Primitive neuroectodermal tumor (PNET) of the kidney is a rare and aggressive tumor, often presenting in advanced stages and progressing rapidly. Renal PNET (rPNET) may affect a wide age spectrum, but the majority of cases are in young adults. We present a case of advanced rPNET in a 78-year-old woman. To our knowledge, this is the most advanced age of presentation of this neoplasm to date. We report on her presentation and management, and discuss the current clinical management of this tumor.


Preoperative localization of small pulmonary nodules is essential for precise resection, besides, the optimal treatment for pulmonary nodules is controversial and the prognosis without surgery is uncertain.Herein we present a patient with compromised pulmonary function harboring synchronous triple ground-glass nodules located separately in different pulmonary lobes.The pathological diagnosis of the nodules were chronic inflammation, inflammatory pseudotumor and atypical adenomatous hyperplasia, respectively.The patient underwent single-stage, non-intubated thoracoscopic pulmonary wedge resection after computed tomography-guided coil labeling of the nodules.The postoperative recovery was encouragingly fast without obvious complications.Non-intubated thoracoscopic pulmonary wedge resection is feasible for patients with compromised lung function, meanwhile, preoperative coil labeling of small nodules is reliable. The differentiation of synchronous primary tumors and intrapulmonary metastasis could be difficult, and minimally invasive surgery might be a proper choice for this dilemma.Pulmonary ground-glass nodules (GGNs) are a common incidental finding on computed tomography (CT), Curative intent shows benefits for patients with synchronous multiple primary lung cancers, as timely resection allows accurate diagnosis. Therefore, if resection can be performed, an aggressive approach is often warranted, and single-stage bilateral resection of synchronous multiple nodules is reported to be feasible and safe.It is reported that 9.5% of the pulmonary nodules are at a low risk, 79.6% are at a moderate risk, and 10.8% are at a high risk of malignancy, and a substantial fraction of intermediate-sized nodules turn out to be lung cancer ultimately. Furthermore, precise resection of deeply situated small pulmonary nodules (<15 mm) is challenging. CT-guided percutaneous biopsy of small nodules is often difficult, along with the risks of pneumothorax and hemoptysis. In our institute, localization of small nodules by CT-guided hookwire insertion before surgery is replaced by coil labeling, because the hookwire is not reliable for all cases.Moreover, preoperative tissue diagnosis does not significantly influence the clinical outcomes of patients with persistent malignant-looking GGNs after surgical resection.Herein, a patient with synchronous triple pulmonary nodules located in different lobes is presented for discussion, as he underwent single-stage resection of the nodules assisted with coil labeling.2A 57-year-old male patient was admitted to thoracic surgery department on April 17, 2016 for asymptomatic, newly diagnosed bilateral synchronous pulmonary nodules during health examination. These nodules were located in the left lower lobe, the right middle and lower lobe measuring 5 to 7 mm in diameter, separately, without obviously enlarged mediastinal lymph nodes, as depicted by CT scan in Fig. 1) = 1.8 L, FEV1% = 65%, and forced vital capacity (FVC) = 3.2 L]. The cranial magnetic resonance, abdominal CT, and bone emission computed tomography of the patient excluded other suspicious lesions. Besides, the score of Eastern Cooperative Oncology Group Performance Status of the patient was 1. Positron emission tomography as a reasonable choice for staging was not performed, because it was not covered by health insurance and expensive.The patient suffered from mild to moderate dyspnea and cough during the winter for nearly 10 years. He had a smoking history of at least 40 pack-years, meanwhile, he had been working as a coal miner for 7 years before admission. His family and social histories were unremarkable. Thorough physical examination failed to identify any other suspicious lesions. Routine tests for hepatic, renal, and coagulation function were normal. Hepatitis B and C, and human immunodeficiency virus tests were negative. In addition, the serum tumor markers including carcinoembryonic antigen, cytokeratin 19 fragment, squamous cell carcinoma, neuron-specific enolase, alpha fetal protein, and carbohydrate antigen  were all in normal range. Cardiopulmonary function tests indicated moderate compromised pulmonary function [forced expiratory volume , under local anesthesia, using 2% xylocaine  before surgery  artificial pneumothorax. The intrathoracic pressure was maintained at 10 to 12 mm Hg. In addition, devices for tracheal intubation and mechanical ventilation were also readily prepared for this patient.Secondly, the anesthesia protocol for nonintubated surgery was underwent as reported, using a combination of target-controlled sedation, intercostal nerve block by 0.5% bupivacaine and intrathoracic vagal block.The patient was positioned in the left lateral decubitus position for right-sided pulmonary wedge resection. An incision was made through the 5th intercostal space along the anterior axillary line. Rib spreading and retractor were avoided. A 10-mm video telescope was inserted for exploration of the pleural cavity. The incision was extended to 5 cm because of severe pleural adhesions, which was released carefully. The coils were easily found along the visceral pleura without dislocation or translocation. Lung tissue containing the nodule and the coil was lifted close to the incision by oval forceps to facilitate finger touch, and the nodules could be easily felt as protrusions. Then pulmonary wedge resection and mediastinal lymph node sampling including 4th, 6th, 8th, and 9th stations were performed using endoscopic stapling and harmonic scalpel. Then a 24-French soft chest tube was placed through the incision. After that, the patient was turned to the right lateral decubitus position for wedge resection of the left lower lobe.2, PaCO2, and central venous pressure were stable throughout the operation. The nodules in right middle and right lower lobe were turned out to be chronic pulmonary inflammation .The patient returned to normal activities 6 hours after surgery, without hemoptysis, dyspnea, arrhythmia, or vomiting, except for mild chest stuffiness and chest pain. The recovery of this patient was mainly uneventful, and he discharged 5 days after the operation. Subsequently, he was followed up remotely by internet-based approaches to deliver cardiopulmonary exercise education for 6 months up to now, who demonstrated satisfactory quality of life. Moreover, his pulmonary function was not significantly changed 3 months after the operation , the nodules are more likely to be invasive adenocarcinomas. Besides, the risk factors of malignancy include nodule size>20 mm, age >60 years old, prior cancer history, current tobacco use, asbestos exposure, and speculated nodules. And invasive adenocarcinomas are diagnosed in approximately 1% of all pulmonary subsolid nodules. In addition, major resected GGNs are positive for epidermal growth factor receptor (EGFR), Kirsten rat sarcoma viral oncogene homolog (KRAS), anaplasticlymphoma kinase (ALK), or human epidermal growth factor receptor 2 (HER2) mutations, and EGFR mutation-positive tumors are correlated with minimally invasive adenocarcinoma or invasive adenocarcinoma and growth, compared with EGFR-negative tumors.Firstly, the advent of CT screening for lung cancer would increase the incidence of pulmonary GGNs, but the screening is associated with a high frequency of false-positive results, because the high prevalent indeterminate are usually benign. It is reported that the frequency of microscopic invasions, disease-free survival and recurrence between stage I pulmonary adenocarcinomas appearing as solid nodules and part-solid GGNs is similar. Additionally, selection of surveillance, biopsy, and resection for evaluation of pulmonary nodules can be complex, and patients’ preferences may not be consistently taken into account during the decision-making process, for absence of high-quality evidence regarding the optimal management. Therefore, a more systematic approach is necessary to ensure correct diagnosis and optimal management.Secondly, the natural course of persistent pulmonary GGNs with solid portions ≤5 mm differs significantly according to their nodule type and diameters. Nonintubated surgery is considered to minimize the side effects of double-lumen endotracheal intubation such as ventilation-induced lung injury and postoperative vomiting, with less hospital stay and decreased inflammatory cytokines, compared with endotracheal intubation under general anesthesia, and it is well tolerated by very elderly patients. In addition, thoracic epidural anesthesia in high-risk patients could avoid ventilator dependency, followed by fast recovery, which could be an alternative of conventional anesthesia for severely compromised patients, nevertheless, it is not suitable for patients with hemodynamic instability and severe pleural adhesions.Thirdly, the optimal therapy for GGNs remains controversial, because patients of this kind would take the risk of delayed diagnosis, for this reason, minimally invasive resection of the lesions might be reasonable to diminish risks of metastasis, however, the treatment depends on the cardiopulmonary reserve, and preference of the patients, comparing the benefits with potential harm of surgery. Surgical treatment is thought to be the most effective strategy for multiple GGNs, and single-stage bilateral resection in selected cases with synchronous bilateral multiple nodules is feasible. However, wedge resection should be carefully considered for patients with mixed GGO nodules because of the high recurrence rate, and segmentectomy is superior to wedge resection for tumors less than 20 mm in diameter.On the other hand, whether patients would benefit from limited resection is uncertain. The standard treatment of early lung cancer is minimally invasive lobectomy with systematic lymph node dissection, however, recent research has shown that some ground-glass opacity (GGO) lesions may be treated with sublobar resections. CT-guided patent blue vital dye localization is more accurate, and it could be utilized for synchronous multiple nodules. It is hard to decide the proper margin of deeply located pulmonary nodules during the operation, thus, preoperative simulation using 3-dimensional CT and rapid prototyping of pulmonary vessels could be considered. The case presented herein shows satisfactory life quality during internet-based follow up, in accordance with the essential of fast track thoracic surgery.Thirdly, there are several methods for localization of small nodules, including CT-guided patent blue vital dye injection, methylene blue dye injection, hookwire placement, coil labeling, lipiodol marking, and radiotracer placement. The methylene blue dye is not very reliable for its quick diffuse from the target site. Hookwire may cause tearing of the lung parenchyma with hemorrhage risk, and fail to localization if pneumothorax develops. Besides, invasive sampling of low-risk nodules and surgical resection of benign nodules remain common, suggesting a lack of adherence to guidelines for pulmonary nodules.It is noteworthy that indeterminate pulmonary nodules are commonly encountered and often result in costly and invasive procedures that eventually turn out to be unnecessary.4In summary, the feasibility of single-stage nonintubated thoracoscopic pulmonary wedge resection for multiple coil-labeled GGNs is tentatively demonstrated from this case. However, more high-quality studies concerning standard of GGNs treatment and nonintubated thoracic surgery are required.


We report the case of a Japanese woman with amyloid light chain (AL) amyloidoma in the abdominal aortic retroperitoneum and mesentery. Irregular soft tissue mass lesions with calcification in the abdominal aortic retroperitoneum and mesentery were initially detected by computed tomography at another hospital. The lesions gradually compressed the duodenum, causing symptoms of bowel obstruction. The patient was clinically diagnosed with retroperitoneal fibrosis, and prednisolone was administered at a dose of 40 mg/day. However, the lesions did not change in size and her symptoms continued. She was transferred to our hospital and underwent mesenteric biopsy for histopathology using abdominal laparotomy. The histopathological and immunohistological findings of the mesenteric specimen demonstrated lambda light chain deposition. Accordingly, the patient was finally diagnosed with AL amyloidoma with no evidence of systemic amyloidosis. After laparotomy, her general condition worsened because of complications of pneumonia and deep vein thrombosis. She died suddenly from acute myocardial infarction. We have concluded that abdominal aortic retroperitoneal and mesenteric AL amyloidoma may have very poor prognoses in accordance with previous reports. In addition, the size and location of AL amyloidoma directly influence the prognosis. We suggest that early histopathology is important for improving prognosis. Amyloidosis is a disease that occurs as a consequence of abnormal insoluble protein (amyloid) deposits in the organs and/or tissues . AmyloidReactive AA amyloidosis is characterized by deposition, predominantly in the gastrointestinal tract and kidneys, of metabolite amyloid A originating from serum amyloid A. Serum amyloid A is produced by the liver and promoted by interleukin- (IL-) 6 in response to chronic inflammatory disorders like rheumatoid arthritis, tuberculosis, osteomyelitis, and so on. Therefore, inflammatory markers like serum C-reactive protein and serum amyloid A levels respond with a high sustained value. In the past, the 5-year survival rate was 50% [Amyloidoma  is a solitary localized tumor-like amyloid deposition defined as the absence of any evidence of systemic amyloidosis. In many cases, the development sites of amyloidoma are in the systemic organs such as the respiratory system , genital18F-fluorodeoxy glucose mildly accumulated only in the irregular soft tissue mass lesions . Based on the PET result, the soft tissue mass lesions were considered unlikely to be cancerous, and she was followed up by regular imaging evaluation.A 65-year-old Japanese woman was admitted to our hospital with a 1-month history of vomiting. She had undergone no medical examination for 10 years prior to admission. Nine months before admission, she was hospitalized at another hospital with dyspnea. She was diagnosed with acute heart failure and three-vessel coronary arteriosclerotic lesions by coronary angiography. She subsequently underwent coronary artery bypass grafting surgery. At that time, computed tomography (CT) revealed irregular soft tissue mass lesions with calcification in the abdominal aortic retroperitoneum and mesentery Figures . PositroOne month before admission to our hospital, the patient was readmitted to the previous hospital with persistent vomiting. Abdominal CT revealed enlargement of the abdominal aortic retroperitoneal and mesenteric soft tissue tumor masses. The masses were compressing her duodenum, leading to symptoms of bowel obstruction. She was clinically diagnosed with retroperitoneal fibrosis, and prednisolone was administered at a dose of 40 mg/day. After 3 weeks of treatment, however, the tumor masses did not reduce in size and her symptoms persisted.μL, hemoglobin level decreased to 9.5 g/dL, platelet count was normal 302,000/μL, and erythrocyte sedimentation rate elevated to 21 mm/h. Her serum total protein and albumin concentrations decreased to 5.4 g/dL and 3.1 g/dL, respectively. Her serum protein fraction revealed that her albumin level was decreased to 57.0%, α1-globulin increased to 5.0%, and α2-globulin (13.6%), β-globulin (8.7%), and γ-globulin (15.7%) were all normal. Her serum IgG  and IgG4 (10 mg/dL) were normal. Her serum IgA decreased to 68 mg/dL. Her serum concentrations of IgM (70 mg/dL), C-reactive protein (0.1 mg/dL), serum creatinine (81.3 μmol/L), calcium (2.32 mmol/L), glycosylated hemoglobin (5.7%), and total cholesterol (188 mg/dL) were normal. She was positive for antinuclear antibody with a titer of 1 : 40, and her serum soluble interleukin-2 receptor level was increased to 662 U/mL. Urinalysis was normal with no Bence Jones protein. Electrocardiography revealed no abnormalities including axis deviation, bundle branch block, or low voltage. In addition, there was no granular brightness in the ventricular wall on cardiac ultrasound. No osteolytic lesion and compression fracture of the skull, femoral neck, or thoracolumbar spine were observed by whole-body CT.She was transferred to our hospital at this point, and on admission she was afebrile (35.3°C), her body weight was 45.1 kg (no weight loss), and she had a performance status of 3. The palpebral conjunctiva appeared anemic, although we observed no marked hair loss, oral ulcers, tongue swelling, thyroid gland and lymph node enlargement, skin rash, arthritis, or Raynaud's phenomenon. The soft tissue masses were not palpable on the abdomen. In addition, there were no findings of syncope, dizziness on standing up, movement disorders, or sensory impairment. The results of laboratory examinations were as follows: white blood cell count increased to 14,750/After being transferred to our hospital, the patient continued to take prednisolone at a dose of 40 mg/day. However, the masses still did not reduce in their size and her symptoms were still persistent. Therefore, on the 40th day of hospitalization, we performed mesenteric biopsy for histopathology and decided to attempt gastrointestinal bypass surgery using abdominal laparotomy. The intraoperative findings showed that the mesentery at the root of the superior mesenteric artery was very hard and thick with white coloration and was compressing the third portion of the duodenum. In addition, two fibrotic masses approximately 1 cm in diameter were found in the mesenteric lesion . The fibThe result of a biopsy of the resected mesenteric lesion indicated poor cellular components and hyalinization and degeneration of tissue with a small amount of fat. No evidence of the infiltration of inflammatory or tumor cells was present . Congo rIn the present case, soft tissue masses occurred in the abdominal aortic retroperitoneum and mesentery and proved difficult to diagnose. We finally diagnosed AL lambda amyloidoma based on a mesenteric biopsy using abdominal laparotomy.Amyloidoma developing in soft tissues, the retroperitoneal and mesentery in particular, is quite rare. To the best of our knowledge, eight cases of retroperitoneal and mesenteric AL amyloidoma have been reported –13, inclIn the five patients who underwent chemotherapy or radiation therapy, partial remission was achieved in a short period. In one case, death occurred shortly after glucocorticoid monotherapy. These results show that retroperitoneal and mesenteric amyloidoma are partially affected by chemotherapy or radiation therapy but not glucocorticoid monotherapy. Our patient unexpectedly died of the incidence of acute myocardial infarction. However, she would have been able to live a little longer if she had been able to receive intensification therapy such as chemotherapy for amyloidoma. It is noteworthy that these reports demonstrated a median survival time of 11.8 months, with no evidence of survival for more than 2 years. Thus, all previous reports suggest that retroperitoneal and mesenteric amyloidoma are associated with extremely poor prognoses.A few reports have shown that AL amyloidoma did not recur for several years after surgical complete resection , 16. How18F-fluorodeoxy glucose mildly accumulated only in the abdominal aortic retroperitoneal and mesenteric lesions. Therefore, as there was no evidence of plasmacytoma in the biopsy of the resected mesenteric lesion, it was possible that the plasmacytoma in the intraperitoneal soft tissue lesions proliferated in the neoplastic stage and produced M protein locally. This M protein was then deposited in the abdominal aortic retroperitoneum and mesentery, causing the irregular soft tissue masses.In the present case, we suspected that plasmacytoma was the cause of AL amyloidoma because the electrophoresis of serum and urine proteins revealed the presence of M protein, which was indicative of IgG and Bence Jones protein lambda light chains. We also considered multiple myeloma as a potential underlying cause. However, the following points contradicted this hypothesis: (1) the patient's total serum protein level was slightly decreased; (2) her M protein level was only slightly increased; (3) although serum IgA was slightly below the normal range, it was less likely that our case had multiple myeloma because the serum level of IgM was within normal limits; (4) there was no evidence of organ damage such as osteolytic lesions and renal insufficiency, which were present in previous case reports of multiple myeloma associated with amyloidoma , 13; andIn conclusion, this is a first report of retroperitoneal and mesenteric AL amyloidoma, a very rare condition that shows poor prognosis. Of special importance is that the size and location of AL amyloidoma directly influence the prognosis. We suggest that early histopathology is essential for improving prognosis. In addition, once AL amyloidoma is diagnosed, complete surgical resection, chemotherapy, or radiation therapy should be considered promptly.


Collagenous fibroma, also known as desmoplastic fibroblastoma, is a rare benign slow growing tumor particularly uncommon in the oral cavity. The aim of this study was to analyze the clinical and histopathological features of an oral collagenous fibroma as well as to compare this data with those reported in an English-literature review. The thirteenth case of collagenous fibroma in the oral cavity and the first to present clinically as a bilateral mass was described. A 48-years-old female patient was referred to a School of Dentistry, complaining about an asymptomatic swelling on the hard palate, lasting around ten years. The intraoral examination revealed two well-defined mass, bilaterally in the hard palate. An excisional biopsy was performed. Microscopically, the connective tissue consisted of dense collagen bundles in which were seen scarcely distributed spindle-shaped to stellate fibroblastic cells. Blood vessels were few, as well as inflammatory cells. Immunohistochemical staining was positive for vimentin, α-smooth muscle actin and factor XIIIa and negative for S-100, CD68, CD34, HHF35, desmin and AE1/AE3. The patient remains disease-free 24 months after excision. In conclusion, oral collagenous fibroma should be included in the differential diagnosis of bilateral sessile nodules in the oral cavity. Key words:Connective tissue, mouth diseases, mouth neoplasms, oral diagnosis, oral pathology. Collagenous fibroma (CF), also known as desmoplastic fibroblastoma, is a rare benign slow growing tumor, first described by Evans in 1995 . CF normThe lesion, however, is particularly rare in the oral cavity -13. The The treatment of choice for oral CF is total surgical excision, and neither local recurrence nor metastasis have been reported . Only feA 48-years-old female patient was referred to the School of Dentistry, in August/2014, complaining about an asymptomatic swelling in the hard palate, lasting around 10 years. Medical, familial and dental histories were noncontributory. Intraoral examination revealed a well-defined submucosal sessile nodule, bilaterally, measuring each one 5.0 x 2.0 x 1.0 cm, with a firm consistency and covered by a healthy mucosa Fig. . The infLiterature ReviewA review of the literature was done considering retrospective English written studies and cases located in oral soft tissues. The search was conducted in the PubMed and Web of Science Databases, using the following keywords: “oral collagenous fibroma” and “oral desmoplastic fibroblastoma”. Twelve articles met the inclusion criteria.CF is a rare tumor in the oral cavity and, to date, no intraoral bilateral case of this lesion was reported ,8. ConsiClinically, oral CF appears as slow growing, asymptomatic and well-circumscribed mass ,5. The mMicroscopically, the lesion is characterized by stellate or spindle cells with a round nucleous, small nucleoli and delicate chromatin. Multinucleated fibroblasts are occasionally observed. The cells are embebed in a prominent collagenous background with inconspicuous blood vessels. Mitotic figures are rare or absent, and necrosis is not observed. The presence of high hyalinized collagenous, few cellularity and scarce inflammatory infiltrate are contributors for the diagnosis of CF ,13. The et al. (The scarce inflammatory infiltrate seen in our case exclude the hypothesis of trauma, ruling out the diagnosis of IFH and even TF, like affirmed by Gonzalez-Moles et al. . In the et al. ,5,7. It et al. ,5-13. SiThe immunohistochemistry can be helpful to the differential diagnosis with other soft tissue lesions and is an important tool to make exclusion diagnosis, since there are many tumours with similar characteristics ,13. The In this way, although the histologic pattern is not exclusive of CF, the association between clinical, histopathological and immunohistochemical features strongly supports the diagnosis of this lesion for the described case.The present work describes the thirteenth case of oral CF in English-language literature and the first to present clinically as a bilateral mass. This paper expands the spectrum of clinical manifestations of oral CF and demonstrates that this lesion should be included in the differential diagnosis of bilateral sessile nodules. Additionally, it is important to discuss the characteristics of this unusual pathology to better understand their clinical, pathological and immunohistochemical profile, helping clinicians and pathologists to conduct the correct diagnosis.


Sinonasal mucosal melanoma is a rare tumor that develops slowly and may manifest with non specific signs and symptoms, causing significant delay in diagnosis. Local recurrence is common and usually occurs within the first two years after the initial treatment. Prognosis of recurrent lesions is poor and 5-year survival ranges between 10-47%. We report the clinical, radiographic, histopathological and immunohistochemical findings of a recurrent sinonasal mucosal melanoma which was diagnosed 10 years after the initial treatment, in a patient who presented with unilateral facial swelling and one-sided difficulty in breathing of two years duration. We discuss the causes of late diagnosis and review the negative predictive factors for recurrence and survival. As early diagnosis is of paramount importance for prognosis, we emphasize the signs and symptoms of patients with a history of sinonasal mucosal melanoma which should raise the suspicion for recurrence, in spite of a long time interval since diagnosis. Key words: Mucosal melanoma, nasal cavity, sinus, recurrence. Sinonasal mucosal melanoma (SNMM) accounts for 0.3-2% of all primary malignant melanomas , approxiMost primary SNMMs 65.5-80.9%) arise within the nasal cavity .5-80.9% ,4,6,8,9.We present a recurrent mucosal melanoma diagnosed 10 years after the initial diagnosis, with unilateral facial swelling and difficulty in breathing of two years duration, and discuss the causes of the delay in diagnosis. Prognostic factors affecting the recurrence and survival rate are also reviewed.A 65-year-old female was referred by her dentist for evaluation and management of right facial swelling, with a provisional diagnosis of a “maxillary cyst”. According to the patient, the swelling was initially noticed approximately 2 years before presentation, grew slowly and was associated with unilateral difficulty in breathing. Her medical history was significant for a primary mucosal melanoma of the right nasal turbinate treated 10 years ago with surgical excision without adjuvant radiotherapy or chemotherapy. The patient did not attend a regular follow-up program. She did not take medications; she did not smoke and her recent complete blood count did not reveal any abnormality.Intraoral examination was within normal limits. A coronal Computer Tomography plane showed a solid, voluminous, hypointense lesion occupying the middle and inferior turbinate and the whole right sinus cavity, with thickening of the sinus mucosa and perforation of the sinus floor (red arrow) and the lateral nasal wall (yellow arrow) Fig. a. A promHistopathologic examination of 5μm thick formalin-fixed and paraffin-embedded tissue sections stained with hematoxylin and eosin showed a tumor composed of spindle and epithelioid cells arranged in sheets or solid nests Fig. a. NeoplaIn the case presented herein the indolent growth of a recurrent SNMM caused significant delay in diagnosis. Primary SNMM develops slowly ; its cliIn our case, diagnosis was also delayed by the underestimation of the patient’s history, possibly because of the long time interval between the initial diagnosis of the SNMM and its recurrence. Local recurrence is expected in 31%-85% of patients ,9 and thSeveral clinical, i.e. advanced age at initial diagnosis, isolated obstructive phenomena and atypical manifestations, including headache, facial pain and maxillary nerve anesthesia, anatomic, i.e. origin within the paranasal sinuses, and histopathological parameters, such as the presence of ≥10 mitoses per high power field, increased level of melanotic pigmentation, pseudopapillary or sarcomatoid architecture, perineural or vasolymphatic invasion, undifferentiated cell morphology, multiple, satellite formation and a positive margin status at initial surgery, have been associated with an increased risk for locoregional recurrence or/and a poor survival in SNMM ,4,6,12. Frequent and regular follow-up sessions for patients with a SNMM history, i.e. every 2-4 months, is recommended for at least the first decade post treatment , as earlIn conclusion, the non-specific clinical manifestations, such as facial edema and breathing discomfort, in a patient with a history of SNMM should raise the suspicion of recurrent tumor, in spite of a long time interval from initial diagnosis.


The management of pregnancy of unknown location (PUL) can be a challenging situation, since it can present as several different conditions. Here we describe a rare case of gestational choriocarcinoma arising in the fallopian tube after ovarian induction in an infertile patient. The patient received clomiphene for ovarian induction and had rising levels of human chorionic gonadotropin (hCG) over nine months without sign of pregnancy. After referral to our center, the patient was diagnosed with a paraovarian tumor, which revealed a gestational choriocarcinoma arising in the fallopian tube; the final diagnosis was supported by pathological and cytogenomic analysis. Malignancies, such as gestational trophoblastic disease, should be in the differential diagnosis of PUL; the early recognition of these conditions is key for the proper treatment and favorable outcome. Pregnancy of unknown location (PUL) is a condition that can be particularly challenging for clinicians due to the variety of diagnoses that PUL can represent . PUL canHere, we report a case of tubal choriocarcinoma that initially presented as PUL in an infertile patient after ovarian induction. The gestational origin of the tumor was confirmed via short tandem repeat (STR) analysis of samples from the tumor and serum samples from the patient and her partner.A 38-year-old nulliparous woman was referred to the University of Sao Paulo Trophoblastic Disease Center due to PUL with increasing hCG levels, amenorrhea for 9 months, and no sign of an hCG-producing site. She had a prior history of primary infertility for years and had received clomiphene for ovarian induction. Her hCG rose from an initial level of 2,845 mIU/mL to 3,917 mIU/mL after 2 days, 5,533 mIU/mL after two weeks, and 381,808 mIU/mL after 9 months, with serial normal ultrasound scans performed during follow-up at another institution.When the patient was referred to our institution, her hCG level was 267,836 mIU/mL, and ultrasound showed a normal uterus, a normal left ovary, a large cystic structure on the right ovary that measured 7.5 cm × 5.5 cm, and an irregular left paraovarian mass that measured 4.6 cm × 3.7 cm and exhibited intense low-resistance peripheral vascularization on Doppler examinations Figures . Pelvic An exploratory laparotomy was performed, resulting in visualization of a 5 cm vascularized left tubal mass, an 8 cm serous right ovarian cyst, and no other evidence of abdominal disease. Excision of the right ovarian cyst and the left uterine tube was performed. Pathological and immunohistochemical analyses revealed a choriocarcinoma infiltrating the tubal wall up to the serosa, the presence of vascular infiltration in tubal vessels, and a corpus luteum as the right ovarian cyst .Also, in order to clarify the origin of the tumor we performed the differential diagnosis by genotyping seven autosomal STR loci  and the sex-determining marker using AmpFLSTR® MiniFiler™ PCR Amplification Kit  according to manufacturer's instructions.Cytogenomic analysis showed the presence of paternal alleles in choriocarcinoma tissue, confirming the gestational origin of the tumor .The patient received 8 cycles of methotrexate, and her hCG levels normalized 4 months after surgery. The patient remains healthy 2 years after the completion of chemotherapy, with no signs of recurrence.PUL can be a challenging dilemma in medical practice, since several clinical entities can present with increased hCG levels and no visible sign of pregnancy , 2. EarlGestational trophoblastic disease (GTD) is a spectrum of disorders that arise from the placental trophoblast , 19. OneSince GTD is a rare condition, the relationship between ART and development of GTD has been debated in the literature. A retrospective report from United States of America disclosed a higher frequency of hydatidiform moles following ART (1 : 659 pregnancies) as compared to spontaneous pregnancies (estimated incidence 1 : 1000 pregnancies), even though it represents a rare complication , 24. TheART is a risk factor for developing extrauterine pregnancies; therefore, ART may potentially increase the risk for gestational choriocarcinoma arising in unusual locations . Other rhCG is a key tumor marker in the management of patients with GTD because its levels are correlated with disease burden , 19. In Choriocarcinoma, particularly when presenting in unusual locations, can be of gestational or nongestational origin. STR analysis is a useful tool for determining tumor origin, which can impact treatment modalities and outcomes for patients with this tumor –30. GestIn conclusion, differential diagnosis for PUL includes a variety of medical conditions. Early recognition of the hCG-producing source is key for the appropriate management of patients, particularly patients with neoplastic processes, which might be suspected based on extremely high and increasing levels of hCG combined with no signs of pregnancy.


In this study, an Inca bundle was examined using computed tomography (CT). The primary aim was to determine the preservation status of bony and soft tissues, the sex, the age at the time of death, possible indicators for disease or even the cause of death, as well as the kind of mummification. A secondary aim was to obtain a brief overview of the wrapping in order to gain additional information on the cultural background.The bundle belongs to the Museum of Cultures in Basel, Switzerland, and was bought in Munich, Germany, in 1921. Radiocarbon dating of the superficial textile yielded a calibrated age between 1480 and 1650 AD. The mummy was investigated using multi-slice CT with slice thickness of 0.75 mm and 110 kilovolt. For standardized assessment of soft tissue preservation, a recently developed checklist was applied.CT revealed the mummy of a seven to nine year old boy with superior preservation of bony and soft tissues allowing detailed assessment. Indicators of neurofibromatosis type 1 , Chagas disease , and lung infection , probably due to tuberculosis, were found. Furthermore, signs of peri-mortem violence  were detected. CT images revealed a carefully performed wrapping.CT examination of the Inca bundle proved to be an important non-destructive examination method. Standardized assessment, especially of the soft tissue structures, allowed for diagnoses of several diseases, indicating a multi-morbid child at the time of death. The careful wrapping pointed to a ceremonial burial. Within the cultural background, the signs of fatal violence were discussed as a possible result of war, murder, accident, or human sacrifice. Bioarchaeology  provides insight into the biological status, including diseases, of past people . PaleoraSoft tissues are the substances that make a skeleton into a mummy and they allow for a diagnosis beyond osteology . In contIn clinical radiology, structured reporting is increasingly being established. That means a diagnosis is made following templates or checklists –23. One As medical history and physical examination are not available for paleopathological studies, knowledge of the cultural environment, such as diet, typical diseases, general life circumstances or mummification and ritual practices, may provide additional information , 24. TheIn this study, CT was applied to an Inca mummy bundle from the Museum of Cultures in Basel, Switzerland. CT was believed to be the best non-destructive imaging method to gain information about the body, which was completely wrapped in thick layers of textile and therefore, was not accessible without unwanted destruction of the wrapping.The aim of this study was to determine the preservation status of bony and soft tissues, sex of the mummified individual, age at the time of death, possible indicators for disease or even the cause of death, as well as the kind of mummification. A secondary aim was to obtain a brief overview of the wrapping in order to gain any additional information on the cultural background of the bundle.The bundle belongs to the Museum of Cultures in Basel, Switzerland, where it is also housed. In 1921, the bundle was bought by Dr. Franz Xaver Weizinger (1883–1945) in Munich. He was a well-known German art historian and auction trader who had good contacts in South America. The provenance of the bundle before its disposal in Munich is still unknown.The cylindrical bundle had a maximum cross section of about 50 cm and a height of approximately 71 cm. On top of the body was a slightly conical false head. The body was dressed in a typical Inca tunic. Four small bags hung around the neck and a necklace with a spondylus shell was present. A slingshot was bound around the body and another smaller one around the neck. Three bags, made from of the fur of llama legs, strung around the waist completed the setting .Permission for the CT examination of the mummy (collection number IVc 2813) was granted by the Museum of Cultures in Basel, Switzerland.The mummy was investigated using a multi-slice CT  at the Institute of Legal Medicine, University of Basel, Switzerland. Additional reconstructions were performed at the CT workstations of the involved institutions, such as multiplanar reconstructions, maximum intensity projections, and three dimensional reconstructions.For the assessment of soft tissue preservation, a recently developed checklist  was applAge at the time of death of the mummy was determined based on tooth development and eruption , fusion The skeleton was completely preserved. The individual was in a squatting position with tightened legs and flexed knees. Both arms were located between the body and the legs. Some minor, presumably post-mortem, dislocations of skeletal elements were present. Otherwise, articulation and alignment were intact . Three mBased on the “CT Checklist and Scoring System”, soft tissue preservation revealed a total score of 137.5 (out of 200), consisting of 89 (out of 100) for the main category “Soft Tissues of Head and Musculoskeletal System” and 48.5 (out of 100) for the main category “Organs and Organ Systems”. Preserved soft tissues of the head included the nose, auricles, ossicles, eye bulb and/or lens, eye muscles, optic nerve, falx, and tentorium. Soft tissues of the musculoskeletal system included: tendons and/or musculature of different sites of the neck and trunk as well as the upper and lower extremities; peri- and intraarticular soft tissues such as the rotator cuff and the capsule and/or labrum of the shoulder, the capsule and/or labrum of the hips, and the posterior cruciate ligament and the medial and lateral meniscus of the knees. Additionally, intervertebral discs of the thoracic and lumbar spine were preserved. From the central nervous system, the brain was preserved in form of undefined remnants in the middle cranial fossa. The spinal cord was detectable in the cervical and thoracic spine. Peripheral nerves were detectable in the entire spine. From the cardiorespiratory system, slight remnants of the hypopharynx and larynx were preserved. The trachea and both lungs were preserved. The upper portion of the heart was discernible consisting of parts of the pericardium, all four chambers, the intraventricular septum, and the aortic valve. Parts of the diaphragm were visible on both sides. From the gastrointestinal system, the tongue, esophagus, stomach, intestine, rectum and/or anus, and the liver were preserved. From the genitourinary, system both kidneys and the penis were discernible. Arteries could be found in the neck and mediastinum.The determination of age at death was conducted by assessing tooth development and tooth eruption, as well as various skeletal markers. Several deciduous teeth were still in the jaw, however, the permanent incisors and permanent first molars had already erupted indicating an age between seven and eight years  28]. Fu. Fu28]. Skeletal abnormalities indicative of neurofibromatosis type 1 (NF 1) consisted of a slight sphenoid wing dysplasia on the left side , a moderSeveral soft tissue findings indicated the presence of NF 1. Paravertebrally at the cervico-thoracic junction, four small round masses (maximum diameter of 6 mm) with hypodense centers were detectable coming from the spinal nerve roots in the extraforaminal course . A lobulCT images revealed dilation of the esophagus in its entire course with a maximum diameter of 2 cm . The traBoth upper lobes of the lung showed only slight collapse and had broad contact to the pleura respective to the chest wall. The right middle, and lower lobe, as well as the left lower lobe were partially collapsed. On the right side, three small calcifications were detected within the parenchyma of the lung and another calcification with maximum diameter of 3 mm was found within the region of the right hilum .On the right side of the neck, an approximately 5 cm long curved discontinuity of the cutis without dehiscence was recognizable ranging from underneath the chin to the dorsal part of the neck .On the anterior chest, on the right side next to the sternum, a transection of the cutis and the underlying cartilage of the second to sixth rib were recognizable with different extents of dehiscence . The traFractures without dislocation and without callus formation were found in the left distal humerus and the left distal tibia.The mummy was seated on layers of hyperdense thick materials, which partially surrounded the feet and the lower part of the pelvis and was then fixed by cords. Several alternating layers of textiles were wrapped around the body with tightly interworked cord structures. Additional packing materials seemed to have been used resulting in multiple small hyperdensities . The wraCT examination of this child mummy allowed for age estimation at the time of death, detailed assessment of the preservation status, especially of the soft tissues, and diagnoses of diseases. Furthermore, distinct signs of violence led to speculations on the circumstances of the child’s death within its cultural environment.Pathologies found in the child mummy fulfill the criteria for the clinical diagnosis of neurofibromatosis type 1 (NF 1), also known as von Recklinghausen disease. NF 1 is a multisystemic neurocutaneous disorder and the most common phakomatosis . NF 1 afTo the best of our knowledge, this is the first definite diagnosis of NF 1 in paleopathological and paleoradiological literature based mainly on soft tissue findings in CT images. In paleopathological literature, few cases consider the diagnosis of NF 1 in skeletal remains –42. ProbThe dilation of the esophagus, stomach and colon  with massive amounts of feces indicated the diagnosis of chronic Chagas disease in this case. Chagas disease is a chronic, systemic, parasitic infection caused by the protozoan Trypanosoma cruzi and was discovered by the Brazilian physician, bacteriologist and epidemiologist Carlos Chagas in 1909 , 49. CurIn the paleopathological literature, Chagas disease is well known and documented by macroscopy, histology, immunhistochemistry, Trypanosoma cruzi DNA extraction, and radiology , 50–55. The differentiation between hollow organ dilation as a result of post-mortem bacterial growth with gas formation and intra-vital development of organ dilation has already been discussed. Distension of the rectum with feces and a colon packed with coprolites were considered a result of chronic Chagas disease, whereas dilatation without coprolites was suggested as a result of post-mortem gas production . In the Adherence of both upper lobes of the lungs to the chest wall as well as the small lung and hilum calcifications on the right side indicated lung infection, possibly due to tuberculosis. In mummies, adherence of the lung to the chest wall is supposedly the result of a past episode of pneumonia with healing , 56. IntUntil recently, it was thought that Europeans brought tuberculosis to America. Archaeological and paleopathological data succeeded in proving that the disease was already in America at least 2,000 years before the first contact from Europe –62. A reChildhood mortality was reported to have been high in populations from coastal Peru and Chile. In most samples, nearly 50% percent of children died before 15 years of age . StudiesCT images revealed different findings that have to be discussed within the context of violence.The cutaneous discontinuity on the right side of the neck was suggested to be the result of manipulation of the already mummified body, as the edges did not gape. However, a peri-mortem injury could not be definitely excluded.The incision on the chest, however, must have occurred during the peri-mortem or early post-mortem phase, as this region would no longer have been accessible in the sitting position after rigor mortis had set in. A similar kind of incision was described by Verano  in five The additional fractures of the left humerus and tibia and the dislocation of some teeth could have occurred in the peri- or postmortem course.In conclusion, the exact circumstances of the injuries, especially of the chest and abdomen, were not clear in this case. Hypotheses should include war, battle, murder, and accident . TraumatFrom the external view, the tunic was cut in two on the back, possibly indicating damage or manipulation. However, CT revealed an intact and very carefully performed inner wrapping of the mummy, comparable to the description of Inca bundles in the literature . The shoThe elaborate wrapping, including a spondylus shell and sandals, indicated a ceremonial burial of the child mummy . FurtherThe wrapping demonstrated the intention to preserve the child after bringing it into the desired position. However, any treatment of the body itself could not be found. The mummification seemed to be the result of dehydration due to the climate.Child sacrifice has been documented in the Andes of Peru and is most clearly associated with the Inca Empire , 69–71. CT examination of the Inca bundle proved to be an important non-destructive examination method. CT images revealed a male child mummy with superior bony and soft tissue preservation. Standardized assessment, especially of the soft tissue structures, allowed for the diagnosis of NF1, the quite certain diagnosis of Chagas disease and the diagnosis of lung infection, probably due to tuberculosis. Additionally, the multi-morbid child seemed to have been cachectic at time of dead. The careful wrapping pointed to a ceremonial burial. Furthermore, CT revealed signs of fatal peri-mortem violence. Within the cultural background, hypothesis on the reason of this violence included war, battle, murder, accident, and human sacrifice.


Primary internal hernias are extremely rare in adults. They are an important cause of small intestinal obstruction and lead to high morbidity and mortality if left untreated. Clinical presentation of internal hernia is nonspecific. Imaging has been of limited utility in cases of acute intestinal obstruction; moreover, interpretation of imaging features is operator dependant. Thus, internal hernias are usually detected at laparotomy and preoperative diagnosis in an emergency setting is either difficult or most of the time not suspected. We report herein a case of a 45-year-old male who presented with acute intestinal obstruction which was attributed later to a very rare type of internal hernia on exploratory laparotomy. A loop of ileum was found to enter the retroperitoneum through a hernia gate which was located lateral to the sigmoid colon in the left paracolic gutter. The segment of intestine was reduced and the hernial defect was closed. Our finding represents an extremely rare variant of retroperitoneal hernias. An internal hernia (IH) is a protrusion of intestines or other abdominal organs through a normal or abnormal orifice in the peritoneum or mesentery, occasionally leading to strangulation or incarceration. Internal hernias (IH) are a rare cause of acute abdomen and intestinal obstruction in adults. IH has a reported autopsy incidence of 0.2 to 0.9% and is the cause of small bowel obstruction in 0.6 to 5.8% of the cases . HoweverA 45-year-old male was admitted to the emergency department with complaints of acute onset severe abdominal pain and vomiting for three days. He was earlier evaluated and treated at a local hospital where he was kept on conservative management with insertion of nasogastric tube for 24 hours. But his symptoms were further aggravated with increased bouts of vomiting; as a result he was referred to our institute for further management. The patient had no history of similar attack in the past. He did not report any fever, dysuria, and change in bowel habit or hematochezia. There was no history of any comorbid illness, past surgical intervention, or trauma. On general examination, pulse rate was 98/minute and blood pressure was 130/80 mm of Hg. Patient was afebrile and tachypneic. Abdominal distension and tenderness were detected on abdominal examination. Bowel sounds were decreased. Digital rectal examination was normal. Rest systemic examination revealed no abnormality. Abdominal radiography in the upright position showed dilated small intestinal loops with multiple air-fluid levels suggesting intestinal obstruction. Ultrasonography of the abdomen revealed gaseous distension of the bowel loops. Routine blood investigations were within normal limit.The patient was planned for exploratory laparotomy in view of acute intestinal obstruction. On laparotomy, a loop of ileum was found to enter the retroperitoneum through a hernia gate (defect of 2.5 cm) located lateral to the sigmoid colon in the left paracolic gutter Figures  and 2. TInternal hernias are either congenital or acquired, the latter constituting the majority. Important causes of acquired internal herniation in adults are previous abdominal surgery (mainly liver transplantation and bariatric procedures like gastric bypass), trauma, peritoneal inflammation, or ischemic changes . PrimaryPatients may be asymptomatic or present with clinical symptoms of small bowel obstruction (SBO) as the most frequently herniated organ is the small bowel. Acute abdomen is seen with ischemia and late cases of perforation . SymptomComputed tomography (CT) now plays an important role in the evaluation of intestinal obstruction and acute abdomen . Both raTimely surgical intervention based on clinical suspicion and/or CT scan findings is warranted for the management of IH presenting with intestinal obstruction . ReductiInternal hernias are a rare but important cause of intestinal obstruction given the high mortality associated, nevertheless still often underdiagnosed. Primary internal hernias should be kept in the differential diagnosis of acute intestinal obstruction in adults with no previous history of surgery or trauma. Since physical examination findings are nonspecific, a high index of clinical suspicion along with urgent CT is suggested to aid in the preoperative diagnosis of IH. Early surgical intervention is crucial to avert the high risk of associated morbidity and mortality. While conducting emergency laparotomy for intestinal obstruction, the rare type of primary IH as seen in our case should be kept in mind.


Introduction. Esophageal perforation in the setting of a malignancy carries a high morbidity and mortality. We describe our management of such a patient using minimally invasive approach. Methods. An 83-year-old female presented with an iatrogenic esophageal perforation during the workup of dysphagia. She was referred for surgical evaluation immediately after the event which occurred in the endoscopy suite. Minimally invasive esophagectomy was chosen to provide definitive treatment for both her malignancy and esophageal perforation. Results. Following an uncomplicated operative course, she was eventually discharged to extended care for rehabilitation and remains alive four years after her resection. Conclusion. Although traditional open techniques are the accepted gold standard of treatment for esophageal perforation, minimally invasive esophagectomy plays an important role in experienced hands and may be offered to such patients. We present the case of an 83-year-old Caucasian female who presented to our institution with a one-month history of abdominal pain, dysphagia, and weight loss. She was admitted to the medical service for inpatient evaluation of her symptoms. Her medical history was significant for depression, lifelong blindness, chronic back pain, cholelithiasis, and a right-sided radical mastectomy.Upper GI endoscopy revealed an exophytic, ulcerating, obstructing mass at 30 cm from the incisors. The stricture was traversed with some difficulty and was noted to end at the gastroesophageal junction. At this point, attempt was made to pass a lubricated nasogastric tube beyond the stricture under direct vision. However it became apparent that there was now an extraluminal tract. Postprocedure computed tomography (CT) scan confirmed the suspected diagnosis of esophageal perforation of the intrathoracic esophagus.Thoracic surgery was consulted and it was elected to perform a minimally invasive esophagectomy given the short interval of time between perforation and definitive surgical treatment. Esophagogastrectomy was performed via a right chest (thoracoscopic), abdomen (laparoscopic), and cervical anastomosis. A feeding jejunostomy was placed with botulinum toxin injection of the pylorus. The procedure was uneventful with moderate contamination of the right pleural space which was irrigated until clean.Postoperatively the patient recovered well with no immediate complications from the surgery. Histopathology confirmed a diagnosis of poorly differentiated adenocarcinoma of the distal esophagus and stomach. Following surgery she suffered from altered mental status secondary to hyperammonemia and required multiple recurrent intubations for hypercarbia. She underwent tracheostomy and when her obtunded state resolved no longer required any ventilatory support. She suffered no other complications, required no additional interventions, and developed no infections. She returned to her preoperative living situation at an extended care facility where she resumed her normal activities. She remains well now four years after her esophagectomy.Esophageal perforation following instrumentation is an uncommon but important complication and one which retains a high morbidity and mortality . The cauEarly diagnosis is essential and to this end, a high index of suspicion must be maintained when managing a bulky esophageal mass. As there are treatment options, there is some debate surrounding the optimum choice of imaging modality. The usual initial test is a contrast esophagram; however such studies may yield false negative results in 10% of cases . Water-sOnce the perforation has been identified, the choice of operative approach must be considered. The use of esophagectomy for perforation was initially reported in the 1960s . At presThe role of minimally invasive surgery continues to expand, and since its introduction there has been concern as to whether or not it can be applied in the setting of perforation and contamination . TraditiMinimally invasive esophagectomy (MIE) has emerged as a means to reduce the morbidity and mortality of esophagectomy; however there is still little documentation of treatment outcomes in the setting of acute perforation. In 2002 Landen and El Nakadi  reportedMIE is gaining recognition as a procedure which offers results equal to open procedures with lessened morbidity at centers experienced in the approach , 18. Wit


We report a case of a 25-year-old primigravida who presented at 28 weeks of gestation with virilisation symptoms. Serum androgen levels were seven-hundred-fold higher than normal. A diagnosis of pregnancy luteoma was made at the time of caesarean section. The ovarian mass, serum androgen levels, and the condition of the patient improved after delivery.Luteoma of pregnancy is a rare, benign condition characterized by a tumor-like mass of the ovary that emerges during pregnancy and regresses spontaneously after delivery. It is usually asymptomatic and the diagnosis is generally incidental. Luteoma arises from the proliferation of luteinised cell under the influence of  Pregnancy luteoma is a rare tumor-like lesion of the ovary, first described in 1963 by Dr. Sternberg, that occurs during pregnancy and generally resolves completely after delivery [It is a benign condition: only in 30% of cases the mass is hormonally active leading to secretion of androgens causing masculinization in mother and female babies , 3.To date, fewer than 200 cases have been reported in literature and most of the patients were mildly symptomatic .An accurate diagnosis is fundamental for the management of the mother and fetus since it can be confused with ovarian malignancy .We report the case of a Caucasian woman with virilisation worsening symptoms in the second trimester of pregnancy.A 25-year-old primigravida presented at 28 weeks of gestation with symptoms of facial acne and abdominal and facial hirsutism appearing first at 23 weeks of gestation.μg/dL; normal range 98,8–340). A magnetic resonance was proposed but patient declined the study.Ultrasound imaging showed a viable fetus with a normal growth and morphology and a 12 × 9 cm right adnexal mass with necrotic areas and low color Doppler signals . The lefIn the following weeks blood tests and ultrasound follow-up showed progressive worsening of the clinical situation with an aggravation of hirsutism  and the onset of clitoromegaly and deepening voice.A caesarean section was performed at 34 weeks of gestation because of the worsening of laboratory tests and symptoms , the incomplete diagnosis due to the patient's denied consent, and the suspected ovarian malignancy. Antenatal corticosteroids with two doses of betamethasone (12 mg i.m.) were administrated to the patient prior to surgery.During the operation the uterus and left ovary were macroscopically normal, while an extremely enlarged right ovary mass was observed ; unilateA healthy male infant of 1560 grams was delivered; Apgar score was 8 at the first minute and 10 at the fifth minute.μg/dL, resp.).A blood sample of umbilical cord showed elevated levels of testosterone and DHEAS , acne on the face, shoulders, back, and chest, hair loss, and masculine symptoms such as clitoromegaly and deepening voice. Some symptoms such as acne and hair loss are fully reversible but hirsutism, deepening voice, and clitoromegaly may be only partially reversible after delivery.Up to 60–70% of female fetus may be affected with different degree by virilisation  , 3. The Rarely pregnancy luteoma of large size may cause ovarian torsion or tumor rupture with symptoms of acute abdomen, haemoperitoneum, or compression of the pelvic structures such as ureters .Diagnosis and follow-up of this condition should be made with noninvasive methods; at ultrasound scan luteoma appears as a solid, single, or multinodular mass. It may be unilateral or rarely bilateral with a cystic appearance due to the presence of haemorrhagic foci. However, because ultrasound exam during the second half of pregnancy may be challenging, considering the poor image quality caused by the uterine enlarged volume, magnetic resonance may be useful in the diagnostic evaluation of the patient .Differential diagnosis must be made with other solid ovarian neoplasm such as luteinised thecoma, granulosa cell tumor, or Leydig cell tumor .The management of pregnancy luteoma depends upon the symptoms and personal condition; different authors reported that surgical treatment with unilateral salpingo-oophorectomy was the most frequent option; however, whenever possible, conservative treatment should be recommended considering the benign nature of the lesion , 8. In fRecurrence of luteoma in subsequent pregnancies is possible but rare.Pregnancy luteoma represents a benign pregnancy related condition that generally resolves spontaneously after delivery. Antenatal accurate diagnosis is challenging but extremely important in order to optimize the obstetrical management of the patient and to improve the maternal and fetal outcome.


The ascending aorta is an uncommon site for non-infective thrombus. In non-aneurysmal and non-atherosclerotic vessels this condition becomes extremely rare, while it represents a source of potential cerebral and peripheral embolic events. Currently, there is no consensus in the guidelines on how to treat a free floating thrombus in ascending aorta, therefore we present our decision making process and therapeutic strategy.A healthy 48-year-old man was hospital admitted with acute abdominal pain. CT-scan showed a right renal embolism in presence of a defect in the distal ascending aorta suggestive for thrombus. After heart team discussion the patient was scheduled for surgery and successfully underwent an emergent thrombus removal. Also, owing to multiple aortic wall insertions, the ascending aorta was replaced. The patient’s recovery was uneventful and histological examination showed no signs of connective tissue disorders of aortic wall while confirmed the thrombotic nature of the mass.We present a patient with a floating thrombus in the ascending aorta who underwent an ascending aorta replacement. While angio-CT scan led to a prompt diagnosis, intraoperative epi-aortic echocardiography allowed to define precise location of thrombus, minimizing operative risk. This case demonstrates that multi-disciplinary heart team discussion is essential to define a successful strategy, that surgical treatment is feasible with specific tools such as epi-aortic echocardiography. The ascending aorta is an uncommon site for non-infective thrombus. In non-aneurysmal and non-atherosclerotic vessels this condition becomes extremely rare, while it represents a source of potential cerebral and peripheral embolic events. The exact mechanism of thrombogenesis is still unknown although several speculations have been proposed. How to treat a free floating thrombus in ascending aorta is still debated and no consensus exists yet –4. We reA 48-year-old male with unremarkable medical history was hospital admitted owing to acute abdominal pain. Computed tomography (CT) scan of the abdomen showed an acute right renal embolism in presence of thrombotic emboli in the distal superior mesenteric artery. Further investigation by means of a thoracic angio-CT scan showed a large intra-luminal free structure in the ascending aorta suggestive for thrombus Fig.  with no The evidence on the topic of floating thrombi in ascending aorta is very limited and dispersed. According to the International Guidelines  consensus is based on experts’ opinion (level of evidence C) and every case becomes peculiar and must be discussed in a Heart Team , 6. HoweOur patient was discharged from hospital on vitamin K antagonist for three months owing to previous renal embolism. After this period Aspirin has been started lifelong. A six months follow-up CT-scan showed no signs of thrombi. Since secondary prophylaxis still represents a debating issue, we propose an effective and feasible solution to treat thrombi in ascending aorta. In a field where no precise indications are available, we aim to contribute to develop specific guidelines and flowcharts that help consultants in the decision making process, ensuring the best care for patients.We present a patient with a floating thrombus in the ascending aorta. While angio-CT-scan led to a prompt diagnosis, intraoperative epi-aortic echocardiography allowed to define precise location of thrombus, minimizing operative risk. Since the treatment of floating thrombi in the ascending aorta is still debated and very little evidence exists, we report the decision-making process and strategy. In this presented case, we would like to underline the importance of the multi-disciplinary heart team and also the usefulness of intraoperative epi-aortic echocardiography. Due to the high risk of embolization and relatively low risk of surgical intervention, the ascending aorta was replaced. This case demonstrates that surgical treatment of a floating aorta is feasible and urgent intervention may offer a safe solution.


Laparoscopic myomectomy offers a real benefit to infertile patients with uterine fibroids and peritoneal adhesions. The procedure requires a skilled surgeon and laparoscopy technique to minimize adhesion formation and other proven benefits. Restrictions arise since this procedure requires power morcellation for fibroid tissue extraction. Two years ago, the Food and Drug Administration in the United States of America (FDA) issued the alert on power morcellation for uterine leiomyomas, addressing the risk of malignant cell spreading within the abdominal cavity . We review a 30-year-old female, without previous gestations, hypermenorrhea, intermenstrual bleeding, and chronic pelvic pain. Transvaginal ultrasound reports multiple fibroids in the right portion of a bicornuate uterus. Relevant history includes open myomectomy 6 years before and a complicated appendectomy, developing peritonitis within a year. Laparoscopy revealed multiple adhesions blocking uterine access, a bicornuate uterus, and myomas in the expected site. Myomectomy was performed utilizing power morcellation with good results. FDA recommendations have diminished this procedure's selection, converting many to open variants. This particular case was technically challenging, requiring morcellation, and safety device deployment was impossible, yet the infertility issue was properly addressed. Patient evaluation, safety measures, and laparoscopy benefits may outweigh the risks in particular cases as this one. Adhesions are abnormal peritoneal fibrotic bands with varying degrees of stiffness that connect two surfaces that are normally separated in the peritoneal cavity. They are responsible for locating the inflammatory reaction when there is an infection or foreign body carrying oxygen to ischemic tissue . Many seAfter surgical removal of adhesions (adhesiolysis), they will form again in 85% of the cases. In a survey conducted among gynaecological surgeons in German hospitals, adhesions were believed to develop in 15% of cases after laparoscopy ; still, Laparoscopic or open myomectomies are used for the removal of subserosal and intramural fibroids (types 4–7 in FIGO classification ). The adWe review a 30-year-old nulliparous female with hypermenorrhea, intermenstrual bleeding, and chronic pelvic pain for 4 months, prior to her office visit. A transvaginal ultrasound revealed a double uterus with a single cervix (bicornuate uterus) with the presence of 3 intramural leiomyomas (fibroids) in the right double uterus, measuring 41 × 33 × 33 mm, 57 mm, and 43 mm, respectively. The patient's relevant medical history included an open myomectomy 6 years ago with the removal of 4 leiomyomas, without the patient's knowledge of their location, and also a complicated appendectomy with peritonitis 1 year afterwards. She is programmed for laparoscopic myomectomy with previous bowel preparation and multidisciplinary evaluation in case of dense bowel adhesions. Bowel preparation was achieved with oral administration of sodium dibasic phosphate and 45 mL of sodium monobasic phosphate in half a glass of water, taking a separate, second glass of water 30 minutes later. Same dose was repeated 60 minutes later. Laparoscopy was performed placing an intraumbilical 5 mm trocar by direct vision and 1 accessory 5 mm trocar on the right and left lower pelvic quadrant, 3 cm above the iliac crest. Upon the introduction of the laparoscope multiple adhesions were observed see ; the uteIncision of the pseudocapsule with ultrasonic scalpel was performed and traction with a toothed grasper assisted in the fibroid enucleation. The 3 fibroids were removed, and hemostasis was performed see . We procReoxygenated methylcellulose gelatin (Surgiflo®) and reoxygenated methylcellulose matrix (Surgicel Snow®) was placed on the uterine serosa with the intention of covering the barbed suture and preventing adhesion formation. The final pathology report identified the tissue as leiomyomas with a total morcellated weight specimen of 101 grams. Patient was discharged 20 h later without complications.Over half of the estimated 400,000 inpatient hysterectomies, performed every year in the United States, are done through laparoscopic techniques; thousands benefit from laparoscopic myomectomy; this particular procedure requires morcellation for the large mass extraction through a small orifice . The casDue to the 2014 FDA recommendations on power morcellation , this caIt is important to remember that the FDA stated that the risk of spreading unsuspected uterine sarcoma is 1 in 350 to 1 in 7000 patients with the use of a power morcellation . The folComplex adhesion cases pose a serious enough challenge to any laparoscopy expert, further complications arise without the use of power morcellation, and the cumbersome use of other devices may hinder the decision of performing this procedure laparoscopically, opting for an open variant and, hence, eliminating the benefits a minimal invasion procedure provides for infertility situations.It is our conclusion that laparoscopic myomectomy, with power morcellation in cases such as these, with proper preventive controls, offers a real benefit for the infertile patient.


Plexiform fibromyxoma is a rare and distinctive benign mesenchymal neoplasm that occurs in the gastric antrum. This tumor has a potential for misdiagnosis as gastrointestinal stromal tumor (GIST). It causes mucosa and vascular ulcerations without advancement of the tumor. Cytological bland spindle cells within a variably myxoid stroma characterize the histology of the tumor. We report the case of a 41-year-old African Tanzanian lady who presented with melena and recurrent anemia. Endoscopy and imaging studies revealed antral mass with initial suspicion of a GIST. However, immunohistochemically it turned to be a plexiform fibromyxoma. Follow-up evaluation 12 months after surgery revealed no evidence of recurrence or metastasis. This is a very uncommon tumor, which, to our knowledge, has been reported only once in Africa. The clinicians need to be aware of this rare occurrence to avoid misdiagnosis as GIST tumor.  The tum protein . No KIT  protein . None of protein . We here9, and platelets count was 485 × 103. The patient also underwent evaluation of tumor markers. The patient's carcinoembryonic antigen (CEA) was 0.96 ng/ml, CA-125 was 17.1 μ/ml, CA-15-3 was 9.8 μ/ml, and CA-19-9 showed the value of 25 μ/ml. The patient's alpha-fetoprotein (AFP) was 3.71 IU/ml. The patient's HIV and hepatitis screen were nonreactive. CT scan showed gastric soft mass near the pylorus with no invasiveness to the surrounding structures (Figures A 41-year-old indigenous African Tanzanian lady was seen at gastroenterology outpatient clinic with recurrent anemia of six months' duration. She gave a history of upper abdominal discomfort associated with passing blackish tarry stools. Patient was mildly tachycardic with heart rate of 100 beats per minute. The patient's hemoglobin was 7.7 grm/dl, white blood cell count was 6.7 × 10 Figures . The pat Figures . ImmunohPlexiform fibromyxoma of the stomach is a novel, very rare entity of gastric mesenchymal neoplasms with nonspecific clinical manifestations. Only few cases have been reported so far since the first case described in 2007 by Takahashi et al. . Until OThe plexiform fibromyxomatous gastrointestinal neoplasm has never been reported in Tanzania and East African population. In Africa, a case has been reported in a South African adult in 2010 . More thThe clinical presentation of plexiform fibromyxoma in terms of signs and symptoms is nonspecific. Radiological features often overlap, and upper gastrointestinal endoscopy has a limited role because of intramural location. Endoscopic ultrasound, which yields opportunity to visualize and biopsy the tumor, needs special skills. Definite diagnosis requires histological and immunohistochemical analysis. Immunohistochemical processes are not done routinely in the developing countries. The fact that plexiform fibromyxoma is a rare tumor with only a few cases in this region can lead to underrecognition and misdiagnosis of this entity and pose a real diagnosis challenge to gastroenterologists, pathologists, and surgeons when encountering such patients and differentiating plexiform fibromyxoma from other gastric intramural tumors, especially GIST.


Laparoscopic cholecystectomy for acute cholecystitis and cholelithiasis is one of the most common operations performed in the United States. Inadvertent perforation and spillage of gallbladder contents are not uncommon. The potential impact of subsequent retained gallstones is understated. We present the case of an intraperitoneal gallstone retained from a previous cholecystectomy eroding into the bowel and leading to intraluminal mechanical bowel obstruction requiring operative intervention. This case illustrates the potential risks of retained gallstones and reinforces the need to diligently collect any dropped stones at the time of initial operation. Laparoscopic cholecystectomy for acute cholecystitis and cholelithiasis is one of the most common operations performed in the United States. The operative risks of bile leak and wound infection and the low incidence of common bile duct injury have been well documented. However, the potential impact of retained gallstones is understated. We present a case of an intraperitoneal gallstone retained from a previous cholecystectomy eroding into the bowel leading to intraluminal mechanical bowel obstruction. Clostridium difficile infection, and remote stroke requiring tracheostomy and percutaneous gastrostomy tube placement. Physical examination of the abdomen was notable for distention and diffuse tenderness to palpation. Plain abdominal film demonstrated a bowel gas pattern concerning an acute intestinal obstruction for which an abdominal computerized tomography scan was performed for further delineation. A 2.7 cm hyperdense lesion suspicious for a stone was appreciated intraluminally within the jejunum causing a high grade bowel obstruction (The Acute Care Surgery service was consulted to evaluate a 79-year-old female with new onset fever, abdominal pain, and emesis. Her complex past medical history included atrial fibrillation requiring anticoagulation, hypertension, recenttruction .This patient's surgical history included a laparoscopic cholecystectomy two months previously for gangrenous cholecystitis. The prior operative report revealed that the retrieval bag had ruptured on removal, and multiple gallstones were spilled into the intraperitoneal space. Complete retrieval was unsuccessful and gallstones were left behind. Of note, the patient had received an additional CT scan in the time span between the prior cholecystectomy and her current obstructive presentation due to an infectious colitis. At that time, the 2.7 cm stone was located resting dependently in the pelvis against the jejunum . We hypoThe patient was taken to the operating room for planned exploratory laparotomy. Intraoperatively a segment of jejunum was found adherent to the pelvis and 50 cm distally an intraluminal 2.7 cm gallstone. The stone was milked from the bowel and a short jejunal segment was resected. A primary stapled anastomosis was performed. Further exploration revealed no additional stones and absence of any cholecystoenteric fistulae.Gallstone ileus is an uncommon cause of mechanical intestinal obstruction occurring most frequently in the female geriatric population. Inflammation in the gallbladder bed coupled with the pressure effect of a gallstone leads to cholecystoenteric fistulae allowing passage of stones into the bowel. Larger stones greater than 2 cm are less likely to be successfully passed through the bowel and can become impacted in the distal small intestine thus leading to a mechanical obstruction . GallstoLaparoscopic cholecystectomy is a commonly performed procedure for acute cholecystitis and cholelithiasis. Inadvertent perforation and spillage of gallbladder contents are not uncommon. Although rarely clinically significant, it is prudent to recognize that subsequent retained stones are not benign. Previous reports have documented an approximately 1–12% complication rate from stones left in the intraperitoneal cavity . ComplicPerforation of the gallbladder most frequently occurs as a result of retraction or during dissection of the gallbladder from the liver bed. An acutely infected, inflamed, or thin walled gallbladder is more easily ruptured and needle decompression should be considered. If rupture does occur, every appropriate effort to recover dropped stones must be made. Previous reviews have recommended against laparotomy for unretrieved gallstones . However


Liposarcoma is an extremely rare primary bone sarcoma.We report a case of primary pleomorphic liposarcoma that arose in an 18 year old male in the metaphysis of the left tibia. Plain radiographs showed a partly sclerotic lesion and MR imaging a heterogeneous tumour predominantly isointense on T1- and high-signal on T2-weighted sequences with focal areas of increased T1 signal that suppressed with fat saturation. PET/CT showed marked FDG uptake (SUV = 17.1) in the primary tumour as well as a metastasis in the right distal femur and multiple small pulmonary metastases. Histologically, the tumour was a pleomorphic liposarcoma containing large tumour cells with vacuolated cytoplasm and hyperchromatic pleomorphic nuclei as well as numerous lipoblasts and scattered brown fat-like cells. Tumour cells strongly expressed FABP4/aP2, a marker of adipocyte differentiation, and UCP1, a marker of brown fat, but not S100. The case was treated with neoadjuvant MAP chemotherapy, resulting in extensive (> 95%) necrosis in the primary tumour and almost complete resolution of the femoral and pulmonary metastases.Pleomorphic liposarcoma can present as a sclerotic primary malignant bone tumour; markers of adipose differentiation are useful in histological diagnosis and neoadjuvant MAP chemotherapy results in significant tumor necrosis. Liposarcoma is an extremely rare primary malignant bone tumour defined in the 2005 AFIP Fascicle as “A malignant tumor with differentiation towards adipocytes” . Catto aWe report the clinical, radiological (including MRI and PET/CT) and pathological findings in a case of primary pleomorphic liposarcoma that arose in the proximal tibia of an 18 year old male. We also review the literature regarding primary liposarcoma of bone and reassess diagnostic criteria and treatment options in the light of our findings.An 18 year old white male presented with a 6 month history of discomfort and swelling in his left knee. An X-ray and MRI of the left knee revealed a large, partly mineralized tumour in the medial tibial diaphysis. In his past history, the patient had been diagnosed at birth with coarctation of the aorta and primary lymphoedema. Balloon dilatation angioplasty was successfully performed on day 13 of life. Subsequent cardiac medical management included nifedipine between the ages of 4 and 9; this was later switched to atenolol because of lymphoedema. His phenotype was consistent with a variant of Irons–Bianchi syndrome or ‘‘Milroy-like’’ lymphoedema. Further details of his medical history and treatment are described elsewhere . GeneticPlain radiographs demonstrated a poorly defined, permeative, radiolucent lesion, centred in the proximal tibial meta-diaphysis. A pathological fracture was present Fig. .Fig. 1a There was postero-medial cortical destruction with adjacent areas of ossification. There was evidence of low density in the medial soft tissue mass, raising the possibility of a fatty matrix.Magnetic resonance imaging (MRI) showed that the tumour filled the medullary cavity of the proximal tibial metaphysis; it had an ill-defined margin and crossed the physis extending to the subarticular surface but there was no intra-articular extension Fig. . ExtensiPositron emission tomography with fluorodeoxyglucose (FDG) integrated with computed tomography (PET/CT) confirmed an ill-defined proximal tibial lesion with medial cortical destruction and a large soft tissue mass containing areas of fat attenuation and ossification Fig. a. The tuHistology of a biopsy of the tibial mass revealed a proliferation of malignant cells that had vacuolated cytoplasm and large atypical pleomorphic nuclei Fig. . There wThe patient was treated with two cycles of neoadjuvant multi-agent chemotherapy, comprising methotrexate, doxorubicin and cisplatin (MAP), as per the closed European American Osteosarcoma (EURAMOS) trial. His baseline pre-treatment 2D transthoracic echocardiogram was within normal limits with an ejection fraction of 70%. The patient tolerated the chemotherapy well with clinical improvement after 2 cycles of chemotherapy as evidenced by a significant reduction in his analgesia use. The preoperative MRI showed an interval reduction of the large primary left tibial lesion but an increase in size and interval of the right distal femoral lesion. The preoperative PET scan demonstrated a discordant excellent metabolic response to neoadjuvant chemotherapy within both the primary tumour and the metastatic lesions. The FDG uptake was significantly reduced in the primary tumour of the tibia from 17.1 to 2.7 and the focus in the femur and right tibia showed only background activity. The majority of the bilateral pulmonary nodules had also resolved.The patient underwent an uncomplicated surgical resection of the primary tumour in the proximal tibia and the lesion in the distal femur with reconstruction using a linked distal femur and proximal tibial endoprosthesis  Fig. . At operPost-operative adjuvant chemotherapy was considered appropriate in this case because of the excellent response to chemotherapy. As routine cardiac assessment demonstrated an asymptomatic significant reduction in ejection fraction (70–53%), adjuvant chemotherapy was changed to 5 cycles of ifosfamide and etoposide, because of the previously reported activity of ifosfamide in liposarcoma . 12 montAlthough liposarcoma is a relatively common malignant soft tissue tumour, it has only rarely been reported in bone Table . In mostIn most previous reports, plain radiographs of primary liposarcoma of bone have shown an ill-defined osteolytic lesion, but sclerotic areas have also been noted , 25, 32.Our case showed typical histological features of pleomorphic liposarcoma. There were numerous small and large lipoblasts as well as many large tumour cells with vacuolated cytoplasm and highly pleomorphic nuclei; in addition, there were many brown fat-like cells with atypical large hyperchromatic nuclei. Tumour cells strongly expressed FABP4/aP2, a marker of adipose differentiation , and UCPMost previously reported intraosseous liposarcomas have been noted to exhibit morphological features in keeping with the pleomorphic liposarcoma subtype. However, other high-grade primary malignant tumours containing liposarcomatous elements have been described in bone. These include a liposarcoma containing osteosarcomatous foci , a mesenOutcome following surgery alone of primary liposarcoma of bone is relatively poor, and there is little data on the use of adjuvant or neoadjuvant chemotherapy or radiotherapy. A review of the literature shows that 16 patients (55%) died after a mean of 13  months, whereas 11 38%) were alive at a mean follow-up of 28 (range 5–60) months (Table % were al


Undifferentiated high-grade pleomorphic sarcoma in gastrointestinal tract is extremely rare, and its prognosis is poor.An 82-year-old man visited a previous hospital complaining of fever, general fatigue, and shaking chill, for which he received antibiotics therapy. As the fever continued, he was referred to our hospital, where computed tomography and upper gastrointestinal endoscopy showed a 6-cm gastric tumor. A preoperative biopsy was consistent with a malignant mesenchymal tumor, but could not provide a definitive pathological diagnosis nor prove a cause-and-effect relationship between the chief complaint and the gastric tumor. The gastric tumor had grown to 8 cm in diameter within a month so we performed a partial gastrectomy. The pathological postoperative diagnosis was undifferentiated high-grade pleomorphic sarcoma that produced granulocyte colony-stimulating factor. The patient’s fever quickly improved, and he showed a good postoperative course.We herein report a case of rapidly growing, undifferentiated, high-grade pleomorphic gastric sarcoma, which presented as a chief complaint of fever. Undifferentiated high-grade pleomorphic sarcoma (UPS) is a soft-tissue tumor usually found in the limbs and in the retroperitoneum. Its prognosis is poor. Occurrence in gastrointestinal tract is extremely rare. Here, we report a rare case of rapidly growing gastric UPS associated with a persistent fever and discuss the relevant literature.An 82-year-old man presented at a nearby hospital with fever, general fatigue, and shaking chill in July 2015. Although he was treated with antibiotics for his high level of c-reactive protein (CRP), his 38–39 °C fever remained. He was therefore referred to our hospital. When first examined, his body temperature was 38.4 °C but had normal blood pressure and pulse rate. No abnormal palpable mass was reported. His blood examination findings showed high inflammatory reaction, slightly low coagulability, and mildly activated fibrinolysis. However, we observed no immunoglobulin or specific antibody that could be considered the cause of his fever  CAM5.2, DOG-1, CD34, caldesmon, and desmin. Although these findings were consistent with malignant mesenchymal tumors, they did not confirm the pathological diagnosis. Upper gastrointestinal fluoroscopy in early August showed a 7-cm, multinodular sub-pedunculated raised lesions with a 12-mm attachment on the upper part of the greater gastric curvature , and then closed by Albert–Lembert sutures.In mid-September, we performed a partial gastrectomy. We used laparoscopic assistance to disconnect the omentum from the cardia toward the gastric body to ensure mobility of the stomach. The upper stomach was then lifted out of the abdominal wall through a 6-cm incision in the left upper abdomen. A vertical incision was added from the cardia to the gastric body in the gastric anterior wall, through which both tumors were pulled outside, and their roots excised from stomach wall by automatic suturing devices , no fever or other complications were observed thereafter. He began taking meals on 5POD, and he was discharged with satisfactory progress on 12POD. Positron emission tomography (PET)/CT examination on 1 month after surgery showed accumulated fluoro-2-deoxy-D-glucose at the wound, but no other abnormal accumulation.Malignant fibrous histiocytoma (MFH) has been classified into three subtypes: pleomorphic, inflammatory, and giant cell. By the World Health Organization (WHO) classification of bone and soft-tissue tumors of 2002, pleomorphic-type MFH was defined as a synonym for UPS . TypicalPrimary gastric UPS or MFH is quite rare. Saito et al. reported 16 case reports of primary UPS or MFH of stomach, including one they reported and 14 found in PubMed using the keywords “undifferentiated pleomorphic sarcoma” and “malignant fibrous histiocytoma” during 1984–2011 [According to the National Comprehensive Cancer Network (NCCN) Soft Tissue Sarcoma Guidelines, surgical resection with sufficient margin is the first-choice treatment for UPS. Amputation is required for tumors in limbs, so various surgical methods have been used with the aim of preserving function . The firAt first we suspected that infection or autoimmune diseases could have caused the fever and high CRP level; we had doubts about their association with the gastric tumor. However, as the fever and CRP level dramatically improved after the surgery, the gastric tumors were clearly related to the main complaints. There are reports about cases of UPS and MFH presenting initially with fever –23, and We found a rare case of gastric UPS. As UPS is a rapidly growing, frequently recurring tumor, its prognosis is poor. Patients require strict postoperative observation.


Although segmental arterial mediolysis (SAM) has been increasingly recognized as arteriopathy and there are some case reports about SAM, it is still very rare. It is characterized clinically by aneurysm, dissection, stenosis, and occlusion within splanchnic arterial branches, causing intra-abdominal hemorrhage or bowel ischemia. Mortality is as high as 50% in acute events. A 51-year-old man was referred to our hospital with hematemesis. Gastroscopy revealed a submucosal-like tumor on the posterior wall of gastric angle with ulceration. Computed tomography indicated a tumor measuring 65 × 50 mm in the stomach, which was suspected to have invaded into the pancreas. Significant hematemesis recurred; the patient developed shock and underwent emergency distal gastrectomy, distal pancreatectomy, and splenectomy. The pathology and the clinical course were compatible with SAM splenic artery rupture causing retroperitoneal hemorrhage that penetrated into the stomach. After that surgery, aneurysm of common hepatic artery ruptured and coil embolization was performed. SAM is an important cause of intra-abdominal or retroperitoneal hemorrhage in patients without underlying disease. SAM typically presents as intra-abdominal hemorrhage, but, in this case, the retroperitoneal hemorrhage penetrated into the stomach and it looked like a submucosal tumor. Segmental arterial mediolysis (SAM), previously called segmental mediolytic arteritis (SMA), is a rare nonarteriosclerotic, noninflammatory vascular disease first described in 1976 by Slavin and Gonzalez-Vitale . Though In this case, we report a case of SAM with heterochronic vessel rupture of the splenic and common hepatic arteries, treated by surgical intervention and coil embolization.The patient was a 51-year-old man who presented to the Emergency Department with hematemesis. His past medical history was unremarkable and he took no drugs. Physical examination revealed normal vital signs. On abdominal examination, the patient had no masses, muscle guarding, or rebound. Complete blood count revealed anemia with hemoglobin of 9.2. C-reactive protein (CRP) was normal, and the remainder of the serologic and immunologic workup was within normal limits. Gastroscopy revealed a submucosal-like tumor on the posterior wall of gastric angle with superficial ulceration . There wOn postoperative day 21, he exhibited peritoneal irritation in the form of a rigid board-like abdomen with shock vital signs. Emergency CTA showed a dissection and vessel rupture of aneurysm of common hepatic artery, causing intra-abdominal hemorrhage, so he underwent emergency coil embolization Figures .Follow-up was done every 6 months, and CTA one year after the first operation indicated the aneurysms  of the bilateral renal arteries remained.SAM is a nonarteriosclerotic, noninflammatory vascular disease that commonly presents as spontaneous intra-abdominal hemorrhage from middle-size splanchnic arterial branches. Abdominal pain, anemia, and shock are the main symptoms. Some cases present as bowel ischemia, hematuria, and hemobilia, but this is rare. Bleeding from the splanchnic artery sometimes causes a fatal results, so prompt diagnosis and treatment are required. To diagnose SAM, computed tomographic angiography (CTA), especially thin slice CTA, is very helpful. Digital subtraction angiography (DSA) is also useful because when the lesion is identified, we can treat it with embolization. For radiographic features, CT scans typically show mesenteric or intraperitoneal hemorrhage, and CTA and arteriography features include fusiform aneurysms, stenosis, dissections, and occlusions within the splanchnic arteries. A pattern of aneurysms and stenosis in series is characteristic, the “string-of-beads” appearance. Pathology is the diagnostic gold standard, and the underlying histological process is lysis of the smooth muscle of the outer media of the arterial wall. In cases in which collecting a specimen is not viable, CTA helps us to discover SAM lesions and it is thought to be sufficient to diagnose SAM . It affeTypically, SAM presents in middle-aged or elderly patients with no underlying disease, and laboratory markers of inflammation or collagen disease are absent. Because some angiographic findings are similar to fibromuscular dysplasia (FMD) findings, SAM is considered by some researchers to be an early lesion, a variant of FMD , 8–10. HThis case differs in that the CT scan showed dissection of the common hepatic artery and aneurysms of the common hepatic artery, splenic artery, and bilateral renal arteries. At first, the splenic artery ruptured, resulting in a retroperitoneal hematoma which penetrated into the stomach and masked as a submucosal tumor. Because the chief complaint was not typical intra-abdominal hemorrhage but hematemesis, at first we considered tumors such as GIST or NET.SAM typically presents as intra-abdominal hemorrhage, but there are a few cases in which bleeding occurs into the bowel lumen. If we diagnosed SAM based on the CTA findings, the first-line treatment for the hematemesis would be coil embolization. We should keep in mind the possibility of SAM alternatively presenting as digestive tract hemorrhage, for which surgery is preferred.SAM is an important cause of intra-abdominal or retroperitoneal hemorrhage in patients without underlying disease. SAM typically presents as intra-abdominal hemorrhage, but in this case, the retroperitoneal hemorrhage penetrated into the stomach and it looked like a submucosal tumor.