Alveolar soft part sarcoma (ASPS) is a rare mesenchymal malignancy. ASPS usually occurs most commonly in the deep soft tissues of the thigh and buttock or the head and neck regions.ASPS that originate from the uterine corpus are even more rare, with only 10 previous cases reported in the English literature.In our case, the alveolar features were completely lost and the tumour shows a solid, non-alveolar pattern and the nuclei have marked variation in nuclear size, and multinucleation. The correct pathological diagnosis has been made by immuno- histochemical and ultrastructural features, which rvealed overexpression of TFE3 and peculiar cytoplasmic crystalline inclusions.In this paper, an additional case of primary ASPS of uterine corpus is reported with immunohistochemical, ultrastructural study and review of literature in the effort to delineate its clinical and pathological features. In this unusual site, the diagnosis can be problematic because ASPS can mimic other primary or metastatic uterine neoplasms.Thus, in this unusual presentation an essential diagnostic marker is the nuclear over-expression of TFE3 as well as ultrastructural study, which reveals the presence of peculiar cytoplasmic crystalline inclusions. Alveolar soft part sarcoma (ASPS) is a rare mesenchymal malignancy with distinctive histologic and ultrastructural appearance. ASPS was first described in 1952 by Christopherson et al. as a neoplasm with of uncertain histogenesis whose cells were arranged in a pattern mimicking the small air sacks  of the lung [In fact, this neoplasm usually shows uniform, organoid nests of polygonal cells, separated by fibrovascular septa and delicate capillary-sized vascular channels. A prominent cellular dyscohesion within the nests results in a distinctive pseudo-alveolar pattern. Sometimes, the alveolar features can be completely lost and the tumour may show a solid, “non-alveolar” pattern . UsuallyRecent cytogenetic studies revealed that ASPSs are characterized by specific chromosomal translocation der(17)t p11;q25) that fuses the transcription factor 3 (TFE3) gene at Xp11 to the ASPL gene at 17q25, producing an ASPL–TFE3 fusion protein ;q25 that.ASPS usually affects adolescents and young adults in the second and third decades with slight female predominance . In adulThis neoplasm has been reported also in the female genital tract –26. As fIn this paper, an additional case of primary ASPS of uterine corpus is reported with immunohistochemical, ultrastructural study and review of literature in the effort to delineate its clinical and pathological features.A 66-year-old female was hospitalized for atypical vaginal bleeding and anaemia. On gynecologic examination, the uterus was enlarged and the cervix was prolapsed. Transvaginal ultrasound identified a well-circumscribed, intramural nodule measuring 5 cm in diameter, located in the uterine corpus. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Intraoperatively, no ascites or adhesions were seen surrounding the uterus, ovaries, and salpinges. No enlarged pelvic lymph nodes were noted neither peritoneal lesions were found.Gross pathologic examination revealed thickening of the cervical mucosa and an intramural, sharply circumscribed, round, firm, grey-white nodule with trabeculated cut surface located in the endocervix. The uterine corpus was enlarged and deformed because of an intramural nodule, measuring 5 cm in diameter. On cut section, this lesion was well circumscribed, soft, with irregular border, colour varying from yellow, brownish and grey and a large haemorrhagic zone centrally located Fig. . The ovaHistologically, the cervix showed atrophic epithelium and an intramural leiomyoma. The nodule of the uterine corpus was characterized by neoplastic cells arranged in an organoid pattern Fig. , with neOn immunohistochemical analysis, the neoplasm showed focal immunoreactivity to muscle-specific actin, caldesmon and caldesmin. Ki 67 index was very low Fig. . Strong Because of immunohistochemical and ultrastructural features, such as focal immunoreactivity to muscle-specific markers , negativity to other makers, strong and diffuse nuclear positivity to TFE3 and the presence of cytoplasmic crystal inclusions, the final pathologic diagnosis was primary uterine alveolar soft part sarcoma of uterine corpus. No images suggestive of other primary or metastatic lesions were observed on abdominal ultrasound, chest X-ray, total computed tomography or bone scan.Seven months after surgery, the patient was free of disease. In fact, when she was readmitted for further examination, no abnormalities were found in both physical examination and imaging studies.ASPS is a rare malignant neoplasm, accounting for 0.5–1 % of all soft part sarcomas . ASPS thThe age at diagnosis ranged from 14 to 66 years . Abnormal uterine bleeding was present in all patients. The size of the neoplasm varied from 0.4 to 7 cm  in diameter. In the majority of cases, the neoplasm was an intramural nodule. The diagnosis of ASPS in all cases was supported by histologic and EM examination, which revealed an alveolar pattern and the presence of cytoplasmic crystalline inclusions Table .Usually, the tumour nuclei were round-to-polygonal and vesicular, with prominent nucleoli, but cells with marked variation in nuclear size, nuclear cytoplasmatic inclusions and multinucleation, with very few mitoses have been observed in our case and in other ASPSs in the uterine corpus , 5 and eIn our case, the alveolar features were completely lost and the tumour show a solid, non-alveolar pattern and the nuclei have marked variation in nuclear size, nuclear-cytoplasmatic inclusions and multinucleation. The correct pathological diagnosis has been made by immunohistochemical and ultrastructural features, which revealed focal immunoreactivity to muscle-specific markers , negativity to other makers, strong and diffuse nuclear positivity to TFE3 and the presence of cytoplasmic crystal inclusions.Moreover, the lesion was considered primary uterine alveolar soft part sarcoma because no images suggestive of other primary or metastatic lesions were observed on abdominal ultrasound, chest X-ray, total computed tomography or bone scan.On the contrary, the review of literature revealed an immunohistochemical profile not very consistent with variable staining using other markers such as vimentin, desmin, myoglobin, muscle-specific actin, S-100 protein, HMB-45, neuron-specific enolase, synaptophysin, chromogranin, cytokeratin, EMA and NK1-C3 (melanoma-specific antibody) Table . Thus, iASPS of the uterine corpus enters in the differential diagnosis with other neoplasms presenting an admixture of spindle-to-epithelioid cells with eosinophilic-to-clear cytoplasm such as epithelioid smooth muscle tumours, epithelioid endometrial stromal tumours, perivascular epithelioid cell tumours (PECOMAs), uterine rhabdoid tumours, carcinomas, melanoma and malignant paraganglioma. These tumours characteristically do not show expression of TFE3 on immunohistochemical analysis and have other peculiar morphologic and immunohistochemical features.Epithelioid smooth muscle tumours are composed by mixtures of epithelioid, clear cell. A transition to typical smooth muscle cells in most instances confirms the smooth muscle nature of these tumours  and are Endometrial stromal tumours with a prominent component of epithelioid cells and abundant eosinophilic cytoplasm usually show areas with fusiform cells, characteristic arterioles and strong diffuse cytoplasmic immunoreactivity for vimentin and for CD10 .PECOMAs can arise in the uterus and are characterized by varying amounts of spindle and epithelioid cells with clear to eosinophilic cytoplasm, with immunoreactivity for melanocytic markers, most frequently HMB-45 .Malignant rhabdoid tumour is a highly aggressive tumour in adults and rapidly fatal and was first reported to have been found in the uterus in 1989 . HistoloMelanoma and carcinoma can be differentiated from ASPS because of greater cytologic atypia, pleomorphism, higher mitotic activity and immunoreactivity, respectively, for epithelial makers and for melanoma-specific antibodies.Malignant paraganglioma is characterized by polygonal to oval cells arranged in distinctive cell balls, called Zellballen, and shows more pronounced cell pleomorphism and immunoreactivity for neuroendocrine markers, such as neuron-specific enolase, protein gene product 9.5, synaptophysin and the presence of neurosecretory granules on EM examination .In extragenital sites, ASPS presents an indolent clinical course with unpredictable prognosis. Metastases to the lungs, bone and brain are the main cause of death , 42. LesThe more favourable prognosis in ASPS located in the uterine corpus may be due to small tumour size, anatomical location or relative short duration of follow-up. Indeed, in all patients, the ASPS of the uterine corpus, except for the present case and the case reported by Nolan and Gaffney, measured less than 5 cm  Table 22. Only iAn essential diagnostic marker in this unusual presentation of neoplasm is the nuclear over-expression of TFE3 as well as ultrastructural study, which reveals the presence of peculiar cytoplasmic crystalline inclusions.Moreover, in our opinion, a larger number of cases of ASPS in the female genital tract with longer follow-up and pathological findings including sizes should help to better define the biological nature of ASPS in the uterine corpus. In addition, although lymph nodes metastasis was observed only in the case of Zhang et al., surgical staging with complete pelvic lymph nodes sampling could be useful to evaluate therapy and prognosis of this rare neoplasm.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


Solitary cecal diverticulitis is a rare cause of acute abdominal pain in the Western world. Its clinical presentation, in most cases, mimics acute appendicitis. A 38-year-old Caucasian man presented with acute abdomen and clinical signs of acute appendicitis. Laparotomy was performed and revealed an inflammatory, solitary diverticulum of the cecum. A typical appendectomy was performed and a catheter was inserted for draining percutaneously the inflamed diverticulum of the cecum. The patient had an uneventful recovery and was discharged on the 4th postoperative day. This frequently misdiagnosed condition, in most cases, is being suspected and identified intraoperatively as acute appendicitis. The aim of this study is to review the available different surgical management options and to present an alternative therapeutic approach that may be valuable under specific circumstances. The cecal diverticulum is a rare disease with reported incidence of 0.04% to 2.1% 1–3). The condition is uncommon in the Western countries, where 85% of diverticulum occurs more commonly in the descending and sigmoid colon rather than in cecum [. The conThe disease is frequently misdiagnosed at the time of its occurrence. The symptoms and signs of the disease are well known to closely mimic acute appendicitis with abdominal pain, low-grade fever, nausea, vomiting, abdominal tenderness, and leukocytosis . Most auImaging studies on the other side could distinguish between right-sided diverticulitis and acute appendicitis with high accuracy and therefore they have the benefit of avoiding unexpected findings during the operation . In mostControversies exist regarding the optimal management in nonperforated cecal diverticulitis, ranging from conservative approach with intravenous antibiotics to surgical procedures such as diverticulectomy and right hemicolectomy . The manA rare case of a young man presenting with acute right, lower abdominal pain with solitary cecal diverticulum who was suspected preoperatively to have acute appendicitis is reported. The aim of the present case report was to review treatment strategies of this entity, since many issues still remain unclear, and to present an alternative therapeutic approach that may be valuable under specific circumstances.μL with 87 band forms. His past medical history was unremarkable. No preoperative imaging studies were performed because a presumptive diagnosis of acute appendicitis was made. The patient was transferred to the operative room for an emergency laparotomy and a standard open appendectomy through a McBurney incision was started. During the operation, a perforation of an acutely inflamed solitary diverticulum of cecum was found  count of 19,000/as found . The diaCecal diverticulitis is a rare clinical problem in the Western world, more commonly seen in the descending and sigmoid colon . Right-sThe differential diagnosis of diverticulitis of the right colon is wide and includes acute appendicitis, gastroenteritis, urinary tract infection, ureteric calculi, pelvic inflammatory disease, cecum malignancy, foreign body perforation, ileocecal tuberculosis, and Crohn's disease . Some stThe preoperative diagnosis of disease is quite difficult without radiological imaging. Barium enema may be helpful in making the diagnosis, especially in patients with previous appendectomy and in those with more indolent symptoms . PreviouThe management of solitary diverticulitis of cecum is varied and controversial due to lack of randomized trials comparing conservative and aggressive surgical treatment methods . If the There is no standard surgical procedure for the treatment of an acute, inflamed solitary cecal diverticulum, since the surgical approaches are not evidence-based. The choice of the surgical approach should be tailored to the operative findings and depends on the experience of the surgeon. Procedures range from simple isolated diverticulectomy and ileocecal resection to right hemicolectomy in patients with continued inflammation and suspicious malignancy .Simple diverticulectomy with appendicectomy may be adequate if the intraoperative diagnosis of cecal diverticulitis is made with confidence. Diverticular resection can take place from appendicular incision; it has low rates of morbidity and mortality and is appropriate for solitary cecal diverticulitis. However, retrospective studies have demonstrated that resection of the cecal diverticulum is not appropriate for large inflammatory lesions and in cases suspected for malignancy. In such cases, there is a need for more aggressive surgical method like ileocecal resection and right hemicolectomy , 31, 33.Other surgical treatment methods like the ileocecal resection have less surgical time than right hemicolectomy but cannot guarantee appropriate therapy in case of malignancy . In the In conclusion, this case report illustrates that solitary diverticulitis of the cecum, even if it is a rare entity, should be taken into consideration during the differential diagnosis of patients complaining of right iliac fossa pain. The surgical trainees must be familiar with the diagnosis and management of this rare surgical disease. Preoperative imaging studies have been shown to correctly identifying this rare surgical disease. However, the surgeon should base his diagnosis on the operative findings as in most cases they mimic acute appendicitis.


Choristomas are rare entities which are aggregates of microscopically normal cells or tissues in aberrant locations. They are a “heterotopic” rest of cells, as they appear as a tumor-like mass. Herein we report a case of cartilaginous choristoma in a 54-year-old male who presented with a swelling on right lower gingiva. The histopathological examination revealed features of a well circumscribed mass of mature cartilage in a dense fibrous connective tissue stroma. Choristoma, a congenital anomaly, is better described as a “heterotopic” rest of cells . ChoristCartilaginous choristomas are rare entities in the oral cavity region which are composed of mature hyaline cartilage. This paper reports one such rare case of cartilaginous choristoma of the gingiva.A 54-year-old male reported to the Department of Oral and Maxillofacial Surgery (OMFS) with swelling of gums on right lower posterior region of mouth for 5 years . The sweChoristomas are dysmorphic proliferation of cells that are not resident to the organ in which they arise and, like hamartomas, they gain a certain size and then cease to grow. The term choristoma was first introduced by Krolls et al. . Some reCartilaginous choristoma was initially explained by Berry in 1890 . The occCartilaginous choristoma is apparently seen as a trouble-free, firm swelling . This waCartilaginous proliferations of orofacial extraskeletal soft tissues apparently reflect the multipotential nature of primitive mesenchymal cells of that part of the body which may be stimulated to grow by trauma, irritation, or inflammation . In thisCartilaginous choristoma should be differentiated from cartilaginous metaplasia, which generally occurs in the soft tissue underneath ill-fitting dentures. On the contrary, our patient was a dentate person. Histopathologically, cartilaginous metaplasia is characterised by scattered deposits of cartilaginous cells and calcium that are arranged in a variety of phases of maturation in solitary or clustered cartilaginous foci .Histopathological examination of our case showed islands of basophilic mature cartilage in a dense fibrous connective tissue stroma with focal collection of chronic inflammatory cells, chiefly plasma cells and lymphocytes. The overlying peripheral epithelium was noted as parakeratinized stratified squamous epithelium.This case report highlights an occurrence of cartilaginous choristoma in a rare site—the gingiva in a male patient. This paper lays stress on the fact that cartilaginous choristoma is completely hamartomatous in nature and should be treated appropriately.Early and precise diagnosis can be made with a great understanding of this entity. The intraoral choristoma is mainly treated with surgical excision. Recurrences have not been reported in most of the cases with follow-up .


Sclerosing stromal tumor of the ovary is a rare, benign, sex cord stromal tumor occurring predominantly in younger women in the 2nd and 3rd decades of life. It typically presents unilaterally with only 2 previously reported cases of bilateral presentation. Common clinical presentations include pelvic or abdominal pain, a mass, or menstrual changes. Although occasionally presenting with hormonal manifestations, virilization as a result of androgen production by the tumor is rare. Here we present an extremely rare case of a sclerosing stromal ovarian tumor in a 14-year-old patient with bilateral presentation and with clinical and biochemical evidence of hyperandrogenemia. Sclerosing stromal tumor (SST) of the ovary is a rare benign tumor classified as a sex cord stromal tumor . First dWe present the third reported case of bilateral SST of the ovaries in a 14-year-old female and describe the ultrasound and MRI findings.A 14-year-old girl was referred for evaluation of primary amenorrhea and absence of breast development. Pubic and axillary hair development with body odor was described after age of 12 years. Physical examination revealed a lean, nondysmorphic female without acne or hirsutism, with Tanner IV pubic hair without clitoromegaly, and Tanner I breast development. Abdominal examination was benign.Baseline laboratory testing demonstrated beta hCG of <1 mIU/mL (0–5), alpha fetoprotein of 2.9 ng/mL (0–8.3), and carcinoembryonic antigen of 1.3 ng/mL (0–4.7) . Additional testing showed luteinizing hormone of 2.0 mIU/mL, follicle stimulating hormone of 4.1 mIU/mL, and estradiol of 18 pg/mL; DHEAS was 420 mcg/dL, with 17-hydroxyprogesterone of 365 ng/dL and testosterone of 135 ng/dL . Chromosomal analysis revealed normal 46,XX karyotype .Upon presentation of initial imaging, transabdominal ultrasound images demonstrated large bilateral solid pelvic masses Figures . A smallIntraoperative findings at laparotomy revealed abnormal appearance of both ovaries. The left ovary, which measured about 12 cm in greatest diameter, was replaced by an irregular, hard, calcified-appearing mass. The right ovary measuring 5 cm also appeared to be replaced by similar irregular, hard, calcified-appearing tissue. A left salpingo-oophorectomy was performed because the left ovary could not be salvaged. Dissection of the right ovary was performed with attempted removal of the abnormal tissue. The remaining right ovary was left in situ. A normal appearing, prepubertal sized uterus was noted. There was no palpable lymphadenopathy while the peritoneal surfaces were clear.On gross examination, the left ovarian mass measured 11 × 9 × 8 cm and weighed 352 grams. Serial sectioning perpendicular to the longitudinal axis revealed multiple solid, adjacent nodules with focal calcification. These nodules were well circumscribed and encapsulated with white-tan, whorled cut surfaces with the largest nodule measuring 7 cm in greatest dimension. Normal ovarian tissue could not be identified. The right ovarian specimen weighed 50 g and consisted of firm, lobulated tissue measuring 8 × 4 × 4 cm. Serial sectioning perpendicular to the longitudinal axis revealed a large calcified central portion with the remainder of the specimen composed of 2 adjacent tan-white, homogeneous, whorled, solid nodules measuring 2 and 6 cm in greatest dimension.Microscopic examination of both specimens revealed a pseudolobular architecture juxtaposed with biphasic hypercellular areas composed of bland collagen-producing spindle cells and hypocellular areas with focally edematous and fibrous stroma. Rare clusters of plump thecomatous cells with round nuclei and vacuolated eosinophilic cytoplasm were also noted interspersed throughout the stroma. Numerous, prominent thin-walled, and hemangiopericytoma-like (staghorn) vessels were also present within these bilateral tumors and in addition to rare ectatic (dilated) vessels in the left ovary. Although immunohistochemical stains were not performed, these morphological features along with the clinical history are most compatible with sclerosing stromal tumors of the bilateral ovaries .Following an uncomplicated postsurgical course, follow-up 2 months after surgery revealed Tanner IV breast development, followed by menarche 1 month later. This has subsequently been followed by regular menses. Blood testing 3 months after surgery also revealed normal androgen and precursor levels.Sex cord stromal tumors of the ovaries arise from embryonic sex cords or mesenchyme. The majority of ovarian sex cord stromal tumors are fibromas/thecomas (50%), granulosa cell tumors (10–20%), and Sertoli-Leydig tumors (5%).Review of the literature (PubMed) reveals numerous case reports describing sonographic and MR findings of ovarian SSTs. Sonographic features include a multilocular cystic mass of heterogeneous or hypoechoic echogenicity with irregular thick septae. Early and strong peripheral enhancement is a key characteristic feature in distinguishing between SSTs and other types of sex cord stromal tumors. MR is the best modality for the imaging of pelvic tumors with a standard protocol that should include T2-weighted sequences and dynamic contrast-enhanced T1-weighted images with fat saturation in multiple imaging planes. T1-weighted images typically show intermediate signal in the outer part of the lesion and low signal intensity centrally. On T2-weighted images, there is intermediate to low signal intensity peripherally with interspersed areas of high signal intensity septae centrally within the mass. The peripheral band-like low signal intensity may reflect compressed ovarian tissue, which is a distinguishing feature between SSTs and fibromas/thecomas. The high signal intensity areas on T2-weighted images correlate with poorly cellular tissue (fibrous portion of the tumor). Early avid peripheral enhancement with centripetal progression and general lack of enhancement within the central area are usually seen on postcontrast images . Our patHistologically, SSTs of the ovary are characterized by distinctive features involving pseudo lobulated cellular and acellular zones with the cellular areas usually containing numerous branched vessels. Microscopic examination of this patient's tumors demonstrated typical findings for SST.In conclusion, ovarian SSTs are very rare, benign, sex cord stromal tumors that typically present with a mean age of 28 years and usually are unilateral. Here we report an extremely rare case of an ovarian SST with bilateral presentation and with clinical and biochemical hyperandrogenemia, of which there are only 2 previously reported cases. Radiologically MR is the best modality for imaging of ovarian tumors with classic findings on T2-weighted images including intermediate to low signal intensity peripherally with interspersed areas of high signal intensity septae within the mass. Our case of SST is atypical as both ovarian masses demonstrate decreased T1 and T2 signal abnormality centrally with the mass.


Chronic expanding hematoma is a rare persistent hematoma that can sometimes be misdiagnosed as a malignant tumor due to its clinical and radiological features.18F]fluoro-2 deoxy-D glucose positron emission tomography images revealed increased uptake with a maximum standardized uptake value of 15.8 in the solid mass. As these findings were considered suggestive of hematoma associated with a malignant lesion, an open biopsy was performed. A pathological examination demonstrated a hematoma with xanthogranuloma, and no malignant cells were evident. Therefore, we resected the tumor including both components, and the histological diagnosis was chronic expanding hematoma. Clinical diagnosis based on 2-[18F]fluoro-2 deoxy-D glucose uptake is sometimes limited by the fact that 2-[18F]fluoro-2 deoxy-D glucose is taken up by not only malignant tumor cells but also macrophages and tissues with granulation or inflammation.A 42-year-old Japanese man with a large mass in his leg, suggestive of malignancy, presented to our hospital. He had been aware of the leg swelling for the last eight years. A magnetic resonance imaging scan demonstrated a large mass with two components. One was a large, well-defined cystic mass (13×9cm) showing high intensity on T1- and T2-weighted images, and the other was a solid mass  with high intensity on T1-weighted images. Two-[18F]fluoro-2 deoxy-D glucose positron emission tomography imaging, mimicking a soft tissue sarcoma, should be recognized as a potential diagnostic pitfall in cases of chronic expanding hematoma.Significantly increased standardized uptake value in the peripheral rim of the lesion on 2-[ Two-[18t tumors ,4.Here we present a case of CEH in the lower extremity. The lesion exhibited significantly increased FDG uptake on FDG-PET, mimicking the characteristics of a soft tissue sarcoma accompanied by hematoma.A 42-year-old Japanese man was referred to our hospital because of pain in his right leg. He had first become aware of pain in his leg during a rugby game eight years previously. Although the pain had subsequently improved, his leg had remained swollen for several years, without having suffered any obvious trauma or received any anticoagulant therapy. Before referral to our hospital, he had developed renewed pain in his leg for one month. Upon physical examination, a large hard mass was palpable in his right leg. A magnetic resonance imaging (MRI) scan showed a 13×9-cm cystic mass with high signal intensity on T1- and T2-weighted images between the soleus and lateral gastrocnemius, and a 3.5×2.5-cm solid lesion with high signal intensity on T1-weighted images in the medial gastrocnemius. This solid lesion adjacent to the cystic mass was enhanced on Gd-enhanced T1-weighted images  in a CEH arising from the extremity has not been reported previously. In this case, despite increased FDG uptake in the solid lesion at the peripheral rim, the final histological diagnosis was CEH with no malignancy. Therefore, a significantly increased SUV in the peripheral rim of CEH in FDG-PET images should be recognized as a potential diagnostic pitfall mimicking a soft tissue sarcoma with hematoma formation.Various kinds of imaging modalities have been utilized for diagnosis of CEH. FDG-PET imaging is increasingly being used in clinical oncology because it enables functional imaging of various tumors. Generally, high-grade sarcomas and aggressive benign lesions such as desmoid-type fibromatosis and giant cell tumor of tendon sheath tend to have higher SUVs than other benign lesions . Howeverlignancy . In the et al. reported that the surgical procedure should be complete resection, because incomplete resection might result in massive bleeding from a hypervascular subcapsular lesion [Although the treatment of CEH is still a matter of debate, most authors have recommended surgical resection. Aspiration or drainage of the material may not remove the clot contents fully, and undoubtedly will not remove the fibrous wall that might retain fluid. Additionally, aspiration of the fluid or incomplete excision could lead either to an unconfirmed diagnosis or to recurrence . Hanagirr lesion . TherefoIn summary, we have presented a case of CEH showing significantly increased FDG accumulation, which could have had the potential for misdiagnosis as a soft tissue sarcoma.Written informed consent for publication of this case report and any accompanying images was obtained from the patient. A copy of the written consent form is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.YN and EK prepared and drafted the manuscript and performed the literature research. EK and AK edited the manuscript and performed the literature research. DK and NS edited the manuscript and performed the literature research. YT and FN edited the manuscript. HC and YK edited the manuscript. EK and KO were the operating surgeons, and were involved in the preparation of the manuscript. All authors read and approved the final manuscript.


Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare soft tissue tumor of low malignant potential, which most often arises in the lower extremities. Lesions occurred in other anatomic locations have been rarely reported. Moreover, their imaging features have not been well discussed. Here we report a case of PHAT arising primarily in the pelvic retroperitoneum. To our knowledge, this is the first radiological description for retroperitoneum PHAT.A 26-year-old female was referred to our hospital for evaluation of a pelvic mass incidentally noted in routine pre-pregnancy ultrasonography examination. Magnetic resonance imaging (MRI) and computed tomography (CT) scan revealed an irregular mass with clear boundary in the pelvic retroperitoneum. Its signal intensity or density was inhomogeneous. On MRI images, it mainly showed isointense and slight hypointense on T1 weighted image and isointense and hyperintense on T2 weighted image. On contrast-enhanced images, it showed marked but heterogenous enhancement. With the delay time increasing, the enhanced area in the lesion increased but the CT value decreased. Dilated vessels and hemorrhage were detected in the tumor. With patience and careful separation, it was completely excised with great amount of bleeding during operation. Pathological and immunohistochemistry analysis confirmed the diagnosis of PHAT of the soft parts. We found no evidence of recurrence 18 months after operation.We present an extremely rare case of PHAT arising primarily in the pelvic retroperitoneum. To our knowledge, this is the first radiological description for retroperitoneum PHAT. The provided information is useful for summarizing the characteristics of this kind of tumor. It should be included in the differential diagnosis of a well-defined, inhomogenously enhanced hypervascular soft-tissue mass in pelvic cavity. Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts is a rare tumor described first in 1996 by Smith et al. . As thisA 26-year-old female was referred to our hospital for evaluation of a pelvic mass incidentally noted in routine pre-pregnancy ultrasonography examination. The lesion did not cause any discomfort, and the patient did not report any symptom. She and her family members had not experienced such lesion in the past. General examination was unremarkable and laboratory findings were normal.Magnetic resonance imaging (MRI) of the pelvic cavity was performed. MRI examination revealed an irregular mass with clear boundary in the pelvic retroperitoneum Fig. . It locaSubsequently, computed tomography (CT) examination was performed for further understanding this lesion and its surroundings, especially the adjacent vessels. Pre-contrast CT scan demonstrated a well-defined, irregular soft tissue mass in the same location showed on MRI Fig. . The intIt could be confirmed this mass with abundant blood supply did not arise in the organs in pelvic cavity according to the imaging examinations. Then, the lesion was radically excised. During surgery, the mass was found behind the posterior pelvic peritoneum. It is very irregular and tightly adhered to the adjacent tissue. No invasion was detected. There were many vessels in the surface of tumor. With patience and careful separation, it was completely excised with great amount of bleeding during operation. The cut surface was grayish-white in color and necrosis and blood clots were seen in its inner part. It showed solid tissue with a somewhat crisp texture. The histopathology findings showed clusters of dilated thin-walled vessels prominent hyalinization of the vessel walls, and a perivascular and intercellular ground substance. Pleomorphic tumor cells were distributed among these dilated vessels. The mitotic counts was 3/10 HPF. Hemorrhage was also detected. Immunohistochemistry revealed that the tumor cells were positive for vimentin, cluster of differentiation (CD) CD34, CD99, CD117 , and B‑cell lymphoma 2 (BCL-2), whereas S-100 protein, CD56, smooth muscle actin (SMA), Desmin, DOG-1, ALK-1 and actin was negative , paraganglioma and gastrointestinal stromal tumor (GIST). SFTs in pelvic cavity appear as well-defined masses with intense heterogeneous enhancement that persists on delayed phase images. Necrosis, hemorrhage, or cystic change may be seen . ParaganImmunohistochemically, the PHAT is positive for CD34, vimentin, CD99, and VEGF but negative for S-100 protein . HoweverIn conclusion, to the best of our knowledge, this is the first radiological description for retroperitoneum PHAT. This newly recognized, rare neoplasm has some imaging features corresponding to its pathological nature. Although its locations and imaging features are variable, the provided information is useful for summarizing the characteristics of this kind of tumor. It should be included in the differential diagnosis of a well-defined, inhomogeneously enhanced hypervascular soft-tissue mass in the pelvic cavity.We pledged to abide by the declaration of Helsinki (2000 EDITION) in accordance with the relevant medical research rules of China in the study. IRB at The Affiliated Hospital of Chongqing Medical University approved this study.Data are available on request from the corresponding author.


The current study presents a case of a 43-year-old female with giant bilateral calvarial hyperostosis across the superior sagittal sinus, secondary to brain meningioma. The patient presented with a huge mass in the bilateral calvarial region, and diagnoses of huge skull hyperplasia and meningioma were strongly suggested by computed tomography and magnetic resonance imaging examination. In addition, digital subtraction angiography demonstrated that the left middle meningeal artery and branches of the left superficial temporal artery were the major sources of blood supply to the tumor, with the little involvement of the right middle meningeal artery and branches of the right superficial temporal artery. The patient successfully underwent simultaneous embolization of the tumor-supplying vessels, total resection of the giant calvarial hyperostosis and intracranial tumor and skull cranioplasty. Additionally, histological study of the mass revealed a meningioma. The management of such a case presents a surgical challenge, however, the current study provides a good reference for the future treatment of similar diseases. Meningioma is the most common type of benign intracranial tumor and accounts for 20–25% of all central nervous system neoplasms. Meningioma usually grows slowly and is frequently found to compress the adjacent anatomical structures, which subsequently leads to the onset of neurological symptoms and signs. Skull hyperostosis is a well-known sign of meningioma and is observed in 4.5% of all types . AlthougIn July 2011, a 43-year-old female was admitted to the Department of Neurosurgery, Clinical Medical College, Yangzhou University  due to the progressive enlargement of a left frontoparietal mass for >30 years, with recurrent headache and hyperspasmia. The patient underwent skull tumor resection 30 years ago and the postoperative pathological diagnosis was skull hyperostosis. Following this surgery, uplift of the left frontoparietal region began gradually, which was accompanied by clinical headache and progressive enlargement of the lesion in the past two years. In addition, one week prior to admission, the patient experienced one episode of grand mal epilepsy which lasted for 15 min.The palpable fixed subcutaneous mass was 16×15×5 cm in size, with a hard texture, an unclear boundary and an uneven surface. The diagnoses of huge skull hyperplasia and meningioma were determined by computed tomography and magnetic resonance imaging examination  and 2. IThe surgical specimen was routinely fixed with 10% formalin and paraffin-embedded, prior to staining with hematoxylin and eosin . Histologically, the intracranial tumor was composed of a large number of meningothelial meningioma cells [World Health Organization (WHO) grade I] in the majority of areas and a few tumor cells exhibited severe atypism . The external section of the tumor that involved the full thickness of the calvarial bone superiorly extended to the extracranial soft tissue. Furthermore, the gray-white tissue observed in the inner side of the examined calvarial bone was resected and measured 15×14×7 cm in size. Microscopically, the hyperostotic bone contained some meningioma tissue  and the The cause of associated hyperostosis in meningioma remains a point of controversy, specifically in terms of whether it presents a secondary change of the bone without tumor invasion versus direct infiltration of the bone by tumor. However, tumor invasion of the bone appears to be generally accepted, as a number of cases with hyperostosis have revealed histological tumor cell infiltration of the bone ,3. In thet al  posed a formidable surgical challenge. In order to reduce bleeding during resection, it is necessary to embolize the major arteries supplying blood to the tumor, including the left middle meningeal artery and branches of the left superficial temporal artery. In addition, wherever possible, resection of the entire involved bone is recommended to prevent recurrence. In the present study, the bilateral frontoparietal flaps were extremely thick bone and, therefore, the removal of the flaps would have left a huge bilateral cranial defect involving the frontoparietal regions which may have required a significant and difficult reconstruction. An additional difficulty of the procedure is that following the removal of the bone flaps and subsequent exposure of the superior sagittal sinus, there is a risk of superior sagittal rupture, which often results in a significant amount of bleeding, even when no tearing of the superior sagittal sinus has occurred. Therefore, a large number of gelatin sponges are often required to achieve a significant hemostasis effect. Goel et al  also rep


The incidence of multiple intracardiac mass is rare. The differential diagnosis of intracavitary mass lesions includes benign, malignant primary, secondary metastatic cardiac tumors, or thrombus.We report the case of a 49-year-old Asian woman, who experienced a 2-week history of progressive exertional dyspnea, orthopnea, bilateral lower limb edema and palpitations. Transthoracic echocardiography showed one fixed round hyperechoic mass with central necrosis over the left ventricular apex, one oscillating hyperechoic nodule over the anterior mitral annulus and one irregularly heterogeneous mass bulging out from the lateral wall of the right atrium. The incidence of multiple myxomas is rare. Unfortunately, high tumor marker, serum lactic dehydrogenase and serum uric acid levels were also present. We could not differentiate between diagnoses of multiple myxomas with thrombi or multiple metastatic tumors.Primary intracardiac tumors are rare. Approximately 75% are benign, and approximately 50% are myxomas, which have an incidence of 0.0017% in the general population. Multiple intracardiac myxomas account for less than 5% of all cases of myxoma. Our case was an atypical picture of right atrial (RA) myxoma, as it was located in the RA lateral wall and extended to the RA auricle at the junction among the superior and inferior vena cava. Two masses in the left ventricle (LV) were thrombi and resolved after heparinization. Initially, elevated tumor markers and high serum uric acid and high serum lactic dehydrogenase levels were related to necrotic tumor-derived tissue, decompensated heart failure with pleural effusion and renal insufficiency. We share our experience of multiple intracardiac masses. Whether the intracardiac mass is benign or malignant, we recommend surgery due to the possibilities of systemic or pulmonary massive embolism, infection, arrhythmia and sudden death if the thrombus ruptures or the mass dislodges.The online version of this article (doi:10.1186/s13256-015-0650-4) contains supplementary material, which is available to authorized users. The incidence of multiple intracavitary masses is rare. Echocardiography is a good tool to detect intracardiac masses, and the differential diagnosis must include thrombus, vegetation and a foreign body. Multiple intracardiac myxomas account for less than 5% of all cases of multiple intracardiac masses . SecondaA 49-year-old Asian woman without systemic disease was admitted to our hospital with a 2-week history of progressive exertional dyspnea, orthopnea, bilateral lower limb edema and palpitation. Previously, she had been in good health except for mildly elevated blood pressure. A physical examination revealed a blood pressure of 195/138mmHg and a rapid pulse rate of 163 beats/minute. Jugular venous distention to 18 cm was noted, a chest examination revealed bilateral lower lung crackles, and a cardiac examination showed an irregularly rapid beat without an audible murmur. No palpable mass was detected in her neck, axillary or inguinal areas. Her lower limbs showed grade II edema. An electrocardiogram showed atrial flutter. Chest radiography showed a lower left lung patch and bilateral lung infiltration  level was 298.10U/mL, cancer antigen 199 (CA-199) was 52.00U/mL and cancer antigen 153 (CA-153) was 33.80U/mL. Cardiac magnetic resonance imaging (MRI) was arranged for differential diagnosis of multiple intracardiac masses. The image showed one 6.0 × 2.3cm lobulated mass bulging out from the lateral wall of the RA along with another two small nodules in the left ventricular apex and anterior aspect of the mitral valve. The one RA mass and two LV masses did not invade into the cardiac walls. All masses were near isointense on T1-weighted image (T1WI) and hyperintense on T2-weighted image (T2WI). We favored a multiple myxoma diagnosis Fig. .Fig. 4OnOn enhanced chest computed tomography (CT), a consolidated patch with central necrosis over the lower left lung and enlarged left anterior mediastinal and right paratracheal lymph nodes were also noted Fig. . However3) in which the left side was composed of muscle cells and the right side contained bland-looking, oval-shaped stellate tumor cells on a fibromyxoid background , RA and inferior vena cava (IVC). RA mass pathology with a 10 times view showed myxoma (5.1 × 3.1 × 1.5cmund Fig. . InteresThe final diagnosis was RA myxoma and LV thrombi that resolved after heparinization. At a follow-up 1 year after surgery, our patient had returned to her daily activities, and all laboratory data, including tumor markers, had returned to normal ranges. Subsequent TTE showed adequate LV performance, and there were no further atrial flutter episodes. The consolidated patch over the lower left lung, which we favored as an arrhythmia- or myxoma-related emboli-induced lung patch, also resolved 1 month later. We suspected that initially elevated tumor markers and high uric acid and high LDH levels were related to necrotic tumor-derived tissue, decompensated heart failure with pleural effusion and renal insufficiency.The incidence of multiple intracavitary masses is rare. The differential diagnosis of intracavitary mass lesions includes benign, malignant primary, secondary metastatic cardiac tumors and thrombus. Because an intracardiac lesion can be detected by echocardiography, the differential diagnosis must include thrombus, vegetation and a foreign body. However, intracardiac metastases, even though rare, should also be included in the differential diagnosis, as well as infectious and nonbacterial thrombotic or marantic endocarditis .Cardiac tumors represent 0.2% of all tumors found in humans. These tumors are divided into primary or secondary/metastatic. Secondary or metastatic tumors are 20–40 times more frequent than primary tumors. Primary intracardiac tumors are rare. Approximately 75% are benign, and approximately 50% are myxomas, which have an incidence of 0.0017% in the general population . Atrial Imaging modalities such as CT and MRI can provide additional information when echocardiography cannot delineate the extent of cardiac wall involvement. If myxoid tissue is abundantly available, the lesion will have an elevated signal in the T2WI, whereas if the fibrous component prevails, the mass will appear hypointense. Enhancement of the tumor is correlated with its histology, and the areas that are not enhanced upon imaging analysis correspond to necrosis or cystic degeneration. Enhancement is the key difference between myxomas and thrombi; the latter do not appear enhanced except in rare cases of vascularized thrombi .Myxoma surfaces may have smooth or lobulated macroscopic features. Oval, rounded, and irregular shapes have been described, and a brownish color appears to predominate. The consistency of myxomas also varies from firm to gelatinous. The microscopic features of myxomas are characterized by a myxoid matrix rich in mucopolysaccharides in which polygonal cells with an eosinophilic cytoplasm can be detected. The polygonal cells may appear as star- or nest-shaped and may be multinucleated. Microscopic characteristics including mitoses, necrosis, or pleomorphisms are usually not detected or are eventually present as mild findings .The classic triad found in patients with cardiac myxoma is characterized by obstruction of blood flow, constitutional symptoms and thromboembolic events. The obstruction of blood flow leads to intermittent heart failure, and, similar to systemic nonspecific flu-like malaise symptoms, there is usually a low fever of long duration, arthralgia, anorexia, and thromboembolic events. RA myxoma in particular can obstruct the tricuspid valve, causing signs and symptoms of right-sided heart failure, peripheral edema, ascites, hepatic congestion, and syncope. The signs and symptoms caused by myxomas are atypical and highly variable and can result in a difficult diagnosis of this neoplasia. According to the size, mobility and location of the tumor as well as physical activity and body position, patients may have an asymptomatic course or progress with thromboembolic events that may even lead to sudden death , 10. We We share our experiences regarding an atypical picture of RA myxoma, which was located in the RA lateral wall with extension to the RA auricle at the junction among the SVC, RA and IVC. Initially, clinical presentation was arrhythmia as atrial flutter and decompensated heart failure. This myxoma was also accompanied by two LV thrombi and pulmonary thromboembolism. Although high tumor markers, LDH and uric acid levels suggested a possibly secondary malignancy diagnosis. The most common mass was thrombus. In our case, the LV thrombi and pulmonary thromboembolism were resolved by heparinization. Whether the intracardiac mass is benign or malignant, we recommend surgery due to the possibilities of systemic or pulmonary massive embolism, infection, arrhythmia and sudden death if the thrombus ruptures or the mass dislodges.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Pyogenic granuloma (PG) is a polypoid lobular capillary hemangioma rarely observed in the gastrointestinal tract. Only a few cases in the small bowel have been described in the literature.A 58-year-old man had been suffering from general fatigue and severe anemia. Esophagogastroduodenoscopy and colonoscopy did not reveal any significant bleeding. Abdominal computer tomography revealed a hypervascular tumor in the small intestine. Oral double-balloon endoscopy (DBE) detected a polypoid lesion (2 cm in diameter) in the jejunum. We performed laparoscopic-assisted partial resection of the jejunum. The histological features of the tumor were consistent with PG. The patient’s anemia gradually improved without the need for oral iron after surgery.In this case report, we present a case of pyogenic granuloma in in the jejunum that was detected by DBE. Obscure gastrointestinal bleeding (OGIB) is frequently observed in the small intestine. Double-balloon endoscopy (DBE) and capsule endoscopy (CE) are highly effective in diagnosing the origin of OGIB , 2. DBE Pyogenic granuloma (PG) is a capillary hemangioma that usually occurs on the skin or in the oral cavity; it is rarely observed in the gastrointestinal tract. We report a case of PG in the jejunum that was detected by DBE.A 58-year-old man had been suffering from general fatigue and severe anemia for several months. His hemoglobin levels were 6.6 g/dl . He had no medical history and did not take any medicine. Esophagogastroduodenoscopy and colonoscopy did not reveal any significant bleeding. Abdominal computer tomography revealed a 2-cm hypervascular tumor in the small intestine (Fig. Examination of the resected tumor showed that it measured 19 × 16 mm in diameter Fig. . HistoloPG, also called granuloma telangiectaticum or lobular capillary hemangioma, was first described in 1897. Gastrointestinal PG is apparently a rare cause of hemorrhage in the digestive tract. PG is a benign lesion that is considered reactive, e.g., it occurs as a result of minor trauma. However, the precise etiology is unknown. Satellite lesions have been described on the skin, which argues against trauma as the sole cause. Lesions are also found more frequently during pregnancy on the skin and in the mouth, suggesting that hormonal influences may also play a role. All gastrointestinal PGs reported thus far have occurred in elderly patients, both male and female . The disTo our knowledge, only 16 cases of PG in the small intestine have been described in the literature, and most involved Japanese patients (Table The tumor size is usually less than 20 mm, with a clinical presentation of anemia. Of the 17 reported cases, 12 involved the ileum and 5 involved the jejunum. Most tumors had an irregular shape without surface ulceration and were reddish in color. Surgical resection is the mainstay for treating small bowel PGs, although endoscopic resection with an electrosurgical snare is an alternative technique if an endoscopic approach is possible. Endoscopic treatment is a relatively easy, safe, and low-cost procedure for small-sized PGs of the gastrointestinal tract . HoweverIn the present case, we performed laparoscopic-assisted partial resection because complete endoscopic resection of the 2-cm hypervascular submucosal tumor with ulceration seemed to be difficult.PG in the small intestine is a rare cause of anemia and is difficult to detect. Awareness regarding this infrequent benign lesion will make it easier to diagnose and treat it properly.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


Atypical teratoid/rhabdoid tumors are rare and highly malignant central nervous system tumors. They have no specific radiological features and often present several histological components that make a problem in differential diagnosis with medulloblastoma and primitive neuroectodermal tumors.We present the case of a newborn girl complained of a gradual proptosis of the left eye secondary to an expansive lesional process of the optic nerve. The location at the optic nerve, reported only twice in the literature, and an exclusive rhabdoid appearance on biopsy added additional differential diagnosis problems.The proptosis worsened and the infant died few days after two cycles of chemotherapy.http://www.diagnosticpathology.diagnomx.eu/vs/2037718783145212.The virtual slides for this article can be found here:  World Health Organization (WHO) defines atypical teratoid/rhabdoid tumour (AT/RT) as a highly malignant central nervous system (CNS) tumour predominantly manifesting in young children, typically containing rhabdoid cells, often with primitive neuroectodermal cells and with divergent differentiation along epithelial, mesenchymal, neuronal or glial lines; associated with inactivation of the integrase interactor (INI)1/hSNF5 gene in virtually all cases [A newborn girl, without perinatal history, presented at birth a gradual increase of the left eye volume. At day 20 of life, parents decided to consult a doctor. Physical examination showed proptosis of the left eye.The ultrasound revealed 2 cm hypoechoic formation occupied the posterior pole of the orbit without Doppler vascularity, well limited by printing capsule at the bottom and outside of the optic nerve. The globe was normal. Orbital magnetic resonance imaging (MRI) revealed an expansive fusiform lesional process in intraconal space encompassing the optic nerve, measured 25/19/17 mm Figure . It showMalignant rhabdoid tumors were originally described in kidney then at other sites especially soft tissue -9. In 19AT/RTs are most often infratentorial ,13, locaAT/RT of the optic nerve, as in our case, is exceptional and only two cases were reported in the literature. In 2006, Allen et al. described a case of an AT/RT of the optic nerve secondarily arising in a ganglioglioma . Verma eCT reveals a hyperdense solid component with moderate to marked enhancement with contrast medium . MRI is Macroscopically, AT/RTs tend to be soft, pinkish-red and bulky, and typically contain necrotic foci and may be haemorrhagic .Histologically, the most striking feature are nests or sheets of cells with classic rhabdoid features: abundant cytoplasm contains paranuclear eosinophilic globular inclusion, eccentrically placed nuclei with a vesicular chromatin and a prominent nucleoli ,12. By eIn our case, we dealt with a biopsy showed only rhabdoid cells. The problem of differential diagnosis arose with rhabdomyosarcoma. The negativity of desmin at first then the negativity of the INI 1 allowed us to rectify the diagnosis. In other locations, the differential diagnosis includes medulloblastomas, PNET and germ cells tumors. ATRT shares some morphological features with those entities, but the presence of rhabdoid cells as well as immunohistochemical study gave the correct diagnosis.Deletions and mutations of the hSNF5/INI1/SMARCB1 locus at 22q11.2 were demonstrated in AT/RTs as well as in rhabdoid tumors of the kidney and extra-renal sites ,22. It sFor treatment, there are no definitive guidelines for AT/RTs because of their rarity. However, it based on surgical resection, chemotherapy and radiotherapy. Surgical resection must be as radical as possible. Indeed, patients undergoing gross total resection have a better survival compared to the patients with incomplete resection ,23. RadiAT/RTs correspond to WHO grade IV, having a poor prognosis . The meaIn summary, AT/RTs are very aggressive CNS embryonal tumors that make several problems in differential diagnosis. In our case, the location and the histological aspect posed additional diagnostic difficulties. An accurate diagnosis is imperative because AT/RTs have worse prognosis. At that context, pathological analysis is critical.Written informed consent was obtained from the parents of the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


We present a case of Kaposi’s sarcoma (KS) of the heart in a 45-year-old non-immunodeficient woman with symptoms of pericardial effusion and cardiac tamponade. Computed Tomography (CT) coronary angiography and transesophageal echocardiography (TEE) showed a low-density tumorous mass (50 mm diameter) at the level of auricle of the right atrium spreading towards the superior vena cava, floating in the cavity of the right atrium. On histological examination, the tumor consisted of fibrovascular connective tissue with areas of necrosis and hemorrhage. Fibrous septae contained different sized thin walled capillary type blood vessels and lymphangioma-type vascular spaces. Vascular spaces were surrounded by extravasated erythrocytes, deposits of hemosiderin and sparse lymphoplasmacytoid infiltrates. On the periphery of tissue fragments and around vascular spaces, there was a cellular kaposiform proliferation of the spindled cells. Slit-like spaces between spindle cells contained erythrocytes. Nuclear pleomorphism of the spindled cells was minimal. Few mitotic figures were present. Spindle cells were Vimentin, CD34 and CD31 positive. More than 10 % of spindled cells were Ki67 positive. This characteristic histology and immunohistochemistry is consistent with Kaposi’s sarcoma. Patient has no history of other malignancies and no other primary tumor was detected. Patient also was negative for HIV infection. There are only 10 documented cases of primary Kaposi’s sarcoma of the heart in non-immunodeficient patients reported in the current medical literature. Our report is the first case in which imaging, histology and immunohistochemistry data are available.The online version of this article (doi:10.1186/s13000-015-0354-5) contains supplementary material, which is available to authorized users. Primary tumors of the heart are very rare. Lifetime incidence ranges between 0.0017 to 0.02 % –11.TableKaposi’s sarcoma (KS), which was described by Hungarian dermatologist Moritz Kaposi in 1872 , occurs Cardiac KS lesion mostly remains unrecognized during clinical and imaging investigation and is diagnosed only by pathologists. It is suspected that, before introduction of molecular techniques, such lesions have been confused with primary angiosarcoma of the heart, which is more common, than Kaposi’s sarcoma . MoreoveHere we present the case of a 45 year old non-immunodeficient woman with primary Kaposi’s sarcoma of the heart and a review of 10 cases, which we found in the medical literature through PubMed search using the search terms “Kaposi’s sarcoma” AND “heart”.45 year old female patient with symptoms of pericardial effusion and cardiac tamponade was referred to the clinic. Pericardiothentesis was performed immediately. Microbiological and cytological investigation of the pericardial fluid showed no evidence of an infection or malignancy. On the Computed Tomographic (CT) coronary angiography at the level of auricle of the right atrium, low-density tumorous mass (50 mm in diameter) was found Fig.  An addi (AdditioParaffin embedded tissue samples from the pericardium and resected intracardiac tumor (auricle of the right atrium) were sent to our laboratory for reference.Tissue fragments had heterogeneous structures. One sample consisted of fibrovascular connective tissue with adipose tissue inclusions. Capillary and venous type blood vessels, with perivascular lymphoplasmocytoid infiltration were found in fibrose tissue. Two other tissue fragments generally were characterized by erythrocytes and “ghost” cells. Areas of necrosis and hemorrhage were surrounded by connective tissue septa Fig. . They coSpindle cells were positive for vimentin, CD34 and CD31 Fig. and d. EnFinal diagnosis was primary Kaposi’s sarcoma of the heart. The patient had no history of other malignancies and no other primary tumor was detected on CT or Magnetic Resonance Imaging (MRI). Patient was tested negative for HIV infection.Our paper presents a case of primary Kaposi’s sarcoma of the heart in non-immunodeficient female patient Table . ClinicaIn the available literature, we found 10 confirmed cases of primary Kaposi’s sarcoma of the heart. Patients’ age varied between 14 to 60 years with mean age of 41.9. 70 % of patients were more than 38 years old. Most tumors were localized in the right atrium . In mostRarity of primary KS of the heart is not the only problem during histological evaluation of the tumor specimens. These lesions contain many necrotic areas, hemorrhages and thrombi, which complicates the tumor sampling. It is very important to perform a careful gross pathological examination and dissection (in case of postmortem study) or tumor tissue sampling . Pathologists have to promptly examine all suspicious tissues for histology. As shown by others  and in oIn some cases, exact type of tumor has no great impact on clinical outcome  but identification of the correct histologic type of the surgically resected tumor may be meaningful for consideration of adjuvant chemotherapy .In summary, we report a rare case of primary KS of the heart, which showed overlapping clinical, radiological, histopathological and molecular features with other neoplastic and non-neoplastic lesions. Only histological and cytological features gave a clue about nature of the lesion. Immunohistochemistry in such cases plays a role of ancillary study and confirms the endothelial origin of tumor cells. It is helpful in cases when tumor mostly consists of diffuse proliferation areas mimicking fibrosarcomas , 20. We Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


During osteogenesis, mesenchymal tissues function to form fibrous matrix which changes into bone by ossification. In rare instances, fibrous matrix persists in which foci of immature bone is evident resulting in progressive enlargement. Such conditions are commonly benign in nature but few are anatomically benign and clinically destructive. Though recurrence and residual defects following surgical treatment are the challenging complications, fatal consequences are infrequent. We report a juvenile case of ossifying fibroma with an aim to highlight its clinical course and salient criteria to differentiate this entity from the common variants. Literature mentions this lesion as aggressive ossifying fibroma and active ossifying fibroma. This fibro-osseous type of neoplasm is nonencapsulated but demarcated well from the surrounding bones, exhibiting rapid enlargement. Thinning and expansion of adjacent cortical bones, eroding the bone to invade the adjacent tissue spaces are the result of its aggressive and active course. Though predominantly occurs in children, 20% of reported cases were above 15 years. JOF is composed of fibrocellular tissues, mineralized materials and small foci of giant cells.A male child aged 4 years was brought to the hospital with a complaint of swelling on the upper anterior teeth region since 15 months. Upon analyzing the history, the father in-formed that, the right upper central incisor was not erupted till the age of 2 and the child was taken to the dental clinic. Dental records stated that opperculectomy was done in 51 region following which the tooth erupted. 6 months later the patient developed a small swelling in the gum region in relation to 51. Swelling was gradually increasing in size with the displacement of incisor teeth and associated with pain.On examination, a single well-defined sessile growth was evident on the anterior maxillary region encircling the crown of right upper central incisor . The sizAbove features made us to think, bony sarcoma and odontogenic tumor as possible differential diagnosis.Maxillary occlusal view in relation to 51 and 61 region showed well-defined radiopaque mass with numerous specks producing ground - glass appearance between widely displaced 51 and 61. The opaque mass was surrounded by unilocular radiolucency. No evidence of root resorption in relation to 51 and 61. Tooth buds of permanent central incisors were found above the radiopaque mass .CT maxillary view demonstrated expanded cortex in the anterior alveolar region with ill-defined mixed mass surrounded by areas of corrugated margins .Microscopic analysis of incisional biopsy specimen demonstrated nonencapsulated rich cellular connective tissue stroma composed of proliferative plumps of fibroblasts along with collagen fibers . Round tClinical, radiographic and histopathological corelation suggested the final diagnosis of juvenile ossifying fibroma.4 Features to be considered to diagnose JOF include the following:7Juvenile ossifying fibroma (ICD-O-code 9262100) was titled by Johnson et al in 1952. Childhood or early adolescent onset of lesion with the history of rapid increase in size and palpatory pain. Maxillary involvement. Radiographic size of the lesion should be greater than 5 cm in diameter. On computed tomography perforation of the expanded cortex is characteristic. Recurrence following treatment.Nature of the lesion can not be predicted histopatho-logically and often ossifying fibroma is the diagnosis.89 Apart from the anatomical site variation differences between the two types are enumerated in Etiology of JOF seems to be De Nova without any apparent causative factor. Two distinct variants of it are psammomatoid juvenile ossifying fibroma (PsJOF) and Trabecular juvenile ossifying fibroma (TrJOF). Majority of PsJOF occur in orbital bone and paranasal sinuses, where as TrJOF primarily affects maxilla followed by mandible.10 Trauma is a definite preceding factor and malignant transformation in the rapidly proliferative reparative tissue is the etiopathology. Radiographic findings of mixed radiopaque and radiolucent type of lesions and surgical trauma in our patient’s dental record further supported the clinical diagnosis. Chondrosar-coma also has the similar features with equal frequency in maxilla and mandible.The age and clinical findings of our patients are favorable towards jaw sarcomas. Osteosarcoms are reported in wide range of age group from young children to 3rd and 4th decade of life with high prediction for males.11 The study of Buchner et al 12 states that the peripheral odontogenic fibroma is the most common type of OT. Clinical manifestations approximate to our patient but age and site are contradictory. Reported mean age was 32.3 years and mandible is the most common site.As the growth involved both labial and palatal aspects of gingiva with bony hard consistency, odontogenic tumor was also considered. Odontogenic tumors (OT) are comparatively rare and comprise about 1% of all jaw tumors.Histologically JOF is composed of cell - rich fibroblastic stroma with osteoid matrix incorporating plump eosinophilic osteoblastic cells. Elaboration of mineralized products by the lesion leads to progressive calcification of the osteoid and immature woven bone formation. Focal collections of multinucleated giant cells are common. Collagen is not a usual finding but its presence favor for older lesions. Cystic transformation in tumor mass may sometimes occur and is the consequence of rapid tumor growth in young patients.12 Displacement and root resorption of teeth are common at lesion site.On conventional radiograph characteristically the lesion appears as well-defined, radiolucent mass with variable amount of radiopacities. The active zones of growth presents initially as radiolucent locules which become radiopaque later.Computed tomographic finding of JOF include well defined expansive lesion with variable amount of calcification.Mixed radiopaque and radiolucent type of lesion that microscopically reveals fibrous stroma with woven bone is unique for fibro-osseous group of lesions and JOF being a member of this group it should be distinguished from the rest of similar lesions. en block resection.13Periosteal elevation with subperiosteal spread leads to local invasion and indistinct separation between tumor and adjacent tissues make the prognosis uncertain and necessitate more aggressive surgical procedures such as 14In our patient the duration and size of the lesion dictates the slow growing nature of the lesion. Slow growth is the uncommon features of this rare lesion and reported literature are also fewWhen the treatment plan was decided, the well-defined nature of tumor with no evidence of bony perforation and slow growing tendency were in favor for opting conservative mode of surgery than radical resection. Complete excision of tumor mass was carried out along with teeth followed by curettage of tumor cavity .The patient is under observation for the past one year with periodic recall visits and conventional radiographic analysis of the lesion site to verify the resolution.


Metastatic laryngeal cancer to the small bowel is extremely rare. Management of small bowel obstruction used to constitute a relative contraindication for the use of laparoscopic modality. We are reporting a case of an elderly man known to have laryngeal cancer who presented with small bowel obstruction due to metastatic deposit to the small bowel. The condition was successfully treated by laparoscopic assisted approach. A review of the natural history of advanced laryngeal cancer, common and uncommon sites of metastasis, and the rare presentation as small bowel obstruction is illustrated in this review. Complete small bowel obstruction secondary to metastatic laryngeal caner is rarely reported in the literature. Advanced laryngeal squamous cell carcinoma usually spread via lymphatic channels to the neck and chest. Most common sites of metastasis are reported to be in the lungs. In the rare conditions where the hematogenous spread of the disease leads to small bowel deposit, it may manifest itself as small bowel obstruction due to constricting involvement of the bowel wall by the neoplastic process. Other rare presentations of small bowel involvement include hemorrhage and perforation.Treatment of the condition is most of the time carried out on emergency basis with resection of the involved bowel segment using conventional techniques. In order to be able to use minimally invasive modality, a working space is usually required. This space is often obscured by the dilated bowel loops secondary to the obstruction, which make the performance of such procedures very challenging. The following case illustrates the successful treatment of the rare metastatic laryngeal cancer to the small bowel using laparoscopic assisted techniques.An 82-year-old Emirati male was diagnosed to have laryngeal cancer in 2007. He underwent surgical treatment with total laryngectomy and adjuvant chemoradiotherapy for a Stage III disease. Follow-up by Ear, Nose and Throat colleagues showed no evidence of recurrence over 6 years. He is well surrounded by very caring family members and was able to manage his daily activities with little help.One month prior to his presentation to the hospital, the patient reported intermittent constipation associated with vague mild abdominal pain. Three weeks later, the patient presented to the emergency department complaining of obstipation for the last three days, nausea, and two episodes of biliary vomiting, mild abdominal distention, and exacerbation of the diffuse colicky abdominal pain. Pertinent physical findings revealed a cooperative patient with an ECOG performance status of 2, dehydrated with dry mucous membranes, a soft abdomen with moderate distention mainly in the upper part, tympanic to percussion, with slight exaggeration of the small bowel sounds on auscultation. Rectal examination showed an empty rectum. His vital signs were normal. He was not known to have previous hypertension, diabetes, or other cardiac illnesses, so aggressive parenteral hydration concurrently with the request of basic laboratory and radiological tests was performed. Laboratory tests showed no gross abnormalities beside a slight elevation of his hematocrit level that was attributed to the dehydration status.Abdominal films showed marked dilatation of the small bowel loops on the supine film and scant air fluid levels on the upright position .With previous virgin abdomen and with no obvious hernia, CT scan with oral and intravenous contrast was requested. It revealed the presence of dilated small bowel loops with edematous walls, mainly occupying the upper and mid-abdomen. Collapsed distal small bowel loops and normal colonic diameter were also noted. A possible transition area in the small bowel loops was appreciated without obvious intra- or extraluminal pathology .Computed tomogram of the chest showed as well the presence of large mass occupying lesion localized in the upper lobe of the left lung and encasing the origin of the left pulmonary artery .A nasogastric tube and a urinary catheter were inserted and accurate intake and output measurement were instituted. Resuscitation was carried out for 24 hours. A decision to take him to the operating room was made, and due to the moderate distention of the abdomen, an attempt to proceed with minimal invasive approach was contemplated.Extra caution was used while insufflating the abdominal cavity using a classical supraumbilical approach to prevent an inadvertent injury to the dilated small bowels. Laparoscopic exploration was made through a 5 mm 30 degree scope introduced through a 5 mm trocar. It revealed the presence of significant dilatation of small bowel loops. No disseminated disease or liver involvement was notified. Assisted by another two trocars inserted in the left lower quadrant and mid-way between the umbilicus and symphysis pubis, and helped by a reversed Trendelenburg position, the bowel was traced from the duodenojejunal junction downward. About 75 cm from the ligament of Treitz, a constricting whitish nodule was seen obstructing. Collapsed distal loops and a normal caliber colonic frame were noted .The collapsed bowel distal to the nodular area was gently grasped using a nontraumatic grasper. The umbilical port site was enlarged to about a 4 cm incision, and the diseased bowel segment was exteriorized through the skin . A 5 cm The minilaparotomy was closed tightly after reintroduction of the bowel in the peritoneal cavity and the abdomen was reinsufflated again to assure normal configuration of the abdominal contents. Total operative time was around 40 minutes. The patient made an uneventful postoperative recovery except for superficial umbilical site wound infection and was able to regain normal bowel habits as soon as the second postoperative day.Histopathology and immunohistochemistry proved to be squamous cell cancer. Napsin A test was negative and favors the metastatic deposit to be of laryngeal origin .Laryngeal cancer contributes to about 2% of malignant fatalities. There was a slight increase in its incidence due to tobacco-alcoholic abuse in both genders .Squamous cell pattern constitutes the vast majority of laryngeal carcinomas. The main modality of disease spread is made via lymphatic pathways especially in the supraglottic location of the disease that is richly invested by lymphatic channels which crosses the midline, in contradistinction to the glottic and infraglottic counterparts. Hence, supraglottic cervical lymph node metastasis can be seen in up to 40% of the cases. Early glottic cancer carries a good prognosis, with 3-year tumor-free survival reaching 90%. On the other hand, advanced supraglottic 3-year tumor-free survivals is estimated to be around 40% .Distant metastasis to other organs is made via hematogenous spread. Several hypotheses were raised to explain very atypical sites of metastasis that are away of the norm of a specific tumor spread, among these a possible alternate pathway of spread induced by surgery or radiation therapy, but none of these could have been proven. The most common organ involved by distant metastasis from laryngeal primary is the lung. In addition to sites like liver and bone, unusual sites of sporadic distant metastasis were reported in the literature to involve the skin , forearmSmall bowel involvement by neoplastic secondaries occurs rarely, but still it outnumbers primary small bowel tumors. It can manifest itself through gastrointestinal bleeding , perforaA recent review of all published cases of SCC from head and neck cancer sites by Dwivedi et al. revealed only 12 such cases, of which only nine cases were derived from a laryngeal primary neoplasm. In their review they described three cases in which a laryngeal SCC metastasis caused intestinal obstruction, two cases of bleeding or melena, one case of biliary obstruction, and three cases of small bowel perforation .Of interest, intestinal obstruction used to be a relative contraindication for laparoscopic surgery mainly due to the loss of the virtual space that is usually created by the institution of pneumoperitoneum. In case of obstruction, this extensible space will be occupied by the distended obstructed bowel loops. Another hazard that made minimal invasive surgery shies away from treating intestinal obstruction is the higher probability of iatrogenic small bowel injuries, due to adhesions from previous operations, which constitute the most common cause of small bowel obstruction. Recent research done about the feasibility of laparoscopy in the management of small bowel obstruction concluded at the safety of the modality if it is carried out by experienced hands .The specificity of this case comes from two distinctive facts. The first is being the rarity of metastatic laryngeal primary to manifest itself as isolated complete small bowel obstruction. The second fact was the ability to manage the condition using minimal invasive techniques, the thing that reflected positively on the recovery of the patient with subsequent earlier return to his preoperative condition.Small bowel obstruction, especially with a negative previous abdominal surgical history and in a patient with known malignant disease elsewhere in the body, should raise the suspicion that both conditions could be related.


Acute appendicitis is a commonly diagnosed surgical problem in the pediatric population. Arterio-venous malformations (AVM) of the colonic tract are rarely reported in the pediatric literature. A 13-year old boy who presented with acute appendicitis with concurrent cecal AVM is reported in whom appendectomy was done. Later on radiological investigations AVM was confirmed. A 13-year-old male presented with moderate to severe intensity episodes of abdominal pain for 2 months. The pain became localized to the right lower quadrant over a period 3 days. It was associated with fever and chills. He also had frequent episodes of diarrhea. On examination he had pulse of 101, blood pressure 113/75mmHg, and respiratory rate of 20/min. There was tenderness in right iliac region. Routine laboratory tests were within normal limits. CT scan showed a thickened appendix with associated lymphadenopathy. The patient was planned for a laparoscopic appendectomy. Intraoperatively a pulsatile mass was noted on the cecum and proximal ascending colon . Patient underwent appendectomy. Histopathology of the specimen was consistent with acute appendicitis. The AVM noted intra-operatively was not addressed during the initial procedure due to lack of informed consent, and it was not emergent.Postoperatively, patient underwent a CT angiography, which showed simultaneous filling of the ileocolic artery and ileocolic vein, suggesting an AVM . The patient had an unremarkable postoperative course and was planned for limited right hemicolectomy but parents did not return for the surgical procedure.Acute appendicitis is a common condition in the pediatric population, and the diagnosis of which is usually made on clinical grounds. The need for imaging modalities becomes necessary when clinical signs are not obvious. On the contrary, a diagnosis of colonic AVM is challenging. AVMs are due to abnormal connections between veins and arteries, bypassing the capillary bed. The absence of capillaries coupled with shunted blood flow, often results in local tissue ischemia. Additionally, arteriovenous malformations progressively enlarge with time due to an absence of the dampening effect that the capillary beds provide. Ultimately this malformation will form a tangled mass of fragile vessels known as a nidus.[3] The process by which AVMs develop is not clear despite extensive research. A large proportion of AVMs are asymptomatic but they can manifest with a myriad of symptoms and imaging is needed for diagnosis. Delay in diagnosis is common.Pediatric patients with suspected AVMs may present with intestinal obstruction, diarrhea, pain, emesis, and/or bleeding.[4] A similar case has been reported by Goldstein et al in 2005.[5] In their patient, appendicitis was imitated by an inflammatory reaction and omental adhesions causing fever and tenderness in right iliac region due to a cecal arteriovenous malformation. However, there is no indexed case of an AVM found with a presentation of acute appendicitis.Source of Support: NilConflict of Interest: None declared


Growing teratoma syndrome (GTS) is a rare clinical entity, which presents with enlarging teratomas masses of the retroperitoneum or other locations, occurring during or after systemic chemotherapy for the treatment of nonseminomatous germ cell of the testis (NSGCT), with normalised tumour markers. Awareness of this syndrome is necessary in order to prevent unnecessary chemotherapy and allow optimal management. Prognosis is excellent after the excision of these tumors, but surgery has to be as complete as possible. Surgical resection of bulky GTS lesions is technically challenging; intraoperative complications may occur; that is, why the treatment must not be delayed. Our experience in the surgical management of these lesions is reviewed in the following work. Growing teratoma syndrome (GTS) is a rare condition related to both testicular and ovarian carcinoma. The incidence of GTS after testicular NSGCT is 1.9–7.6%, while in the setting of ovarian germ cell neoplasia is unknown , the defA 23-year-old man underwent right orchiectomy and four cycles of bleomycin, etoposide, and platinum chemotherapy at another hospital for T2 mixed NSGCT . The initial clinical stage of disease was IIC. He was classified, according to International Germ Cell Cancer Collaborative Group (IGCCCG) classification, as the intermediate prognostic group.α-fetoprotein was 2.1 ng/mL, lactate dehydrogenase was 122 IU/L, and beta subunit of human chorionic gonadotropin was 0 mIU/mL. The tumor was completely resected  scan revealed a giant mass with a diameter of 21 cm which displaced the inferior vena cava, right kidney, and left psoas muscle. MRI examination was performed in order to characterize the mass better and its relationship to adjacent structures. MRI images showed no infiltration IVC . His α-fresected . Surgeryα-fetoprotein, lactate dehydrogenase, and beta subunit of human chorionic gonadotropin levels were 2372 ng/mL, 216 IU/L, and 395 mIU/mL. The chest radiography shows metastatic lesions. Abdominal CT evidenced giant retroperitoneal tumor from the renal vessels to the left iliac vessels. The clinical stage was IIIA for cervical lymph nodes. He received three cycles of bleomycin, etoposide, and platinum chemotherapy until normalization of serum tumor markers. CT scan showed an increase in tumor size (30 cm) after five months from chemotherapy  without adjuvant therapy, six months later, started having gastrointestinal symptoms, weight loss, and lower back pain as well as abdominal mass. Workup revealed that otherapy  and chesotherapy . Closed The etiology of GTS is unknown but there are two must-quoted hypotheses: chemotherapy cures immature malignant cells but remains untreated and grows the mature benign teratomatous elements; chemotherapy alters the cell kinetics toward transformation from a totipotent malignant germ cell toward a benign mature teratoma .3/month, though the growth patterns are variable [While the growing teratomas are considered benign, they have rapid expansion with median linear growth of 0,5–0,7 cm/month and volume increase of 9,2–12,9 cmvariable . Their bThis syndrome has been reported in the retroperitoneum (the most common site), lung, cervical lymph nodes, mediastinum, supraclavicular lymph nodes, inguinal lymph nodes, forearm, mesentery, liver, and pineal gland , 7.According to André et al. , there aSurgical resection is currently the gold standard treatment for GST, since teratomas are resistant to chemotherapy and radiotherapy .Two described cases are bulky teratomas (defined as >10 cm)  with higVarious series supported that surgical treatment is curative and the local recurrence is lower when the tumor is completely removed , 8, 9. LData reported into two large studies, by Spiess et al., the 5-year overall survival were 89-90% in patients who underwent complete resection , 8.Patients with advanced germ cell tumors should receive coordinated care by treating urologist and oncologist to recognize promptly GST and to achieve good outcomes. Complete surgical treatment is recommended, even if it is technically challenging, to avoid mechanical complication and malignant transformations. In cases of bulky teratomas, surgery should be performed at a highly specialized center with a skilled surgeon.


A 3-day-old male child was brought to the hospital with complaints of abdominal distension and a mass in the upper abdomen causing respiratory difficulty. Child underwent exploratory laparotomy and a large multicystic mass arising from postero-inferior wall of the stomach along its greater curvature was excised and stomach repaired. On histopathology it came out mature gastric teratoma. Gastric teratoma is an extremely rare neoplasm, accounting for less than 1% of all teratomas occurring in infancy and childhood [1]. The first case of gastric teratoma was reported in 1922 by Eustermann and Sentry, since than only 102 cases have been reported in the literature [2]. They usually present as a palpable mass in the epigastrium and left abdomen with abdominal distension. Because of rarity of the tumor, we are reporting this case with review of literature.A 3-day-old male child (weight 2.9 kg) was brought to the hospital with complaints of abdominal distension and a mass in the upper abdomen noticed by them just after birth. Patient was in respiratory distress because of large size of tumour. The child was born full term by normal vaginal delivery and cried immediately after birth. The patient tolerated breast feeds without any vomiting. There was no history of exposure to any drugs or radiation to the mother in the antenatal period. Initial abdominal examination showed fullness in the left upper abdomen and a large, irregular firm mass at the left hypochondrium and epigastric region leading to massive abdominal distension.Hematological profile revealed hemoglobin of 15.0 g/dL and total leukocyte count of 14,500/cm3. Kidney function tests, liver function tests were within normal limits. X-ray abdomen showed a mass effect in the left hypochondrium with diffuse calcifications. Ultrasonography revealed a multicystic mass with mixed echogenicity in left upper abdomen, origin of which could not be appreciated, displacing the left kidney downwards. There was no free fluid in the peritoneal cavity. Computed tomography demonstrated a large lobulated heterogeneous mass with solid and cystic components with calcification and inferiorly reaching upto pelvis, displacing gut loops .The child was taken up for surgery. Exploratory laparotomy revealed a large multicystic mass arising from postero-inferior wall of the stomach along its greater curvature . Greater part of the mass was lying outside the stomach while a small part was extending into the lumen of the stomach. The mass was excised in toto with a small fringe of the gastric wall from which the lesion originated. The stomach was repaired in layers. The excised specimen measured 13x10x8 cm. Cut surface revealed large cystic areas with solid areas. Solid tissue was composed of greyish white areas with foci of cartilage and bone . Histopathology report was mature gastric teratoma.The most common site of teratoma in infancy and childhood is sacrococcygeal teratoma (60-65%), gonadal (10-20%), mediastinal (5-10%), presacral (5%) and rarely intracranial, retroperitoneal and cervical [1].Gastric teratomas can be mature and immature based on the presence and differentiation of neuroglial tissue. Mature gastric teratomas contain mature glial tissue along with other derivatives of all germinal layers. Mature gastric teratomas are considered benign tumours, whereas, the malignant potential is present in immature gastric teratoma. The term ‘malignant teratoma’ is restricted to the endodermal sinus tumor (EST) or yolk sac tumor and choriocarcinoma. Almost all reported gastric teratomas have been mature and benign with only two cases of malignant transformation reported so far [3].Majority of gastric teratomas are exophytic (>60%), endophytic growths are present in about 30% of cases, mixed exophytic and endophytic growths are present in only few cases. Although gastric teratomas can arise from any part of the stomach, common sites are the lesser curvature of stomach, antrum and fundus of stomach along the posterior wall. Some of these tumours are pedunculated .These large tumours presenting in the newborn may cause premature labor or dystocia. Respiratory difficulty is also common caused by upward displacement of the diaphragm by the tumour. Some infants have vomiting, haematemesis or malena especially those with endogastric component causing ulceration of overlying mucosa [6].In most of the cases the preoperative diagnosis of gastric teratomas is difficult. Abdominal radiograph, ultrasonography, CT/MRI and endoscopy are important diagnostic tools. Plain films usually reveal a soft tissue mass with associated irregular areas of calcifications. US demonstrate a heterogeneous mass with mixed echogenicity. CT scan demonstrates a mass with solid and cystic components and internal calcifications and fat. CT scan is the modality of choice. When combined with intravenous and oral contrasts, they can detect the origin of the tumour, its relation with gastrointestinal tract and major blood vessels, presence of bones and calcifications, and tumour extent and presence of metastasis. Other modalities like barium meal and gastroscopy has a limited role in the diagnosis of gastric teratoma [7].Surgical excision is the treatment of choice. Depending upon the extent of tumour partial, subtotal and total gastrectomy has been done. Prognosis following surgical excision has been excellent with report of one case of recurrence [8].Source of Support: NilConflict of Interest: None declared


Schwannoma is a benign nerve sheath tumor composed of schwann cells. Oral cavity is a rare site for schwannomas, tongue being the most common location. Here we are presenting a case of a young adult who presented with a huge swelling in the tongue which was removed by mandibulotomy approach and pre-operative tracheostomy. A 22-year-old male patient presented to the outpatient department with a history of swelling in the tongue for the last 4 years with progressive difficulty in swallowing food and change of voice over the last few months. Upon examination a large swelling was observed on the posterior part of the tongue compromising the oropharyngeal inlet. The approximate size of the swelling was 5cmx4cm. After proper clinical evaluation the patient was advised to obtain a magnetic resonance imaging (MRI) study of the oral cavity, which showed it to be a nerve sheath tumor (Schwannoma) originating from the hypoglossal nerve branch. The patient was admitted for surgery. As difficult intubation was anticipated, pre-operative tracheostomy was performed.The tongue mass was approached by right paramedian mandibulotomy using a transcervical lip split incision. Post operative histopathological examination of the removed specimen showed hypercellular ‘Antony A’ area with plump spindle cells and hypocellular ‘Antony B’ area in a Hematoxylin & eosin stain (200x). This confirmed the diagnosis for a schwannoma.As each and every case is unique in its presentation, so is the management. The idea of presenting the above case is to emphasise the role of selection for the proper approach and foresee the preventable complications while working around the airway. .


Hypergastrinemia and the resultant peptic ulcer related to an enteric duplication has been quite rarely reported in the literature.We herein report the case of a 4-year-old girl who presented with hypergastrinemia and a duodenal ulcer at 2 years of age. She had been followed up with a proton pump inhibitor, which resulted in resolution of the ulcer; however, unexplained hypergastrinemia had continued. A cystic lesion at the antrum was discovered at 4 years of age, which we suspected to be a gastric duplication. After we resected the lesion, the hypergastrinemia resolved without recurrence of the duodenal ulcer. The histology was compatible with a gastric duplication, and the lumen was lined with antral mucosa that strongly stained positive for gastrin. We presumed that the antral mucosa inside the duplication in our case had no hydrogen ion feedback inhibition of gastrin release from gastrin cells and increased release of gastrin from the mucosa inside the duplication led to the duodenal ulcer. Only two cases have been reported in the literature that had hypergastrinemia related to enteric duplication.Gastric duplication should be included in the differential diagnosis of sustained hypergastrinemia in children. Gastric duplications occur infrequently in children and most of the cases may become symptomatic as an abdominal mass, gastric outlet obstruction, or gastrointestinal hemorrhage . We hereA 4-year-old girl with unexplained hypergastrinemia was referred to our department after abdominal ultrasonography detected a cystic lesion at her prepyloric antrum. When she first presented with a duodenal ulcer at 2 years of age, her serum gastrin levels had ranged between 700 and 1000 pg/ml. The imaging studies with a contrast-enhanced computed tomography (CT) scan had repeatedly revealed no tumor around the pancreas or duodenum suggestive of gastrinoma but revealed only a thickened wall of the duodenum potentially due to the initially severe duodenal ulcer. She had continuously taken a proton pump inhibitor (PPI) until the referral since the first presentation of the duodenal ulcer, which had relieved its symptoms such as epigastric pain.Helicobacter pylori. According to these findings, we suspected gastric duplication. As we speculated that the lesion might have caused the duodenal ulcer in this patient, we proceeded with operative treatment for the lesion. The operation was performed with the umbilical approach. A 2 × 3 cm protruded lesion was observed on the anterior wall of the gastric antrum . Upper gastrointestinal series revealed a 2.5 × 5 cm cystic lesion on the anterior wall of the antrum, communicating with the gastric lumen  to stimulate the release of somatostatin, thereby preventing gastrin release from gastrin cells (G cells) by a paracrine mechanism. When gastric antral mucosa is isolated from its usual acidic environment, such as in cases of a retained gastric antrum following partial gastrectomy with Billroth II reconstruction, there is no hydrogen ion feedback inhibition of gastrin release, leading to hypergastrinemia followed by increased gastric acid production, recurrent ulceration, and bleeding . We specAlthough the time course of the serum gastrin level of the latter patient was not described, that of the former patient normalized promptly after the operation. In contrast, the reason why hypergastrinemia in our case did not improve until PPI and famotidine were stopped may be explained by another mechanism related to the preoperative long-term use of PPI. A modest increase in the serum gastrin level in adult patients under a long-term treatment with acid-lowering compounds, especially PPI, has been reported . PPI cauAccording to previous reports , 7, we sIn conclusion, gastric duplication should be included in the differential diagnosis of sustained hypergastrinemia in children. In addition, clinicians should be aware that the long-term use of PPI may also cause hypergastrinemia.