficult because of their rarity. This is compounded by the diverse clinical presentation of these aneurysms. They may present as an enlarging mass that is clinically palpable or visible on radiology. Patients may also describe pain at the site of the aneurysm which may be accompanied by fever. Rupture or perforation of the aneurysm may lead to major haemorrhage and haemodynamic shock . BleedinIt must be borne in mind that tuberculous aneurysms can occur in patients without a prior diagnosis of TB. Long et al. reported that 38% of patients with tuberculous aneurysms of the aorta did not have a diagnosis of TB at the time of presentation . A tuberAlthough unavailable at the time our patient was assessed, we strongly recommend ultrasound for the initial investigation of suspected tuberculous lymphadenopathy; ultrasound facilitates prompt examination at the bed-side, provides visual guidance during aspiration and may also identify abnormal vascular structures such as pseudoaneurysms. Once pseudoaneurysm is suspected, contrast-enhanced CT should be the investigation of choice , 6, 9. MTreatment of tuberculous aneurysm must include both repair of the vessel wall and antituberculous chemotherapy. Repair of the vessel wall can be performed by surgery , 6, endoIn summary, we report a case of a forty-year-old gentleman with tuberculous lymphadenitis with pseudoaneurysm formation. Pseudoaneurysm is a rare complication of TB. Its clinical presentation is variable making diagnosis difficult. It is recommended that contrast-enhanced CT is the investigation of choice. This should be followed by angiography. Treatment should include repair of the arterial wall by surgery, endovascular stent-graft insertion, or embolization as well as a full course of antituberculous chemotherapy. A high index of suspicion is required to diagnose these aneurysms; if unrecognised, they may rupture, perforate, or fistulate with possibly fatal consequences.


Unprovoked superficial thrombophlebitis and subsequent venous thromboembolism are well-described albeit rare presenting features of advanced visceral malignancy that often manifest too late for curative intervention to be beneficial.We present the first reported case of early gastric carcinoma presenting with these paraneoplastic phenomena in an otherwise healthy farmer. The early presentation allowed for a curative partial gastrectomy, which itself was complicated by the presence of a deep vein thrombosis extending into the inferior vena cava. Fortunately, stabilization of the clot allowed for surgery to proceed without the need for a caval filter. The patient was referred for adjuvant chemotherapy and has since made an excellent recovery.This case provides new evidence for the presentation of superficial thrombophlebitis in early gastric carcinoma and the potential for curative surgery in such instances. A high index of suspicion and a prompt diagnostic workup are essential for timely planning and execution of surgery in these early albeit rare presentations. Unlike the well-established association of spontaneous venous thromboembolism (VTE) with occult neoplasia, single episodes of unprovoked superficial thrombophlebitis have been suggested not to be associated with malignancy . In contA 41-year-old farmer with no significant past medical history presented to Casualty with acute, severe pain over the medial aspect of his right leg and thigh. Examination revealed exquisitely tender cord-like thickening along the distribution of the long saphenous vein. The patient firmly denied external trauma but an infective cause of the inflammation was presumed 'until proven otherwise'.Intravenous penicillin and flucloxacillin were administered but the inflammation persisted for a further three days. During this time, routine blood tests revealed a microcytic anemia  of unclear etiology, suggesting the need for an esophagogastroduodenoscopy (EGD) to exclude upper gastrointestinal tract bleeding. Concurrently, acute swelling of his right calf prompted a duplex ultrasound scan (USS), which revealed a deep vein thrombosis (DVT) with vague suggestion of extension up to the iliac veins. The patient was therefore switched from his prophylactic regimen of low molecular weight heparin  to a therapeutic regimen .The EGD unexpectedly revealed an annular growth around the pylorus partially obstructing the gastric outlet. Biopsy at endoscopy revealed a primary gastric adenocarcinoma. A contrast CT scan demonstrated mucosal confinement of the tumour with no evidence of regional lymph node involvement or distant metastasis Figure . In addi2N0MX or Stage I disease). The patient was subsequently referred for adjuvant chemotherapy and at 6-month follow-up, displayed no signs or symptoms of recurrent disease.In anticipation of surgery, anticoagulation was maintained with therapeutic-dose LMWH. A vascular surgical consult advised against the placement of an IVC filter as the relatively prolonged confinement of the DVT and therapeutic heparinization made further extension or dislodgement unlikely. A partial gastrectomy was performed with added precaution taken during dissection around the IVC to avoid thrombus dislodgement. Histology of the resected specimen confirmed a diffuse-type adenocarcinoma with marked acute and chronic inflammation but no lymph node involvement , this is nonetheless the first reported case of early gastric cancer presenting with this phenomenon and subsequently amenable to curative surgery. The other significant feature of our case was the development of DVT, which is similarly unusual in the early stages of malignancy , and occThe propagation of DVT to the IVC initially posed a challenge to the surgical management of our patient's gastric carcinoma, as it was unclear whether the risks of deploying a caval filter were warranted prior to major surgery. A recent prospective study by Birkmeyer et al involving 6376 patients revealed that prophylactic IVC filters for gastric surgery did not reduce the risk of pulmonary embolism (PE) but resulted in filter-related complications such as filter migration (e.g. into the heart or pulmonary arteries), insertion-site thrombosis and IVC thrombosis . NeverthWe were reminded that proximal propagation of stable DVTs after two weeks was highly unlikely, thereby negating the risks of filter insertion. The evidence supporting this includes a study by MacDonald et al, in which 91% of all DVT propagation occurred within 2 weeks of initial diagnosis, leading to the authors' suggestion that follow-up after 2 weeks may be unnecessary . SimilarThe stability of our patient's thrombus is very likely attributable to the full therapeutic heparinization administered upon DVT diagnosis, the efficacy of which has been proven on many occasions. A study of DVT outcomes by Gillet et al showed no VTE extension or recurrence within the therapeutic anticoagulation period of 1 month, and also within the subsequent 2 months . SchwarzIn conclusion, this case provides new evidence for the presentation of superficial thrombophlebitis in early gastric carcinoma and the potential for curative surgery in such instances. A high index of suspicion and a prompt diagnostic workup are essential for timely planning and execution of surgery in these early albeit rare presentations. For resectable malignancy associated with DVT extending up to the IVC, we support a conservative approach (i.e. therapeutic heparinization) until surgery, provided that surveillance can demonstrate adequate clot stabilization over two weeks. Invariably, if DVT occurs on a suspected background of malignancy, therapeutic-dose heparin should be used as the sole means of anticoagulation until the need for surgery is excluded (or surgery is performed), before warfarin is commenced.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 declare that they have no competing interests.MIMdZ and DNS were responsible for delivering patient care. SGT and AHMP contributed equally towards to drafting of the manuscript while MIMdZ and DNS provided overall supervision and edited the final version of the manuscript. All authors have read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2482/11/1/prepub


Dear Sir,Teratomas are germ cell tumors. Mediastinum is the second most common extra-gonadal site of these tumors.[1-3] Mediastinal masses in children and adolescents are usually considered malignant. Even benign teratomas have been reported to transform to malignant ones in case of delayed diagnosis or presentation.[3-5] Moreover, large mediastinal teratoma without any long-term symptomatology is quite rare. We describe here a large anterior mediastinal mature teratoma diagnosed in a paucisymptomatic adolescent.A 15-years-old girl was admitted for a two-days-long history of low-grade fever associated with acute-onset of cough and mild thoracic pain. Pain was referred to the left hemithorax and increased with breathing and movements. Vital signs were normal, with a room-air oxygen saturation level of 99%. Physical examination was unremarkable, particularly with normal breath sounds at chest auscultation. Unexpectedly chest x-ray revealed a large homogeneous mediastinal paracardiac mass in the left hemithorax . Echocardiogram showed normal cardiac function with mild pericardial effusion. Contrast enhanced computed tomography (CT) scans confirmed a well-demarcated mediastinal partially cystic mass of about 11x9x8 cm with regular borders, completely enclosing the thymus and containing soft tissue, fat and a small calcification . The mass mildly compressed the adjacent structures. There were no radiological signs of invasion into the surrounding mediastinal structures. These radiological findings were consistent with the diagnosis of cystic teratoma. Biochemical evaluation showed lymphocytosis with neutrophilia  and increased ESR (67 mm/1sth). Alpha-fetoprotein and beta human chorionic gonadotropin were within normal limits. At left postero-lateral thoracotomy, the mass occupied the anterior mediastinum and focally infiltrated pulmonary parenchyma of the inferior lobe of the left lung. The mass and the infiltrated part of the inferior lobe of the left lung were totally excised. Histopathological analysis confirmed the diagnosis of mature cystic teratoma without immature elements. Chemotherapy was not administered. After a one-year of clinical, biochemical and radiological follow-up no recurrence of disease was evident. Long term follow-up is in plan.Up to 80% of pediatric mediastinal teratomas are benign, with 20% revealing immature elements by histological evaluation. Naturally occurring malignant transformation of mediastinal teratoma is rare, but long-standing, mature teratomas may be at risk of malignant change during childhood and adolescence . In a recent review Paliwal et al suggested that mature mediastinal teratomas had increased risk of malignant transformation in adenocarcinoma when the tumor was at least 10 cm in size or in presence of areas of thickening.[4] Takahashi et al. reported a case of an anterior mediastinal teratoma with malignant transformation in embryonal rhabdomyosarcoma in a 12-year-old-boy.[5] Dimensions and margins of mediastinal teratomas and the possible presence of areas of thickening should always be assessed to suspect malignant transformation.Mediastinal teratomas produce non-specific symptoms  when they attain large size or may rupture into the lung and bronchial tree, pleural space, pericardial space, or erode great vessels.. In our case, the patient reported with acute-onset of febrile illness with unremarkable past history and physical examination. The referred mild thoracic pain induced us to perform chest x-ray allowing the detection of a large mediastinal mass. Chest x-ray is usually able to identify mediastinal masses up to 90% of cases.[7] However, CT is the modality of choice to establish the nature of the mass (solid or cystic), the location and its relationship to the adjacent structures.[2]Complete surgical resection is the treatment of choice for mediastinal teratomas followed by an extensive histological examination.[8] Postoperative chemotherapy is required in malignant tumors.[1] However, long-term clinical, biochemical and radiological surveillance is necessary.[8] In conclusion, mediastinal teratomas are rare and usually benign, but long term follow-up is required to detect recurrences.Source of Support: NilConflict of Interest: None declared


The occurrence of retroperitoneal schwannoma is uncommon and its presence may only be expressed by insidious onset of nonspecific symptoms such as vague abdominal pain. Imaging modalities like computed tomography and magnetic resonance imaging may demonstrate the tumor, but due to heterogeneity and degeneration in some tumors, it may mimic malignancy. So, fine needle aspiration cytology followed by tissue sampling through needle biopsies may be essential to confirm the diagnosis prior to the surgery. This case is reported for its rare clinical presentation, having duration of more than 40 years with cystic degenerative changes. Retroperitoneal schwannomas are benign tumors arising from Schwann cells of the peripheral nerve sheath. Benign sWe report a case of right retroperitoneal schwannoma diagnosed by guided FNAC in an elderly man who presented with right flank pain and slowly growing abdominal mass for a period of 40 years.A 60-year-old male presented with history of vague abdominal pain since 6 years and mass per abdomen since 40 years. Pain was on and off and was felt around the umbilical and epigastric region, increasing after taking food, and was non-radiating. Mass was seen to occupy the right lumbar, right iliac fossa, epigastric and umbilical region with a firm consistency. The mass was regressing in size since 2 years. There was history of loss of weight and appetite since 4 months. He was a known alcoholic and smoker for 20 years.Ultrasound scan showed a well-defined capsulated mixed echogenic retroperitoneal mass with areas of necrosis and calcification seen extending from the right paravertebral umbilical region. CT scan showed a large well-defined peripherally enhancing retroperitoneal hypodense mass with areas of peripheral calcifications noted in the right lower abdomen, causing mass effect suggestive of retroperitoneal schwannoma.Ultrasound guided FNAC of the retroperitoneal mass was performed using a disposable 10-ml syringe and 22- gauge disposable lumbar puncture (LP) needle; at multiple sites in the mass, maintaining negative pressure. FNA smears were stained with hematoxylin and eosin (H and E), Papanicolaou stain (Pap) and May-Grünwald-Geimsa (MGG) stain.Moderately cellular smear showed a few sheets of polygonal cells with round, uniform nuclei and vacuolated cytoplasm (cyst macrophages) Figure  and b alThe patient was taken up for surgery, and pre-operatively, the mass was identified in the right loin area, attached to right abdominal wall via mesentry. Ureter was identified medial to the mass. Posteriorly, mass was attached to the transverse process of the underlying vertebra, inferior venacava and to right common iliac artery. Debulking of the mass was done and sent for histopathology.Gross examination revealed two thick membranous soft tissue masses measuring 11×7×1.5 cm and 10×7×0.5 cm. External surface appeared smooth. Cut surface appeared ragged showing multiple nodules, the largest measuring 3×2.5×1 cm . Cut secHistopathological examination revealed schwannoma surrounded by thick fibrocollagenous wall, showing collection of foamy histiocytes and areas of hyalinization . VerocayImmunohistochemical staining for S-100 protein strongly highlighted the spindle cells . There wThe final diagnosis was retroperitoneal schwannoma with cystic degeneration.Schwannomas or neurilemmomas or neurinomas are benign, slow growing, solitary and well-encapsulated lesions that arise from the Schwann cells. Extracranial schwannomas present as solitary mass anywhere in the body. The common sites include the head and neck, the flexor surfaces of the upper and lower extremities, the posterior mediastinum in the thorax and on the trunk. Cases inEarly symptoms of retroperitoneal tumors are vague and none can be considered diagnostic. The common symptoms include vague, poorly localised pain and discomfort. Atypical presentations are very rare and include flank pain, haematuria, headache, secondary hypertension and recurrent renal colic pain.7 CT and 8et al[et al.[Dahl et al describeal[et al. Grossly,al[et al.7Histologically, schwannomas may demonstrate biphasic pattern with compact areas of high cellularity (Antoni type A) and loose, hypocellular myxoid areas with microcystic spaces (Antoni type B). Cystic changes occur more commonly in retroperitoneal schwannomas (upto 66%) than in other retroperitoneal tumors. Other degenerative changes such as calcification, hemorrhage and hyalinization reported by other authors were also seen in our case. OccasionHeterogeneity caused by cystic degeneration designates the tumor as “ancient” schwannoma. Degeneration is due to central tumor necrosis as the schwannoma grows to a size beyond the capacity of its blood supply. CalcificWide surgical resection is advocated in cases of benign retroperitoneal schwannoma. Care must be taken in attempting removal of retroperitoneal and intrapelvic schwannomas. There ar


Intraosseous lipoma is very rare, usually benign tumor of flat bones. However, the localization in skull bones is described in sporadic cases. The differential diagnosis includes end stage of infection, infarct lesions, intraosseous meningioma, angiolipoma, or myxofibrous tumors. We report a patient with intraosseous lipoma located in the sphenoid bone. The diagnosis was established due to the characteristic radiological features. According to the history of seizures, the lesion was removed via endoscopic endonasal approach. Histopathological examination showed adipocytes. The patient underwent control neuroimaging studies. Intraosseous lipoma (IL) is a rarely diagnosed, usually benign tumor placed in the flat bones, hardly ever in the long bones. Most often reported localizations include calcaneus, rib, and frontal and basal skull –3. LipomA patient, 23-year-old woman, has been referred to neurological department by an ophthalmologist with suspected papilledema. Two weeks earlier, she suffered from the first generalized tonic-clonic seizure after unexpected sudden pain. Medical history includes 2 attacks of unconsciousness after acute pain. A CT scan revealed a hypodense mass with central calcifications within sphenoid sinus and clivus. Density of the hypodense portion of mass ranged from −30 to −90 Hounsfield units suggesting presence of fat which surrounded central, scattered calcifications. Multilobulated margins of the lesion were sharp and sclerotic suggesting nonaggressive growth . MR scanClinical neurological examination was normal except for a bilateral Babinski sign. All biochemical tests were normal, as well as EEG pattern. Patient has not been treated with antiepileptic drugs due to the first seizure and finally unrecognized cause of the epileptic event; besides that, reflex epilepsy was taken into account. Cerebrospinal fluid (CSF) examination revealed increased level of protein (59 mg%). Other components of the CSF were normal. MRI of the brain and angio-MRI were normal. Cerebral intravenous thrombosis was excluded. A month later, she was admitted to the otolaryngology department and an intraosseous mass was removed via endoscopic endonasal approach. Histological examination revealed only adipocytes . Three mThe presented case with radiological and clinical picture resembles cases described by MacFarlane et al. and Lanišnik et al. , 8. AccoRecently, noninvasive followup has been proposed in case of the intraosseous lipoma due to low possibility of transforming into malignant forms, a relatively precise diagnosis based on neuroimaging tests and sometimes spontaneous involution , 13–15. Our patient underwent invasive procedure because of the additional neurological symptoms as seizure and elevated CSF protein level. However, the relation of these symptoms to lipoma seems to be coincidental.


Inflammatory myofibroblastic tumor is a rare type of lesion that mimics malignancy and has various clinical manifestations. The current study presents a 36-year-old female with a colonic mass, which closely resembled a stromal tumor during imaging. The patient experienced intermittent fever and slight abdominal pain for one month. The fever remained at ≤38.5°C until the day of surgery. The patient underwent a right hemicolectomy and the preoperative fever disappeared and did not recur until the patient was discharged. Inflammatory myofibroblastic tumor (IMT), also known as an inflammatory pseudotumor, is an uncommon mesenchymal neoplasm of intermediate malignant potential. IMT usually presents in children and young adults and has been found to occur in numerous locations, including the lungs, mesentery, omentum, soft tissues of the head and neck, retroperitoneum, liver and urinary bladder ,2,3. HowMycobacterium tuberculosis. The body temperature remained abnormal following antibiotic medications, including cephalosporin and penicillin, which were administered at the First Affiliated Hospital of Nanchang University  where the patient was initially admitted. The patient had no history of serious illness, surgery or hospitalization.A 36-year-old female was hospitalized at the Department of Infection  due to fever of unknown origin. The patient experienced an intermittent fever of ≤38.5°C, which was preceded by feeling cold, however, this was not accompanied by marked shivering or sweating; in addition, the patient experienced slight right abdominal pain for one month. Non-steroidal anti-inflammatory agents, such as indomethacin, alleviated the fever. However, the body temperature of the patient gradually increased again several hours later. The tuberculin skin test was negative for 9/litre; neutrophil count, 68%; and platelet count, 358×109/litre). The erythrocyte sedimentation rate and C-reactive protein levels were elevated to 120 mm/h and 159 mg/l, respectively. Electrolytes, urinalyses and renal function tests were normal, however, liver function was abnormal , as a result of long-term use of antibiotics. In addition, the patient’s antistreptolysin O and anti-Jo-1 antibodies were identified as positive. Serum markers for hepatitis B and C, anti-dsDNA, antinuclear antibodies and antineutrophil cytoplasmic antibodies were all negative. No pathogens, including parasites, were observed in the stool samples.On admission to the Huashan Hospital, no abnormalities were detected on physical examination and a laboratory evaluation revealed almost normal blood routine test results  scan, which localized the tumor to the ascending colon and was considered to be a stromal tumor. In addition, a positron emission tomography/CT scan showed an exogenous tumor at the ascending colon (close to the hepatic flexure), which was associated with the increased metabolism of fluorodeoxyglucose . The radThe fever remained between 38.5 and 39°C up to and including the day of surgery. The intraoperative examination revealed an irregularly margined, round and rubbery mass, which appeared to arise from the ascending colon (close to the hepatic flexure). The mass was tightly adhered to the side of the peritoneum, transverse colon and a section of the great omentum. A right hemicolectomy was performed to include the region of the tumor.Gross examination of the primary tumor revealed an ill-defined, smooth, glistening mass that infiltrated the colonic wall . The excised tumor measured 6.5×4.5×4.0 cm, and the cut surface of the primary tumor showed a white/gray lobulated mass and sections of the surface exhibited a cross-hatched appearance. Histologically, the tumor was characterized by fibrosis and proliferation of the spindle cells. In addition, polymorphous inflammatory infiltration of the plasma cells, histiocytes and lymphocytes with fibroblasts were noted . The patFurthermore, the gene test showed no mutations in exons 9, 11, 13 or 17 of the c-KIT gene, or exons 12 or 18 of the platelet-derived growth factor receptor A gene. The results of the gene test combined with the negative immune reactivity of DOG-1 and CD117 excluded the diagnosis of a gastrointestinal stromal tumor. Leiomyoma and schwannoma were also excluded, due to the unclear positive immunoreactivity for desmin and S-100.The preoperative fever subsided and did not recur until the patient was discharged. The patient was considered to be healthy without evidence of recurrence during the one year following surgery.IMT is a clinical and pathological disease entity . It is aet al (Previously, Coffin et al  describeet al , which iThe majority of extrapulmonary IMTs, including colonic IMTs, are successfully curable by surgical resection without the development of recurrence ,6. The pIn conclusion, IMT is an extremely rare lesion that mimics malignancy and is accompanied by various clinical manifestations; however, they are often benign lesions and are surgically curable. Therefore, an IMT must be considered as the possible cause for an abdominal mass that is accompanied by various clinical manifestations. Thus, all patients must undergo a careful long-term follow-up due to the unknown risk of recurrence.


Primary adenocarcinomas resembling submucosal tumors are rare in the gastrointestinal tract. Almost all the submucosal tumor-like adenocarcinomas previously reported invaded the submucosa or deeper. Therefore, submucosal tumor-like lesions are usually treated by surgical resection, and those that arise in the duodenum have been treated by pancreaticoduodenectomy.A 65-year-old Japanese man was diagnosed with a submucosal tumor-like adenocarcinoma in his duodenum. We considered it possible that the tumor invasion was limited to the mucosal or submucosal layers and could be removed by endoscopic resection. Tumor histopathology revealed a well-differentiated adenocarcinoma confined to the muscularis mucosae with no lymphovascular invasion. Complete resection of the carcinoma was achieved and there has been no recurrence three years after endoscopic resection.We suggest that submucosal tumor-like adenocarcinomas arising in nonampullary duodenal sites should be diagnosed carefully with a view to possible endoscopic resection. Adenocarcinomas are epithelial-derived tumors while submucosal tumors (SMTs) originate from nonepithelial cells. Hence, there have been few reports of adenocarcinoma lesions resembling SMTs in the digestive tract. Previous reports have described SMT-like adenocarcinomas in the stomach and colon -5. HowevWe report a rare case of duodenal adenocarcinoma resembling a SMT, which was resected completely, not by pancreaticoduodenectomy as originally scheduled, but by endoscopic resection (ER).A 65-year-old Japanese man, with no symptoms and receiving only oral medication for hypertension, underwent an esophagogastroduodenoscopy for a medical checkup in November 2009. A polypoid lesion, about 10mm in diameter with a deep depression on top, was found on the opposite side of the Vater papilla in the second portion of his duodenum (Figure Conventional endoscopy prior to surgery showed a polypoid lesion resembling a SMT with a central depression Figure B. ChromoHistopathology of the resected lesion showed a tumor with a central depression and bilateral elevations Figure A. The biThe prognosis for duodenal adenocarcinoma is reported to be a five-year survival rate of less than 30% . Thus, tThis case of early duodenal carcinoma showed characteristic features of a SMT on endoscopy. Histopathology demonstrated an inverted growth of cancerous glands with the tumor confined to the muscularis mucosae. We searched reports in English using the key words ‘early carcinoma’, ‘duodenum’, and ‘submucosal tumor-like’ and were unable to find any reports on early duodenal adenocarcinoma resembling SMT. However there were several reports of SMT-like cancer in the stomach and colon, but no reports describing SMT-like adenocarcinomas being completely removed by ER.According to previous reports of SMT-like cancer in the colon, the deep central depression in these tumors seems to be related to an inverted growth of cancerous glands into the muscularis mucosae, giving the endoscopic appearance of SMT. The histological features of these tumors are similar to an inverted hyperplastic polyp . Repeatede novo cancer and invade the submucosa, breaking the muscularis mucosae at a very early phase of tumor development. Our case seems to be very rare because the SMT-like tumor was detected before breaking the muscularis mucosae.Adenocarcinomas resembling SMTs located in the duodenum and colon have the same endoscopic characteristics of precipitously raised lesions with central depressions. A previous report in 2005 reviewed 24 cases of colorectal adenocarcinoma resembling SMT -5, and wMucosal cancers are good candidates for ER because there is no risk of lymph node metastasis . TherefoWe performed ER as a total biopsy. If histology from the ER had revealed the tumor was confined to the mucosal layer and the horizontal margin was positive, we would have planned an additional ER or endoscopic coagulation therapy. Otherwise, if histology had shown that the tumor had submucosal invasion or lymphovascular invasion, or the vertical margin was positive, we would have planned a partial resection or pancreaticoduodenectomy with lymphadenectomy.Nakajima and colleagues reported that cancerous glands were evident in the depressed portion of the lesion in most of the SMT-like carcinomas . When diThere has been no recurrence in this patient in the three years since ER. We succeeded in completely removing the SMT-like duodenal cancer, which appeared to be invading the submucosa or deeper, in a minimally invasive manner by means of ER rather than by performing a pancreaticoduodenectomy. There is a vast difference in quality of life after treatment between ER and pancreaticoduodenectomy. We suggest that SMT-like cancer in the duodenum should be carefully diagnosed, with a view to performing ER, if possible.Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.ER, endoscopic resection; EUS, endoscopic ultrasonography; NBI, narrow-band imaging; SMT, submucosal tumor; SR, surgical resection.The authors declare that they have no competing interests.HI is a doctor in private practice and he introduced the patient to the Department of Surgery at our hospital. KY is a professor in the Department of Surgery who arranged esophagogastroduodenoscopy for preoperative assessment. AD, KG, NY, KS, HT and SS have made substantial contributions to the acquisition and interpretation of clinical data. KG has been involved in drafting the manuscript or revising it critically for important intellectual content. MI provided and revised the pathological data. TK and HT have given final approval for the version to be published. All authors have read and approved the final manuscript.


A-20-year old male, with no significant medical history, presented with clinical features mimicking a perforated acute appendicitis. Because of features of peritonitis, a laparotomy was performed which showed a segment of small bowel with multiple large diverticula and mesenteric cysts. A segmental small bowel resection was performed. The patient made an uneventful recovery from surgery. Histology revealed features of a small bowel hamartoma. The rarity of this lesion made the histological diagnosis a debatable issue. Histologically, the unusual appearance and the presence of the mixed vascular, lymphatic, smooth muscular, and adipose tissue components in the identified lesion meets the principles implied in the concept of hamartoma. Hence, we present a rare case report of small intestine (ileum) hamartoma.A 20-year-old male was admitted via the Emergency Department on the Acute General Surgical Intake with a 24-hour history of right iliac fossa pain, in association with vomiting. His only medical history was of acne vulgaris for which he received Lymecycline 408 mg OD. He had experienced similar abdominal pain for a seven-day period, one month earlier. At that time, laboratory investigations and plain radiology were unremarkable. The patient was offered laparoscopy and appendicectomy, declined intervention, and was self-discharged against medical advice as he felt improvement. Examination findings on the readmission comprised a fever (temperature 39.6°C), pulse rate of 93 beats per minute, a respiratory rate of 19/minute, oxygen saturation of 96% on air, and normal Glasgow Coma Score. Palpation of the abdomen revealed diffuse tenderness throughout, with generalised guarding with no abdominal distension, and bowel sounds were absent.Laboratory investigations showed haemoglobin of 16.7 g/dL, white cell count of 13.6, normal renal function, and normal C-reactive protein. A plain abdominal radiograph was normal. In view of the degree of tenderness, a contrast-enhanced CT scan of the abdomen and pelvis was obtained. This revealed what looks like a 50 cm multiseptated collection, occupying much of the abdominal and pelvic cavities. The collection contained pockets of air. There was a small quantity of free fluid around the liver and in both paracolic gutters. The appendix was not visualised.Intravenous fluids, broad spectrum antibacterials, and proton pump inhibitors were administered. The patient was catheterised and taken for emergency exploratory laparotomy.At laparotomy, a 20 cm abnormal segment of small intestine (ileum) was identified; this gave a false appearance of 50 cm collection on the CT scan images Figures  and 2 thInterpretation of the resection specimen histology proved challenging. Macroscopically, the resected 25 cm segment of small bowel contained two thin-walled cysts located on the mesenteric border, one 15 by 14 cm and the other 11 by 1 cm. There was evidence of perforation and a purulent serosal reaction. On sectioning, both lesions showed “honeycombing” with numerous cystic loculi, with their size ranging between 5 and 20 mm. The small bowel mucosa had a granular, thickened, and ulcerated appearance.Microscopically, the cystic (diverticula) lesions were characterised by numerous mural and subserosal dilated vascular spaces, lined by endothelium surrounded by a layer of smooth muscle fibers. Some of the spaces contained proteinaceous lymph-like material, and others contained blood. The small intestine showed focal ulceration. The lamina propria were expanded with numerous small lymphatic channels. There was evidence of perforation and an acute suppurative serositis. There was no evidence of granulomata or malignancy.The differential diagnosis lay between cystic lymphangioma, angiomyolipoma, and hamartoma. The consensus opinion was that the lesion was a hamartoma.The patient was followed up in clinic six months postoperatively with a repeat normal CT scan abdomen and pelvis and was discharged without any further followup.Hamartoma is very rare benign condition associated with an abnormal location and arrangement of tissues normally found in small intestine . DiagnosIn 1940, Clark, used the term myoepithelial hamartoma (MEH) to describe the submucosal masses of epithelial glandular cells and smooth muscles were found in eight cases . MyoepitNakayama et al. present a case of Cowden hamartomatous syndrome complaining of GIT bleeding and melena. It was identified as having multiple GIT hamartomas with arteriovenous malformation as a source of the bleeding, and the histopathology of the resected jejunal and ileal specimen showed the presence of adipose tissue and ectatic lymphatic vessels .To our knowledge, we present the first case of small bowel hamartoma in young male manifested with lower abdominal pain, and CT scan showed massively dilated small bowel mimicking large intra-abdominal collection . IntraopSmall intestine (ileum) hamartoma is a rare benign entity, but like other small bowel tumours, it presents with features of obstruction and is managed by small bowel resection.


Malignant fibrous histiocytoma (MFH) is an aggressive spindle cell cancer of soft-tissue sarcoma type in the elderly, mostly affecting the extremities. Lesions > 5 cm, positive margins, and local recurrence are significant poor prognostic indicators. The strongest predictor for distant metastasis was tumor size (> 5 cm), and for overall survival, presence of local recurrence. Limb-sparing extensive tumor resection is preferred to achieve negative surgical margins. However, in some circumstances, amputation is inevitable. Recent studies demonstrated that adjuvant radiotherapy for microscopically positive surgical margins significantly improved local control and disease-free survival rates. Therefore, effective therapeutic strategies against locally relapsed high grade MFH are required to prevent distant metastasis and to achieve long-term disease-free survival. Here, we report local relapse of high grade MFH treated by successive application of autologous formalin-fixed tumor vaccination (AFTV) with limb-sparing surgery and postoperative radiotherapy. The patient is alive and well, disease-free and with no functional impairment, more than five years after treatment. Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma. When located in a limb, MFH is currently treated with limb-sparing surgery followed by > 65 Gy external beam radiotherapy ,2. For p3 and a CRP of 0.35 mg/dl. We removed the tumor with 1.5 cm horizontal margins, preserving the fascia lata. DM was treated orally. Pathological examination revealed that the mass was a malignant fibrous histiocytoma (MFH) composed of strongly proliferating fibroblastic and histiocytic tumor cells with bizarre nuclei, abundant mitotic activity, a pleomorphic pattern and necrotic areas. These findings were identical in the primary extirpated tumor (7.5 cm) reported in the meantime by the previous clinic  at the location of a scar on the right thigh, where a mass 7.5 cm in diameter had been resected at a different clinic three months earlier. No pathological information was available at the time, but because of the painful infected lesion, we promptly removed the tumor, which had cloudy exudates at the ulcerative surface. The patient had suffered > 5 years from poorly controlled diabetes mellitus (DM) with blood sugar at 272 mg/dl and an elevated HbA1C of 9.4%, but no ketone bodies in the urine. Other laboratory tests revealed signs of slight inflammation, including a WBC of 6,540/mm.5 cm at Combining limb-sparing surgery and postoperative radiotherapy with > 65 Gy is standard treatment for patients with high grade large MFH . TreatmeWritten 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.The authors declare that they have no competing interests.TK carried out postoperative wound care. SS participated in the postoperative radiotherapy. MT participated in the pathological studies. KM participated in the radiological imaging studies. TO participated in preparing AFTV treatment design and coordination. All authors read and approved the final manuscript.Table1. Immune Parameters 2 wk after completion of AFTV.Click here for file


We report the case of a 2-year girl who presented, in 2007, lameness with swelling of her right knee. Her leg has been putted in plaster but pain remained intense one month after. Blood tests did not show any inflammatory syndrome and standards radiographies were normal. Synovial fluid ponction and synovial biopsy were performed and confirmed a non specific synovitis. These results led to evocate a juvenile oligoarthritis despite the absence of antinuclear antibodies and uveitis. Treatment with Non Steroid Anti-Inflammatory Drugs (NSAIDs) allowed remission of symptoms.Symptoms reappeared 2 years later with swelling of the right knee associated with a localized tumefaction of the external part of the patella. Standard radiographies showed a hypertrophic patella due to abnormal growth. New complete investigations invalidated the diagnosis of oligoarthritis. Right knee echography showed synovitis in the sub-quadricipital recessus and a mass with several tortuous lesions which evocated 	a hemangioma in Hoffa’s fat. Another ultrasound confirmed that this hemangioma was at the expense of the lateral chief of quadriceps.Currently, this young girl is treated by NSAIDs and an interventional treatment by sclerosis first and then by surgery is discussed but the age of this patient leads to delay these treatments.Vascular malformations are divided into various subcategories depending on the predominant anomalous channels: venous, lymphatic, capillary and arterial. Prevalence of hemangiomas is estimated at 1.5% in general population.Clinical symptoms  depend on their localisation  and their local extension. The main localisations of hemangiomas are: pelvis, limbs and cranial circulation.Venous malformation are the most frequent (2/3 cases). They can be localized, as in our patient, multifocal or diffuse.The best imageries to diagnose and describe hemangiomas are MRI and ultrasound with colour Doppler. In MRI, the aspect of the lesion is in isosignal T1 and hypersignal T2. Ultrasound allows the localisation of the mass and the visualisation of tortuous lesions evocating hemangioma.Medical treatment is often insufficient and needs to be completed with interventional procedures: sclerotherapy for diffuse lesion or surgery for localized mass. Combination of these two techniques is, in some cases, necessary.Prognosis is largely favorable without sequellae.Recurrent swelling of the knee is not necessarily due to idiopathic juvenile arthritis but can be due to other local diagnosis as hemangioma. In atypical symptoms or evolution, ultrasound and/or MRI is helpful to rectify the diagnosis.


Parachordoma is an infrequent neoplasm that bears some histologic resemblance to chordoma. It affects both sexes, occurs typically during the fourth decade of life, and tends to present as a slow-growing painless mass at the level of the soft tissues of the extremities. Diagnosis should be based on immunohistochemical and cytogenetic studies, as the findings of imaging techniques are often unspecific. Although it is considered a benign lesion, its behavior tends to be locally aggressive, with reports of a recurrence rate of up to 20% and of several cases of metastasis. Fewer than 60 cases have been published in the English-speaking literature. In this paper we present the case of a 32-year-old male with a two-year history of parachordoma in the right wrist. Parachordoma is an infrequent benign neoplasm of uncertain origin. It is morphologically reminiscent of chordoma, but its location is extra axial. It affects both males and females of different ages and usually presents as a slow-growing mass at the level of the soft tissues of the extremities.Fewer than 60 cases have been published in the English-speaking literature. The purpose of this study is to present a new case of parachordoma, laying special emphasis on the importance of a judicious differential diagnosis. A review of the literature on the subject is also presented.The patient was a 32-year-old male with no relevant medical history who presented to our department in December 2009 with a two-year history of a growing neoplasm in the dorsum of the wrist. The lesion had developed slowly and was not related to a traumatic event. The patient did not mention any recent episodes of fever, weakness of weight loss. Our initial examination revealed a firm neoplasm on the dorsal radial metaphysis fixed to the deep planes but not to the skin. There was thus no skin involvement or local redness. Pain was elicited on palpation and on finger flexion, but no pain was present at rest. The patient submitted an earlier computed tomography (CT) scan that showed a lytic lesion of 1.5 cm in diameter and well-defined margins affecting the dorsal cortex of the distal radius . The imaIn the face of these findings, the differential diagnosis included a wide variety of entities including benign conditions ranging from a fibrous cortical defect or a mixed tumor with a stromal component (myoepithelioma) to a soft tissue sarcoma or an osseous sarcoma with an associated soft tissue component .An ultrasound-guided percutaneous biopsy was performed, which revealed a neoplastic proliferation comprising nidi and cords of eosinophilic cytoplasmic cells separated by dense fibrous stroma . In othePrior to surgery, a thoracic-abdominal-pelvic CT scan was conducted to rule out the presence of additional lesions. During the operation, an en bloc resection of the soft tissue neoplasm was performed as well as an excision of the associated bone lesion, which required a semicircular transverse osteotomy on the dorsal aspect of the distal radius. The bone defect was filled with a strut allograft from the iliac crest that was shaped to measure and stabilized with two 3.5 mm screws Figures . The hisThree years after the procedure, the patient is asymptomatic with wrist range of motion of 110° for flexion-extension, 160° for pronosupination, a 60° radial/ulnar tilt arc; the pain score is 0 on the visual analog scale. Radiographs show complete graft incorporation Figures  with no Parachordoma is an extremely rare soft tissue tumor first reported by Laskowski in 1955 and revisited by Dabska in 1977 .The most common location of parachordoma is the soft tissues of the extremities (78%), followed by the chest, the trunk, and the pelvis . ClinicaThe CT study shows a well-defined homogeneous, or slightly heterogeneous soft tissue neoplasm that may present with calcifications. The destruction of adjacent osseous structures seen in our case is highly unusual; only two similar cases have been reported in the literature , 4. On MThe most crucial factor about parachordoma is its differential diagnosis. Conditions such as extraskeletal myxoid chondrosarcoma, epithelioid sarcoma, and metastatic clear cell carcinoma must be ruled out. Nevertheless, the main controversy lies in whether parachordoma can or cannot be distinguished from peripheral chordoma. Both neoplasms share four cell types grouped in the form of nidi and cords: epithelioid cells, small glomoid cells, spindle cells, and cells with a vacuolated cytoplasm  . AlthougParachordoma has traditionally been considered a benign lesion whose treatment of choice is surgical resection. However, the recurrence rate has been found to be up to 20% by different studies, with follow-up periods ranging from three months to 12 years , 8, 10. It should also be noted that seven of the cases published to date developed metastasis: in three cases the tumor invaded the lungs, in two it involved regional lymph nodes, and two patients had generalized metastasis. For that reason we considered it necessary to follow up these patients using the usual protocol for soft tissue sarcoma, subjecting them to periodic imaging studies.


Pulmonary tumor thrombotic microangiopathy is a rare complication of malignant diseases. The diagnosis is extremely difficult and is most often performed after death. Invariably, patients develop acute pulmonary hypertension causing right heart failure, shortness of breath and death in a few days. We describe the clinical and radiological findings of a patient who presented with this complication.A 28-year-old Caucasian woman with a previous history of pelvic tumor resection two months previously, suggestive of metastatic adenocarcinoma, presented with intense shortness of breath. A computed tomography scan showed signs of acute cor pulmonale and diffuse nodular opacities associated with a tree-in-bud pattern disseminated through her lungs, suggestive of bronchiolitis. Our patient's condition worsened and she underwent a surgical biopsy. Pathologic analysis of the biopsied specimens revealed pulmonary tumor thrombotic microangiopathy. Our patient's tumor evolved from a gastric origin (Krukenberg tumor). She underwent progressive clinical deterioration and died less than 24 hours after the biopsy. None of the cases described previously in the literature had diffuse centrilobular nodular opacities associated with a tree-in-bud pattern disseminated through the lungs, as in our case.Pulmonary tumor thrombotic microangiopathy should be considered in cancer patients with rapidly progressing dyspnea, chest computed tomography findings compatible with pulmonary hypertension and typical findings of inflammatory bronchiolitis. Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare complication of malignant diseases. The diagnosis is extremely difficult and is most often performed after death . InvariaA 28-year-old Caucasian woman presented to our hospital with a previous history of abnormal vaginal bleeding and abdominal pain that had begun four months prior to her initial presentation. A transvaginal ultrasound was performed at that time and demonstrated solid lesions in her left adnexal region, confirmed two weeks later with magnetic resonance imaging. She then underwent an exploratory laparotomy with a bilateral anexectomia. A pathological examination diagnosed a solid poorly differentiated adenocarcinoma, with signet ring cells diffusely infiltrating the ovaries and fallopian tubes bilaterally, compatible with a metastatic pelvic tumor of probable gastric origin, traditionally known as a Krukenberg tumor. After surgery, during the staging and treatment planning, our patient presented to our Emergency Room complaining of dry cough and shortness of breath of recent onset, with intense and progressive worsening. She had no other complaints and no fever. On examination, she was in generally poor condition, malnourished, dehydrated, with tachycardia and dyspnea; pulmonary auscultation revealed decreased breath sounds and mild wheezing in both lungs. Her leukocyte count showed no signs of infection; a blood test revealed the presence of mild anemia and a chest X-ray showed no abnormalities. Due to clinical suspicion of a pulmonary thromboembolism, our patient underwent a computed tomography (CT) scan of her chest, pelvis and thighs. Although the angiographic phase did not present any signs of pulmonary thromboembolism, the CT scan showed signs of acute cor pulmonale characterized by increased right heart chamber dimensions, slight interventricular septum deviation and a right ventricular to left ventricular ratio greater than 1.5 Figures , 4 and 5Laboratory investigations were remarkable for microcytic anemia  and a strongly positive D dimer (1100 ng/mL). Her arterial blood gas examination breathing room air showed hypoxia with respiratory alkalosis . Our patient was admitted with a presumptive diagnosis of pulmonary thromboembolic disease.PTTM is a rare complication of malignancies commonly detected after death. It was first described by von Herbay and colleagues as a diffuse intimal myofibroblast proliferation that causes pulmonary hypertension, leading to cardiopulmonary failure and death . This diDistinguishing between the clinical presentation of lymphatic and microvascular disease can be challenging, and many authors do not distinguish between these two. Soares and colleagues evaluated a series of 222 patients who had undergone autopsy, including 19 with microvascular pulmonary arterial embolism and 44 with lymphangitic carcinomatosis . AlthougOn histopathological investigation, pulmonary tumor thrombotic microangiopathy is characterized by widespread fibrocellular intimal hyperplasia of small pulmonary arteries, also called carcinomatous endarteritis induced by tumor microemboli . DiffuseNone of the cases described previously in the literature had diffuse centrilobular nodular opacities associated with a tree-in-bud pattern disseminated through the lungs, as in our case.PTTM should be considered in cancer patients with a rapidly progressing dyspnea, chest CT compatible with pulmonary hypertension and typical findings of inflammatory bronchiolitis.Written informed consent was obtained from our patient's next of kin 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 declare that they have no competing interests.All the authors contributed to the writing of the paper. The original manuscript was written by MFA and AB, and the final version by MDG. RC and JLG contributed the CT images and additional references. All authors read and approved the final manuscript


An 11-year-old girl presented with progressively increasing swelling in scalp of 8-month duration with no neurological deficit. Local examination showed a hard swelling that seemed to be arising from frontal bone. General and systemic examination was normal. MRI revealed a well-defined lytic lesion in left frontoparietal bone with a subgaleal component. The patient was operated upon and excision of tumor with reconstruction of skull was done. Histopathological examination showed a monomorphic small round cell tumor of bone infiltrating into the subcutaneous tissue. Immunohistochemical stain showed diffuse immunopositivity for MIC-2 in tumor cells, thus final diagnosis of Ewing's sarcoma was made. The patient was kept for follow up for 3 months and had no symptoms. Ewing's sarcoma is most commonly seen in children and young adults with a peak incidence in the second decade of life. It most commonly arises in long bones of the extremities (predominantly femur) and pelvis . PrimaryAn 11-year-old girl presented with painless swelling in left frontoparietal region of scalp from 8 months and progressively increasing in size. The mass was detected incidentally when she had a fall. There was no history of headache, vomiting, fever, and seizure or any focal neurological deficit. Local examination revealed a hard mass of 50 × 48 mm in size with normal overlying skin. She has no sensory and motor deficit. Her general and systemic examination was normal. Blood investigations were normal. CT scan shows a well-defined lytic lesion in left frontoparietal bone. On MRI, a lytic lesion in left frontoparietal calvarium was seen with sun ray periosteal reaction, measuring 60 × 53 mm in size with a subgaleal and epidural component causing bulking of parenchyma . The patThe tumor was sent for histopathological examination. Grossly, the specimen consists of one large grayish brown soft tissue attached to a flat bony fragment measuring 7.0 × 7.0 × 4.5 cms. External surface of the soft tissue was smooth and partially encapsulated. Cut surface of the soft tissue was gray white to yellowish gelatinous and showed few hemorrhagic areas also . On micrEwing's sarcoma involving the skull is rare and occurs in less than 1% of cases . CommoneOn plain X-ray many patients may show areas of bone destruction as in our case, with irregular poorly defined margin. The most common CT finding is isodense lesion with marked heterogenous enhancement. Biopsy is essential for definitive diagnosis. In our case, the histological diagnosis is made by examining the morphology and immunohistochemical profile of tumor cells .The main differential diagnosis of tumor involving the skull with adjacent soft tissue extension in children would include metastatic neuroblastoma, PNET, chordoma, and lymphoma. Less common differential diagnosis includes rhabdomyosarcoma, osteosarcoma, meningioma, Langerhan's cell histiocytosis, desmoplastic small round cell tumor, plasmacytoma, and solitary metastasis. The differentiation between these may not be possible on light microscopy and require special stains and immunohistochemistry for final diagnosis. Although cytoplasmic glycogen content was considered to be important in differential diagnosis, in specimen fixed in formalin and embedded in paraffin, the glycogen may not be always demonstrated with PAS staining as in our case .19, CD20, and CD2,5,8. Chordoma express strong positivity for Pan CK and Epithelial membrane antigen [Primitive neuroectodermal tumor expresses neuronal marker such as synaptophysin, neurofilament protein, nonspecific enolase or S-100. Lymphoma cells express CD antigen .Immunopositivity for MIC-2 confirmed the diagnosis in our case. Early diagnosis and treatment prior to metastasis is essential for long-term survival in patient with Ewing sarcoma. The disease is treated through multidisciplinary approach that includes surgery, chemotherapy, and radiotherapy. This patient was kept for follow up and had no symptoms. In conclusion, primary cranial Ewing's sarcoma is to be considered in the differential diagnosis in children with a tumor involving the skull with destruction of bone and the presence of extra axial soft tissue involvement. Primary Ewing's sarcoma is reported to have a better prognosis as compared to Ewing's sarcoma elsewhere.