We are reporting a case of a 91-year-old male with a primary malignancy of the right parotid gland with radiographic thrombus extension within the right external jugular vein. He was treated with palliative radiation therapy to the right parotid mass with a marked clinical response. The rarity of this occurrence as documented in the review of the literature provides for uncertainty with regard to proper management. Radiographic evidence of thrombus in the absence of clinical manifestations, the role of anticoagulation, and the proper radiation target delineation were all challenges encountered in the care of this patient. Our case represents a rare occurrence with unique radiologic findings that has implications for management. We are reporting a case of a 91-year-old male with a primary malignancy of the right parotid gland with thrombus extension within the right external jugular vein. He was treated with palliative radiotherapy to the right parotid mass with a marked clinical response. Tumor thrombus within neck veins is an uncommon event and in the head and neck it has most often been reported in association with thyroid malignancies . ThrombuA 91-year-old male with no smoking, drinking, or prior malignancy history was initially referred to the Department of Radiation Oncology for palliative irradiation to a growing painless right parotid mass that had been present for one year.One year prior to presenting to our clinic, the patient had noticed a right neck mass. CT of the neck demonstrated an unencapsulated, poorly defined hypodense mass measuring 2.3 × 1.5 × 2.1 cm in the superficial portion of the right parotid gland with fine needle aspiration (FNA) suggesting Warthin tumor. Further management was complicated by a stroke that left him wheelchair bound with aphasia. CT of the neck one year later noted the mass to have increased in size to 8.8 × 6 × 4 cm with no evidence of associated thrombus.Upon presentation at our institution, physical examination demonstrated significant enlargement in the size of the mass with no evidence of swelling, palpable veins, or tenderness in the neck. FNA of the mass showed pleomorphic malignant cells suggestive of a high grade carcinoma . CT of tThrombus associated with malignancy can result from either tumor compression of a vein leading to stasis or direct extension of the primary tumor. Clinically this is important to distinguish when determining either the appropriate extent of surgical resection or radiation target volume delineation. Direct tumor extension into an adjacent vein from a head and neck malignancy is rare and was first reported by Kaufmann in 1879. Since then, less than 20 cases have been reported associated with thyroid cancer , 5, 7, 8There are reports of increased FDG avidity in an intravenous tumor thrombus from a thyroid carcinoma . HoweverOur case illustrates several interesting radiological findings that have implications for management. The first being the extensive nature of the thrombus associated with the tumor extending down the length of the right external jugular vein to the level of the right subclavian vein. Although in our case anticoagulation was not given, the decision to give anticoagulation should be carefully considered particularly with malignancy related hypercoagulable states. The long time course from initial detection to initiation of therapy adds ambiguity to the nature of the thrombus. During that time, either tumor extended into the vessel or prolonged stasis allowed blood thrombus formation.The uncertainty of the nature of the thrombus with regard to whether it represented tumor or simple blood thrombus presents further therapeutic challenges, and the fact that it showed FDG avidity on PET adds another layer of opacity. As noted previously, there have been reports in the literature of increased avidity not being conclusive of tumor in a thrombus, as was in our case. Caution should be exercised when making treatment decisions solely based on increased FDG avidity. If doubt exists about the nature of the thrombus and the site in question is readily accessible, pathologically sampling could greatly aid in guiding the correct therapeutic intervention. In our case, FNA demonstrated lymphocytes, which were more consistent with blood thrombus than tumor extension. If a FNA is not feasible, a magnetic resonance imaging (MRI) scan may be useful in distinguishing between tumor and blood thrombus. Lastly, correct target delineation corresponding to the known tumor is critical in delivering safe and effective radiation therapy. If the thrombus had been treated as if it contained tumor, a far larger area would have been irradiated with minimal to no increase in tumor control or palliation of symptoms. Furthermore, increased amounts of normal tissue would have been irradiated leading to increased acute and late radiation toxicities. Our case represents a rare case with unique radiologic findings that has implications for management.


Complete abdominal wall infiltration with neoplastic gastrocutaneous fistula is an unexpected and out of the ordinary presentation of locally advanced gastric cancer. It is very rare to encounter case reports presenting diffuse abdominal wall invasion, but a complete parietal destruction is an exceptional event.en bloc gastric resection including colon and abdominal wall, followed by a parietal reconstruction through positioning of prosthesis and reverse abdominoplasty.Here we describe the case of an 81-year-old Caucasian woman presenting a carcinoma perforating her anterior gastric wall and infiltrating all layers of her abdominal wall. The gastric tumor infiltrated her transverse mesocolon, the rectus abdominis muscles bilaterally and overran them anteriorly, causing a large parietal deficit and a complete external fistula. Treatment consisted of a complex surgical procedure requiring general and reconstructive surgery cooperation in order to perform an Clinical presentation, histology and therapeutic options are discussed. The importance of a multidisciplinary approach when encountering extremely rare clinical presentations is emphasized.The online version of this article (doi:10.1186/1752-1947-9-13) contains supplementary material, which is available to authorized users. Gastric adenocarcinoma is one of the main causes of cancer-related mortality –4. In soAn 81-year-old Caucasian woman with a medical history of hypertension and cholecystectomy for perforated acute cholecystitis was admitted to a community medical ward. At the time, she presented with asthenia, weight loss, occasional vomiting, abdominal pain and anemia. At clinical examination a parietal swelling was observed and at surgical consultation an incisional hernia through the previous cholecystectomy incision was suspected. A gastroscopy revealed the presence of a neoplasm shrinking the gastric antrum. In the following days a small cutaneous ulcer above the parietal swelling was observed followed by the appearance of a fistula a few days later. After surgical consultation she was moved to our university surgical ward.Our laboratory tests revealed hypoproteinemia (4.6g/dL), hypoalbuminemia 2g/dL) and anemia (8.3g/dL) which required transfusion of three blood units. A computed tomography (CT) scan  , 14 rathIn advanced gastric cancer possible therapeutic options include neoadjuvant chemotherapy with gastrostomy or jejunostomy. However, although evaluated by a multidisciplinary team, such a standard approach would not have been feasible in our case due to the fact that the huge parietal defect, the perforation, the stenosis and, above all, the transverse colon involvement represented a contraindication to performing either a gastrostomy or a jejunostomy. The massive cancer infiltration of the wall and surrounding organs, without great vessels infiltrations, led us to perform a gastric resection including transverse colon, rectus abdominis muscles and skin.The large parietal defect was reconstructed by positioning a biological prosthesis which is the gold standard procedure when tissue contamination is present or high risk of anastomotic dehiscence exists .Full-thickness abdominal defects following oncological resection are considered a reconstructive challenge, especially when wide excision margins are required to avoid cancer recurrence. Most advantageous reconstructive options may involve local, pedicled or free flap transfer . Local fPedicled superior or inferior rectus abdominis flap or extended latissimus dorsi flap can be used for upper abdominal wall reconstruction. In some cases , free tissue transfer may be required for reconstruction . AlthougReverse abdominoplasty may represent a valuable alternative to pedicled or free flap reconstruction due to its recruitment of the adjacent abdominal tissue into the defect . The infBased on our experience, we can therefore consider reverse abdominoplasty a valuable surgical option when confronting extraordinary parietal destruction as it enables the surgeon to create a skin cover for an abdominal wall defect. Considering that our patient was elderly with skin laxity, we were able to obtain a large inferior abdominal flap by employing this technique.The above described clinical presentation of gastric cancer is quite rare and even rarer in the Western hemisphere. Proper management of gastric cancer requires a multidisciplinary approach involving the oncologist, surgeon and radiation oncologist. This is particularly paramount when gastric cancer has an unfamiliar manifestation such as in our reported case. When such rare cases are presented, an even larger pool of specialists has to be involved in the management of the disease. In our experience we benefited from involving a plastic surgeon in our integrated approach to patient treatment.en bloc resection of the tumor burden including stomach/colon/abdominal wall), our approach was to frame the reconstructive issue, which was carried out by implementing adequate surgical techniques and devices. In conclusion, as our case study attests, we are convinced of the utility and importance of a multidisciplinary approach when confronted with highly abnormal cancerous growths. We would like to cite the additional file at the end of the case presentation as summary of the all the clinical history.Although the reported case had an unsatisfactory outcome and adversely ended with the death of the patient, it is worth highlighting that this was not due to surgical complication. The measurement of IAP has been incorporated routinely in critical units in order to monitor and control those clinical situations that make us suspicious of intra-abdominal hypertension (IAH). Abdominal compartment syndrome (ACS) and IAH are increasingly recognized as potential complications in patients in intensive care units. ACS and IAH affect all body systems, most notably the cardiac, respiratory, renal, and neurologic systems. ACS/IAH affects blood flow to various organs and plays a significant role in the prognosis of the patients. Recognition of ACS/IAH, its risk factors and clinical signs can reduce the morbidity and mortality associated . We usedWritten informed consent was obtained from the patient’s next-of-kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.Absence of genetic history; absence of environmental and lifestyle influences.(DOC 36 KB)Additional file 1:


Mixed mucinous cystadenoma with serous cystadenoma of the pancreas is rare. There have been only two previous case reports in the English-language literature. We present a case of a 46-year-old woman who was diagnosed with mixed mucinous cystadenoma with serous cystadenoma of the pancreas. Computed tomography and magnetic resonance imaging showed a cystic neoplasm in the dorsal/proximal body of the pancreas with a clear-margin multilocular cavity and enhanced internal septum. The patient underwent laparoscopic central pancreatectomy. The diagnosis of mixed mucinous cystadenoma with serous cystadenoma of the pancreas was confirmed by pathological examination. The patient was followed up for 3 months and there were no signs of recurrence, or pancreatic exocrine or endocrine insufficiency. To the best of our knowledge, this is the first reported case treated by laparoscopic central pancreatectomy. Pancreatic cystic lesions are being detected with increasing frequency due to high-resolution imaging. The prevalence of incidentally detected pancreatic cysts on magnetic resonance imaging is 13.5%, and increases with age , 3. We pA 46-year-old woman was admitted to our department because of epigastric dull pain for 1 year. She had no fever, no nausea or vomiting, no diarrhea or melena, and no weight loss. Her past medical and surgical history was unremarkable. The physical examination found no abnormalities. The laboratory tests, including tumor markers (carcinoembryonic antigen (CEA), alpha fetoprotein, carbohydrate antigen 19–9, carbohydrate antigen 724 and carbohydrate antigen 242) were all within normal limits. Computed tomography and magnetic resonance imaging disclosed a 10 × 14-mm cystic neoplasm in the dorsal/proximal body of the pancreas with a clear-margin multilocular cavity and enhanced internal septum  using a Veress needle. One initial 10-mm trocar was placed for the laparoscope below the umbilicus. A 30° telescope was inserted to examine the peritoneal cavity to rule out metastasis disease. After general examination, the other four trocars (one of 12 mm and three of 5 mm) were inserted into the left upper flank, left flank, right upper flank and right flank quadrants; the five trocars were arranged in a V-shape . The mobilization of the pancreas began at the superior border until the common hepatic artery and the proximal splenic artery were visible Figure A. Then tNext, an end-to-side pancreaticojejunostomy was performed , 5. A roThe operative time was 240 min and the blood loss was 50 ml. The postoperative course was uneventful. The patient started to take in semi-fluid on day 3 after surgery. The patient was discharged on postoperative day 8. She was followed up 3 months later and there were no signs of recurrence, or pancreatic exocrine or endocrine insufficiency.The final pathological diagnosis confirmed mixed MCA with SCA of the pancreas. The gross finding was a 15 mm × 15 mm round cystic mass Figure .Figure 5et al. [et al. [The concurrent occurrence of SCA and a variety of primary pancreatic neoplasms, such as pancreatic neuroendocrine tumor, ductal adenocarcinoma, neuroendocrine carcinoma or intraductal papillary mucinous neoplasm, has been reported –9. MCA het al.  reported [et al.  reportedet al. [et al. [To date, the origin of mixed MCA with SCA of the pancreas is still unclear. MCA has been thought to arise from duct cells, whereas SCA has been thought to arise from centroacinar cells . Abe et et al.  and Bogo [et al.  reported [et al. . Therefo [et al. .The American College of Gastroenterology practice guidelines published in 2007 propose that mucinous lesions are characterized by a macrocystic lesion containing viscous fluid rich in CEA, whereas serous lesions have the characteristics of a microcystic morphology with non-viscous fluid and a low level of CEA . Most exFunction-preserving pancreatectomy, especially enucleation and central pancreatectomy, is thought to be an ideal procedure for benign or low-grade malignant tumors . CentralMixed MCA with SCA of the pancreas is very rare, and an accurate preoperative diagnosis is difficult. Being aware of the existence of this disease allows for timely management of the pancreatic cystic neoplasm. To the best of our knowledge, this was the first reported case treated by LCP.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.


In small children, retropharyngeal abscesses usually occur after upper respiratory tract infections. Unlike in adults, these abscesses are difficult to diagnose in small children, and can rapidly develop into deep neck or mediastinal abscesses.A 2-year-old Japanese boy recently presented to our department with a chief complaint of neck swelling. Physical examination revealed bilateral tonsillitis and swelling of the left posterior pharyngeal wall. Emergency neck computed tomography angiography showed a contrast-enhanced abscess cavity posterior to the left retropharyngeal space, and a low-density area surrounded by an area without contrast enhancement in the posterior neck. The latter was suspected to be a deep neck infection secondary to a retropharyngeal abscess. After surgery, the patient was diagnosed with a retropharyngeal abscess and concurrent cystic lymphangioma. The lesions improved after intraoral incision and drainage, and administration of antibiotics.Lymphangiomas and retropharyngeal abscesses are both known to be more common in children than in adults. However, we found no other reports of concomitant presentation of lymphangioma and retropharyngeal abscess in the literature. Retropharyngeal abscesses occur more often in children than in adults, and are not rare . AlthougStreptococcus pneumoniae (PRSP). We also percutaneously punctured the mass on the posterior neck, and drained approximately 10 mL of clear yellowish lymph, but no pus. Rapid cytology showed that this fluid contained mostly protein-like substances and lymphocytes. The mass was no longer palpable after drainage, and no further surgery was performed. The patient was placed under observation with postoperative administration of meropenem (450 mg) and clindamycin (150 mg). On postoperative day 3, his laboratory test results showed improvement, and the antibiotic therapy was changed to ceftriaxone sodium hydrate (700 mg) based on the sensitivities of the cultured organisms. There were signs of improvement in the pharynx, but the mass on the posterior neck had recurred. Repeat neck CT angiography showed a low-density area in the posterior neck, and signs of improvement in the posterior pharyngeal wall. Subsequent magnetic resonance imaging (MRI) angiography showed an area with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, and an area of low signal intensity surrounded by an area of high signal intensity on post-gadolinium images  angiography showed an enhanced abscess cavity posterior to the left retropharyngeal space, and a low-density area surrounded by an area without contrast enhancement in the posterior neck , Staphylococcus aureus (13%), Candida (6%), and Haemophilus influenzae (3%). The proportion of cases with S. aureus infection was particularly high in children aged less than 1 year. The rates of detection of methicillin-resistant S. aureus are increasing. Many patients have also been shown to have mixed infections. Brook [S. aureus, and H. influenzae. Studies by Asmar [et al.[Because retropharyngeal abscesses are often accompanied by upper respiratory tract infections, the microorganisms isolated from these abscesses are often similar to those isolated from patients with upper respiratory tract infections. In a study by Hoffmann et al.,  the bacts. Brook  performeby Asmar  and Crair [et al. had simir [et al..Lymphangiomas are uncommon lesions of the lymphatic channels, and are often present at birth and usually diagnosed during childhood, mostly before the age of 2 years. Diagnosis is by MRI and fine-needle aspiration cytology findings . In the Lymphangiomas and retropharyngeal abscesses are both known to be more common in children than in adults. However, we found no other reports of concomitant presentation of lymphangioma and retropharyngeal abscess in the literature. Concomitant occurrence of these conditions may increase with increasing age.We reported here a case of retropharyngeal abscess caused by PRSP in a young child who presented with neck swelling. Investigation revealed a retropharyngeal abscess and an enlarged cystic lymphangioma secondary to the abscess. After intraoral surgical incision and drainage and administration of antibiotics, the patient recovered uneventfully.Written informed consent was obtained from the patient’s parents for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.YH and HF were the main consultant surgeons involved in the management of the patient. YH reviewed the literature and wrote the manuscript. TH was involved in caring for the patient in hospital and contributed the case history notes used in this report. All authors read and approved the final manuscript.


Paragangliomas are neuroendocrine tumors that arise from sympathetic nerve ganglia. They can develop anywhere from the neck to the pelvis, but are most commonly found in the abdomen, particularly at the aortic bifurcation or in the periaortic region. Malignant paragangliomas account for 29–40% of cases. We report a case of 36-year hypertensive female presented with and right flank pain and accelerated hypertension. On evaluation she was diagnosed to have non unctioning kidney due to malignant pelvic paraganglioma with right ureteric encasement. We believe our case is one of the first reported in literature as rare presentation of malignant paraganglioma presenting as nonfunctioning kidney and accelerated hypertension. Paragangliomas are malignant tumors that arise from the extra-adrenal paraganglionic cells of the sympathetic or parasympathetic systems. They can be observed from the base of the skull to the bladder or along the sympathetic ganglionar chain. Retroperitoneal paragangliomas can represent a truesurgical challenge due to their tight relation to largesize vessels. We present a case of malignant pelvic mass presenting as nonfunctioning kidney and accelerated hypertension. Its final histologic diagnosis was paraganglioma that was functional with metastasis to paraaortic nodes.A 36-year-old hypertensive female on multiple antihypertensive medications presented to our institute with complaints of right flank pain of 2-month duration. No history of paroxysmal hypertension, sweating, headaches, hematuria, dysuria, fever, and bowel complaints was present. There was no significant family history and no history of sudden unexplained death in the family. On general physical examination, pulse was 98/min and BP was 174/90 mmHg. On per abdomen examination no mass was palpable. Serum biochemistry showed serum creatinine of 2.1. Rest of the routine work-up was normal. USG abdomen showed gross hydronephrosis of right kidney with suspected pelviureteric junction obstruction. Renal dynamic scan revealed nonfunctioning right kidney. Patient was admitted with plan for right nephrectomy as cause of accelerated hypertension. However, her blood pressure was uncontrolled. On further evaluation her serum metanephrines levels were found to be 660 pg/mL . Serum normetanephrines levels were normal. CECT abdomen revealed heterogeneously hypodense mass lesion 7 × 6 × 4 cm at the level of L4-5 level with areas of central necrosis and peripheral rim enhancement abutting bilateral common iliac vessels near bifurcation; ureter and adjacent vertebral body multiple enlarged lymph nodes were present in paraaortic and paracaval region. Right kidney was grossly hydronephrotic with thinned out cortex and left kidney showed mild hydronephrosis . 68 Ga Dα and β blocker. Surgical excision of the tumor with involved tissues is the primary treatment of choice because these tumors are commonly chemo- and radioresistant [Pheochromocytomas are tumors that arise from chromaffin cells of the adrenal medulla. They are called paraganglioma if chromaffin-cell tumors originate from extra-adrenal sites along the sympathetic and/or the parasympathetic chain . Paraganesistant . Patientesistant . Severalesistant . All paresistant .Patient who present with nonfunctioning kidney and accelerated hypertension should be worked up to rule out other systemic causes which may be missed if work-up is not done. As was our case, patient was uncontrolled on four antihypertensive medications, and on work-up she was found to have pelvic paraganglioma. Histopathology revealed malignant paraganglioma with metastasis in paraaortic lymph nodes. Patient was followed up with 68 Ga DOTATATE PET CT at three months which showed no somatostatin receptor uptake in the pelvis and rest of the body, suggesting no recurrence or metastasis. Patient is started on adjuvant chemotherapy cyclophosphamide, vincristine, and dacarbazine. Genetic testing is being planned for the patient as well as family members in the follow-up period.


Linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. Patients often present with neurologic symptoms. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. Infratentorial lesions and other lesions not closely associated with the overlying scalp abnormality, such as those found in the cerebellum, have been reported, but are extremely uncommon. We present a case of an 8-year-old boy with a left fronto-parietal “en coup de sabre” scalp lesion and describe the neuroimaging findings of a progressively enlarging left cerebellar lesion discovered incidentally on routine magnetic resonance imaging. Interestingly, the patient had no neurologic symptoms given the size of the mass identified. Linear scleroderma “en coup de sabre” (ECDS) is a rare subset of localized scleroderma. Affected individuals typically have a characteristic atrophic skin lesion involving the fronto-parietal scalp. The disease usually has a benign course and has been distinguished in the past from systemic scleroderma by lack of significant internal organ involvement . HoweverA 5-year-old boy was referred for routine brain MRI by the department of neurosurgery after presenting for consultation in the setting of his primary illness, linear scleroderma ECDS, affecting his left fronto-parietal scalp. The patient was initially diagnosed with linear scleroderma at that time on the basis of physical exam findings. The diagnosis was confirmed via a biopsy of his left fronto-parietal scalp. He subsequently received treatment with methotrexate weekly for 22 months. Prior to his diagnosis of linear scleroderma ECDS, the child had no other relevant past medical history. At the time of diagnosis, as well as at the time of the first MRI, the patient had no neurologic deficits or neurologic symptoms. On physical examination, the patient had a band-like scalp lesion in the left upper fronto-parietal region consisting of a 7.5 x 2.5 cm atrophic, shiny, pink-white plaque with alopecia and hyperkeratosis. The patient was noted to have a smaller lesion of similar characteristics, anterior and inferior to the large plaque. Both the patient and his mother reported that these lesions had improved with initial treatment and had appeared stable in size since the discontinuation of the medication. Brain MRI was performed using a 1.5- T MR scanner . At the time of diagnosis, imaging demonstrated a single, ill defined, non-enhancing, 10 x 4 mm focus of T2 prolongation in the left cerebellar white matter . On post contrast images, there was mild diffuse leptomeningeal enhancement in the posterior aspect of the left cerebellar hemisphere . Subsequently, a head CT scan demonstrated a focal area of scalp thinning within the left fronto-parietal region with associated flattening and thinning of the underlying frontal and parietal bones. There was no evidence of intracranial calcifications . A follow-up brain MRI was performed 14 months later, at age six, while the patient was on treatment with methotrexate, demonstrated new patchy, large, confluent areas of T2 prolongation in the white matter of the left cerebellar hemisphere without significant mass effect. On diffusion weighted imaging (DWI) these parenchymal abnormalities showed increased diffusivity. Post contrast images demonstrated new scattered nodular foci of enhancement throughout these areas of parenchymal signal abnormality with corresponding susceptibility effect, most compatible with micro-hemorrhages . The degree of leptomeningeal enhancement in the left cerebellar hemisphere had increased since the previous study . The third and most recent brain MRI was performed at eight years of age (twelve months after the prior exam) and demonstrated continued increase in size of the confluent areas of T2 prolongation in the left cerebellar hemisphere . Treatment with methotrexate had been discontinued three months prior to obtaining this most recent brain MRI. These lesions again demonstrated increased diffusivity on DWI . Additionally, new areas of T2 prolongation were identified involving the cerebellar vermis and the medial aspect of the right cerebellar hemisphere. Post contrast images showed more confluent nodular parenchymal enhancement and persistent diffuse leptomeningeal enhancement . Corresponding magnetic susceptibility on gradient-recalled echo (GRE) T2* was present within the nodular foci of enhancement .Linear scleroderma “en coup de sabre” is a rare subset of localized scleroderma, a distinct and separate disease entity from systemic scleroderma . LocalizIn conclusion, typical neuroimaging features of linear scleroderma including atrophy, white matter lesions and calcifications typically involve the cerebral hemisphere ipsilateral to the skin lesion. However, CNS lesions can also present away from the skin lesion in the cerebellar hemisphere with imaging features similar to a demyelinating process or infiltrating tumors, as opposed to the more common presentation of parenchymal atrophy. It is important to recognize that even in the absence of neurological symptoms, patients with linear scleroderma may present with atypical neuroimaging features found on routine imaging which should not be mistaken for an infiltrating neoplasm.


Myopericytoma is a soft tissue tumor with perivascular myoid differentiation. It accounts for 1% of the vascular tumors and involves mostly cutaneous or subcutaneous tissue of the limbs in adults. Malignant myopericytoma is exceedingly rare. A 15-year old girl presented with slowly progressive mass over left shoulder region. Histopathology and immunohistochemistry after complete excision revealed it as malignant myopericytoma. A 15-year-old girl presented to a local clinic with a slowly growing mass over the left shoulder for several months. Her past medical history was unremarkable. On physical examination, a 5 cm x 5 cm mass with relatively soft consistency was found in the left shoulder area. Its borders were relatively distinct and there was no fluctuation. Ultrasound revealed a well-demarcated heterogeneous solid mass (4.4 cm x 5.2 cm). The preoperative diagnosis was lipoma. MRI showed a soft tissue lobulated mass on the superior aspect of supraspinatus muscle . Surgical excision of the lesion was performed. The mass was densely adherent to the surrounding structures and possibility of microscopic residual disease could not be ruled out. Histopathology showed a non-capsulated tumor composed of round to oval cells with eosinophilic cytoplasm, ill-defined cell borders, and myoid features with interspersed areas of closely packed small undifferentiated round cells. Broad zones of geographic pattern coagulative necrosis were seen. There was concentric perivascular accentuation of the cellular proliferation around hemangiopericytoma-like vascular spaces. A mitotic rate of up to 17 mitoses/10 high power fields was seen in the most cellular areas, including atypical mitosis. Immunohistochemically, most of tumor cells showed positivity for smooth muscle actin, vimentin, and CD99 but they were negative for cytokeratin, S100, desmin, and CD34 . Based on these findings the diagnosis of malignant myopericytoma was made. Patient received six cycles of chemotherapy (ifosfamide-etoposide/cyclophosphamide-vincristine-doxorubicin) following which radiotherapy was given by using 18 MV photon of linear accelerator. A 54Gy radiation dose (in 30 fractions) was delivered. MRI examination performed at 18 months after surgery showed no evidence of any residual or recurrent tumor in the left shoulder region.Myopericytoma is an uncommon perivascular benign tumor having diameter often less than 2 cm. It develops as a single, painless, well circumscribed, and slowly growing lump in the subcutaneous tissues. Distal extremity is often involved however this tumor may occur in any anatomic site including neck, left atrium, urinary bladder, arm, thigh, and foot.[1-4] In the index case the left shoulder area was involved. These tumors are rarely reported in children. A slight male predominance is reported.[2]Most myopericytomas are benign tumors; a malignant myopericytoma is extremely uncommon, often with local recurrence and distant metastases. Microscopically, this tumor is composed of oval to fusiform cells which illustrate a notable concentric growth around the vessels. Malignancy is characterized by deeply infiltrative growth, severe atypia, increased mitotic index, high cellularity and necrosis.[1] All these findings were present on histopathology in our case. Immunohistochemistry also supported the diagnosis. It is frequently misdiagnosed as other sarcomas. Histologically, it has overlapping features with other perivascular tumors such as glomus tumor and myofibroma.[1]These tumors require more extensive surgery with adjuvant therapy as malignant lesions may have an aggressive clinical course.[2] Radical surgical excision is the treatment of choice, as adjuvant chemotherapy and radiation therapy offer limited success in this malignancy. Our patient underwent surgery and received chemotherapy and radiotherapy. At 18 months follow-up, the general condition of the patient was good without any evidence of recurrence.Source of Support: NilConflict of Interest: None declared


Two cases of incidentally found follicular lymphoma during surveillance for ureteroscopically treated upper tract urothelial carcinoma with cross-sectional imaging are described. Multiple independent primary malignancies should be considered in this population. Cross-sectional imaging at regular intervals is recommended because of the potential for local advancement and/or metastatic disease. Given the age demographic for patients with sporadic UTUC, unrelated primary malignancies must be considered especially when imaging shows lymphadenopathy (LAD). We present two cases of primary low-grade UTUC managed endoscopically and both were found to have new onset LAD on CT scan, which ultimately were both diagnosed as unrelated primary follicular lymphomas (FLs). Correct diagnosis is essential to differentiate metastatic disease from multiple independent primary malignancies to allow appropriate treatment.C2. A CT urogram delineated a 1.5 cm right renal pelvic lesion with no evidence of local advancement or LAD in the abdomen or pelvis  was 54 mL/minute/1.73 mr pelvis .Cystoscopy showed no bladder lesions and the left retrograde pyelogram was unremarkable. Right ureteroscopy demonstrated a 1.5 cm papillary lesion, which was biopsied with a 2.4F stainless steel flat wire basket. The lesion was treated with the combination neodymium/holmium YAG laser. A ureteral stent was placed for 1 week.The ureteroscopic biopsy and site-specific cytologies demonstrated low-grade UTUC. All options were discussed with the patient and ureteroscopic treatment and surveillance were recommended given the patient's age, comorbidities, low-grade pathology analysis, and relatively small tumor size. The patient was followed endoscopically for the next 36 months, occasionally finding low-volume, low-grade recurrences, amenable to endoscopic treatment. No evidence of high-grade UTUC was noted during this time on biopsies or site-specific cytologies. The patient developed small high-grade, noninvasive bladder tumors 12 months after initial presentation, mandating intravesical therapy with BCG, which was well tolerated and effective.The patient had cross-sectional imaging yearly with contrast-enhanced CT scans of the abdomen and pelvis without findings of local advancement or metastatic disease. At 42 months from initial presentation, the patient developed significant pain in her right upper leg and complained of a 10-pound weight loss. A CT scan of the chest, abdomen, and pelvis demonstrated new onset of right-sided pelvic LAD with a 3.3 cm right external iliac node , 1.6 cm Formal lymph node biopsy was deferred by the patient, but a fine needle aspiration of the right external iliac lymph node with flow cytometry demonstrated findings consistent with a B-cell lymphoproliferative neoplasm. The patient's right leg pain spontaneously improved. The presumed diagnosis was an insidious B-cell lymphoma and active surveillance was instituted.aN0 stage with four negative obturator lymph nodes. Within 3 months, PET/CT scan showed progression of osseous lesions but no significant LAD. The patient underwent five cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. Partial remission was noted on follow-up PET/CT scan.Six months later, a positron emission tomography/computed tomography (PET/CT) scan demonstrated resolution of LAD save for a right axillary node. After formal biopsy, this demonstrated a grade 3A FL. Multiple osseous lesions were now noted but the patient was asymptomatic. Endoscopic surveillance of the right renal unit demonstrated low volumes of recurrent UTUC, which had advanced to high grade. Because of the grade change, a right laparoscopic nephroureterectomy (NU) was performed and pathology analysis demonstrated multifocal high-grade UTUC with pT2. A CT urogram demonstrated a 1.5 cm left renal pelvic filling defect and a 2.5 cm right distal ureteral filling defect, but no evidence of locally advanced or metastatic disease was present  incidentally after a Holmium laser enucleation of the prostate (HoLEP) for obstructive voiding symptoms. He also developed a high-grade, nonmuscle invasive urothelial carcinoma of the bladder, which responded to a 6-week induction course of BCG.Over 18 months, endoscopy showed that the right side remained clear but there were occasional low-volume, low-grade UTUC recurrences on the left, amenable to ureteroscopic treatment. He was found to have a low-risk prostate cancer  is the seventh most common cancer diagnosed in the United States with 19.7 new cases per 100,000 persons at risk per year with median age of 66 years at diagnosis.The challenge with this approach is dealing with the possibility of new onset LAD in an advanced aged population in which multiple primary malignancies may coexist. Nevertheless, after complete endoscopic tumor treatment, we employ yearly cross-sectional imaging. Our practice is to monitor patients with normal creatinine clearance (>60 mL/minute) using CT of the abdomen/pelvis. In patients with mild renal insufficiency (creatinine clearance between 30 and 59 mL/minute), MRI of the abdomen/pelvis is preferred. In patients with chronic creatinine clearance <30 mL/minute, noncontrast CT or MRI can be ordered with the acceptance of lower detection rates for metastatic disease. Alternatively, MRI with gadolinium can be selectively obtained only when the benefit of derived information for the patient is perceived to be greater than the potential risk of nephrogenic systemic fibrosis.4The development of LAD in well-selected low-grade UTUC patients treated endoscopically and sporadic LAD secondary to a new diagnosis of lymphoma are both relatively rare events. The rate of incidental LAD is variable in the radiology literature depending on the definition of an abnormal lymph node radiographically, degree of confirmatory follow-up, and the specific patient population. The prevalence of malignant lymphoma in a healthy Japanese population (59 years median age) undergoing elective cancer surveillance was found to be 0.16% .5 Right-sided collecting system and proximal ureteral lesions tended to involve nodes in the paracaval and renal hilar distribution, whereas left-sided collecting system and proximal ureteral lesions demonstrated nodal involvement in the para-aortic and renal hilar areas. Mid and distal ureteral lesions affected retroperitoneal and pelvic lymph node chains almost equally. By contrast, indolent lymphomas, such as FLs, present most often with a palpable or radiographically identified enlarged lymph node in the neck, axilla, abdomen, or groin.Clinically the best way to distinguish LAD as from metastatic UTUC or a new primary lymphoma is to consider the pattern of nodal involvement. In urothelial carcinoma of the bladder or upper tract, metastases typically develop in regional lymph nodes before advancing to the viscera. Matin and colleagues described a mapping study in which patients with UTUC undergoing NU also had wide retroperitoneal lymph node dissections to define tumor landing sites based on laterality and primary tumor location.Bone marrow involvement is seen in 40% to 50% of FL cases and demonstrates a patchy pattern of infiltration, leading to frequently false negative bone marrow biopsies. Excisional lymph node biopsy is necessary to diagnose the specific malignant lymphoma because adequate amounts of tissue are required to give histological architecture.7 We noted this phenomenon as well as bone marrow involvement in one of our cases. Explanations for spontaneous regression of indolent forms of lymphoma have been proposed based on host immunoregulatory control mechanisms.8 Observations of tumor regression following viral or bacterial illness indirectly suggest infection as a stimulus to suppress tumor growth potentially through complement activation. Spontaneous regression of lymphomas in organ transplant patients after discontinuing immunosuppressive therapy suggests that other humoral and/or cellular effector mechanisms may play a role.Interestingly, the natural history of FL suggests that as much as 20% to 30% of patients will have transient spontaneous regression of LAD on scans, thus leading to the observation that affected patients might display LAD that “waxes and wanes” with relapses generally within 1 to 2 years.These patients demonstrate that a second malignancy can develop during long-term surveillance of low-grade ureteroscopically treated UTUC. Full evaluation including excisional biopsy is necessary to give a diagnosis with new LAD.


Malignant peritoneal mesothelioma with invasion of the liver is an invariably fatal disease. We aimed to clarify the characteristics of malignant peritoneal mesothelioma cases with liver involvement. The clinical presentation, computed tomography images, and immunohistochemical and histopathological features of 5 patients with malignant peritoneal mesothelioma and liver involvement were evaluated. The diagnosis was established by imaging and immune profiles of the tumours. A review of 8 cases with primary or invading malignant mesothelioma in liver is presented. All 5 mesothelioma cases were asbestos-related. CT images of malignant peritoneal mesothelioma with the liver involvement typically showed that the lesion grew inside the liver along the capsule and was possibly accompanied by capsule breakthrough and extrahepatic infiltration. The tumours exhibited a common epithelioid appearance in all 5 patients and most cases revealed positive Cal, CK, and MC with negative CEA and HeP. Different from our findings, the review of literature revealed that most malignant mesothelioma of liver was due to primary intrahepatic malignant mesothelioma. Finally, we concluded that the diagnosis of malignant peritoneal mesothelioma cases with liver invasion is reliably achieved by the history of asbestos exposure, the characteristic CT imaging, and immune profiles of the tumours. Malignant mesothelioma is a tumour of the lining of the lung and chest cavity or the lining of the abdomen and is typically related to asbestos exposure –4. Next The study was approved by the ethics committee of Central Hospital of Cangzhou City, Cangzhou, Hebei, China (reference number: 2012-012-01).Five patients treated at our hospital with the diagnosis of MPM invading the liver from May 2011 to September 2014 were evaluated. All the patients received thoracic, abdominal, and pelvic computed tomography (CT) scans as well as enhanced CT, which identified malignant mesothelioma of the peritoneum without other primary tumours. Radiologists  in our hospital had interpreted the images of the 5 patients. Three radiologists independently interpreted the CT images, and discrepancies in the CT findings were resolved by a consensus opinion of at least two interpreters. The histological diagnosis of eligible patients was in accordance with the Guidelines for Pathologic Diagnosis of Malignant Mesothelioma in 2012 from the International Mesothelioma Interest Group .The principal characteristics of the cases are summarised in The 5 patients included 1 male and 4 females. The mean age at the time of diagnosis was 57.6 (range: 45–69) years. All 5 cases were positive for asbestos exposure and not comorbidity which included malignant pleural mesothelioma. All patients complained of abdominal distention and pain; 4 cases displayed massive ascites, and 1 case had no ascites. One case presented with ovarian mass. The average serum cancer antigen 125 (CA125) level  was 263.5 U/mL (range 36.6–725.3 U/mL), and the cholestatic parameters, transaminases, AFP, CA19-9, and CEA, were within normal range in the 5 cases.Abdominal enhanced computed tomography (CT) for case 1 demonstrated a mass in the hepatic flexure of colon invading the liver and lung with peritoneal thickening, and the mass was enhanced significantly in enhanced CT . For casPathologic examination of the 5 cases proved that the liver was involved. Liver involvement displayed a nodular and infiltrative pattern. The tumours exhibited a common epithelioid appearance in all 5 cases. Each tumour was examined by immunohistochemical markers according to the potential differential diagnosis. All 5 cases had follow-up. Only case 5 remains alive at this time, and the average survival time for the other 4 cases from initial diagnosis was 6.5 months.MPM is a rare malignancy, with an incidence of one case in a population of 4-5 million , and it Asbestos exposure has been shown to be an aetiology of MPM –20. In tSeveral reports have reported secondary liver involvement by MPM or primary hepatic mesothelioma. MPM invading the liver was reported in 1 case, and primary hepatic mesothelioma was reported in 7 patients  Table 2Table 2. It has been reported that MPM is more prevalent in males  and thatBecause symptoms and clinical course are usually nonspecific during the early presentation of MPM, a suspected diagnosis of carcinomatosis of unknown origin is occasionally given to patients. All 5 patients complained of abdominal distention and pain, and 1 case presented with an ovarian mass, which demonstrates that the clinical presentation of MPM cases with liver involvement is not specific.It is known that MPM frequently expresses CA125 –30. All CT findings are useful for detection and staging of peritoneal masses. For malignant pleural mesothelioma, the chest CT mainly shows a nodular or cyclic thickening of the pleura, the mediastinal pleural involvement with different degrees of pleural effusion. The enhanced CT images can be characterised by the multiple reinforcement nodules on the pleura . For MPMPleural plaques are found in approximately 50% of all patients with MPM . In our The review of the literature revealed some case of malignant mesothelioma in liver due to primary intrahepatic malignant mesothelioma –14. HoweSasaki et al. speculated that primary hepatic mesothelioma might originate from mesothelial cells of Glisson's capsule which subsequently invade the liver . In contCT findings that suggest MPM are not sufficient to establish a definitive diagnosis of mesothelioma. Peritoneal biopsy could confirm the diagnosis of MPM. Immunohistochemical panels are absolutely essential for the pathological and differential diagnosis of MPM , 34. HerHistologically, MPM conforms to one of three patterns: epithelial (the most common type), sarcomatoid, or biphasic (mixture of epithelioid and sarcomatoid) types . The tumBecause there is no single absolute marker for mesothelioma , 36, twoMPM is difficult to distinguish from liver cancer or localised tuberculous peritonitis near the liver.As noted above, CT imaging of MPM invading the liver usually shows a lesion growing inside the liver accompanied by capsular breakthrough and extrahepatic infiltration. Peritoneum around the liver and/or omentum was usually thickened with masses present.CT images of cholangiocellular carcinoma and hepatocellular carcinoma show a mass with an intrahepatic location, usually with an internal mosaic pattern, a pseudocapsule around the nodule, the presence of fat, vascular invasion, and satellite nodules. There are rare tumours breaking through the peritoneal cavity, and not lesions, which suggest primary tumours in other organs. In addition, portal vein tumour thrombosis is a well-known complication. These types of carcinoma can induce distant metastasis by hematogenous spread. The immunohistochemical findings usually indicate that these cancers are positive for AFP and HeP and negative for Cal and MC.MPM may present with abdominal distention, pain, and ascites. It is difficult to distinguish tuberculous peritonitis when the lesion is near the liver from MPM invading the liver. Most patients with tuberculous peritonitis have a history of tuberculosis and have symptoms of mild fever and night sweats. We compared the CT scans of MPM invading the liver with a case of localised tuberculous peritonitis near the liver. As the images show, the lesion was near the liver capsule with liquid accumulation and central calcification but no intrahepatic lesion or peritoneal breakthrough in localised tuberculous peritonitis . The hisFor small and limited tumours, the mass and the involved organs should be completely resected. The nonsurgical therapeutic options in MPM are very limited. Radiation is only feasible for local tumour control, and multimodal treatments with chemotherapy  can achieve partial remission in most cases of MPM . In our In conclusion, there are likely rare cases of primary hepatic mesothelioma but more cases of MPM with hepatic invasion. MPM invading the liver should be included in the differential diagnosis of hepatic tumours or other diseases that present similar CT results. Patients who have the following characteristics may have MPM with hepatic invasion: (a) history of asbestos exposure; (b) presence of pleural plaque in CT image; (c) clearly thickened peritoneum in CT image; (d) subcapsular liver lesions that are continuous with the peritoneum in CT image; (e) thickened omentum and peritoneum around liver and/or with presence of masses in CT image; and/or (f) positive for Cal, CK, and MC but negative for CEA and HeP in immunohistochemical assays. Careful CT imaging and histological and immunohistochemical analyses are required to reach a final diagnosis. Furthermore, the accumulation of additional cases similar to the current cases is necessary to characterise the features of MPM with liver involvement, including its biological behaviour and prognosis.


Odontomas are the commonest odontogenic tumors of the oral cavity and are by nature asymptomatic. They consist mainly of dental tissue that may or may not be arranged in an orderly fashion. Their presence is often detected accidentally or due to the presence of a dental disturbance such as an unerupted tooth. The very rarity of odontomas erupting into the oral cavity validates the need for more current literature on the phenomenon. Our report of two cases aims to present and discuss the rare event of an erupting odontoma with the dental community. The term odontoma was first coined by Paul Broca in 1867 and was employed as a generalized term to refer to all odontogenic tumors . These lRecent times have seen this entity being regarded by some authors as more of a hamartoma than a true neoplasm . AccordiThey account for 22–67% of all maxillary tumors , 8. OdonThe eruption of odontomas into the oral cavity is a rare phenomenon and may be associated with complications such as pain, infection, and delay in the eruption of permanent teeth. As of 2009 only 20 cases of erupted odontomas have been reported in the literature . Though A 12-year-old male patient presented with complaint of the presence of a small hard mass in maxillary anterior region. He had no complaint of pain nor was any other relevant medical history elicited. The intraoral examination revealed a small whitish oval tooth-like structure present in maxillary anterior region between 11 and 12, and both teeth exhibited a slight rotation . On enquThe lesion was hard and nontender and the gingiva around it appeared normal. An intraoral periapical radiograph  and a paThe odontoma was removed surgically under local anaesthesia and the specimen (as seen in The patient was subsequently followed up for a period of one year and no recurrence of the lesion was seen.A 16-year-old female patient reported with the complaint of a mass in the anterior maxilla since the age of eight years. The eruption was not associated with pain or any other symptoms. During the intraoral examination an agglomerated mass of calcified structure was seen in the place of missing 11 and 12 .An anterior maxillary occlusal view showed a mass with varying radiodensity resembling enamel, dentin, and cementum. This mass appeared to be attached to a root-like structure with wide root canal and open root apex . The calThe patient is currently under follow-up for the last seven months and has not shown any incidence of recurrence so far.An odontoma is a tumor of unknown etiology and is found frequently associated with a dental germ in development, a supernumerary tooth, or even a retained primary tooth . They arTheir exact etiology remains obscure but possibly includes trauma to primary dentition, inflammatory and infectious processes, odontoblastic hyperactivity, mutant gene , and herThe spontaneous eruption of an odontoma into the oral cavity is an exceptional circumstance. Such an instance may be associated with pain, inflammation of the surrounding soft tissue, or infection and suppuration . Due to The radiographic appearance and histopathology are distinctive. The complex odontoma appears as an irregular calcified mass surrounded by a thin radiolucent area with a smooth periphery and the compound odontoma appears as multiple calcified tooth-like structures in the centre of a well-defined radiolucent region . The hisTimely detection and prompt surgical treatment followed by curettage are recommended to prevent complications such as tooth loss, cystic changes, bone expansion, and delayed eruption .Odontomas are commonly occurring benign entities but rarely do they erupt into the oral cavity. We have presented two such rare cases where the odontomas have erupted into the oral cavity. Despite their benign nature, it is important that odontomas be treated promptly and conservatively so as to prevent the formation or facilitate the early correction of any dental and occlusal disturbances.


Inflammatory myofibroblastic tumour arising in the adrenal gland is exceptional. As far as we are aware, there have been only three previous reports in the literature. We report a fourth case.A 29-year-old Caucasian man presented with upper quadrant pain due to a 15cm heterogenous adrenal mass that displaced his liver. He underwent an open right adrenalectomy. Histopathological examination showed the mass to be an inflammatory myofibroblastic tumour, a histologically distinctive lesion composed of myofibroblasts, plasma cells, lymphocytes and histiocytes. Ten months later he is well with no sign of recurrence.The lesion was indistinguishable on imaging from an adrenal cortical tumour. Surgical treatment is the same but inflammatory myofibroblastic tumour carries a favourable prognosis. Inflammatory myofibroblastic tumour (IMT) is an uncommon spindle-cell proliferation that occurs at various sites including the abdomen, lungs, orbit, gastrointestinal and genitourinary tracts, usually of children and young adults ; however® (rifampicin and isoniazid).A 29-year-old Caucasian man presented with a 3- to 4-month history of discomfort in his right upper quadrant and right flank. He had no gastrointestinal or urinary disturbance and no endocrine symptoms. He had a previous medical history of latent tuberculosis, which was detected 3 months earlier, for which he was taking RifanahOn physical examination, there was anterior displacement of his liver by a palpable mass in the right hypochondrium. His blood pressure was normal. A dexamethasone suppression test, 24-hour urine catecholamine and metanephrine assays, and a plasma aldosterone: renin ratio were normal. An ultrasound scan of his abdomen and a computed tomography scan of his upper abdomen revealed a 15cm heterogenous adrenal mass compressing his liver. These appearances raised the possibility of a primary adrenal carcinoma. A metaiodobenzylguanidine (MIBG) scan and single-photon emission computed tomography scan showed no significant uptake of tracer, making a phaeochromocytoma less likely. A fluorodeoxyglucose-positron emission tomography scan revealed a right adrenal mass of size 14.5×9.5×17.2cm which was intensely metabolically active and showed dystrophic calcification and central necrosis, consistent with either adrenal cortical carcinoma or tuberculosis (Figure MDM2 gene amplification analysis by fluorescent in situ hybridization was negative.On gross examination, the resection specimen comprised a lobulated cream-coloured tumour weighing 1.3kg and measuring 18cm in maximum dimension, with some compressed normal-looking adrenal gland at one end. The tumour had a smooth outer surface and a homogenous cut surface with a whorled appearance. There were occasional areas of haemorrhage. Microscopic examination showed a circumscribed tumour composed of plump, spindled cells and histiocytoid cells arranged haphazardly and in fascicles with intervening thick collagen bundles Figure . There wThe term ‘inflammatory myofibroblastic tumour’ was introduced in 1986  to descrOn histological examination, IMTs are characterized by a proliferation of plump fibroblasts or myofibroblasts accompanied by a prominent infiltrate of chronic inflammatory cells, in particular plasma cells. This feature best distinguishes IMT from fibromatosis and fasciitis, with which it overlaps. A rare epithelioid variant of IMT is associated with an abdominal location and an aggressive clinical course .ALK gene rearrangements, resulting in overexpression of ALK protein, are present in 30 to 40% of cases, mainly in the paediatric age group. Immunostaining for ALK is therefore seen only in a minority of cases. Of interest, the IMTs that have metastasized are most often ALK negative [On immunohistochemical examination, IMTs usually express actin and may also show staining for desmin and keratin. negative .The three previous cases of adrenal IMT presented with flank pain or were incidental findings on abdominal imaging Table . ClinicaIMT in the adrenal gland is rare but must be considered in the differential diagnosis of radiologically suspicious adrenal masses. Recurrence or metastasis of adrenal IMT has not been reported but in view of the lesion’s occasional metastatic potential at other sites careful postoperative follow up including repeat imaging at 6 months to exclude local recurrence is advised.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.


A case of 19-year-old male patient reported with the chief complaint of slowly growing diffuse painless swelling over the right part of the face from last 6 months. Intraoral examination revealed a swelling on right side of palate in relation to molar region with buccal cortical plate expansion. Radiographic examination (orthopantograph and 3DCT) showed large multilocular radiolucency in right maxilla with generalized loss of lamina dura. Incisional biopsy was done and specimen was sent for histopathological examination which showed multinucleated giant cells containing 15–30 nuclei. Based on clinical, radiological, and histopathological findings provisional diagnosis of central giant cell granuloma was made. Blood tests after histopathology demonstrated elevated serum calcium level and alkaline phosphatase level. Immunoassay of parathyroid hormone (PTH) level was found to be highly elevated. Radiographic examination of long bones like humerus and femur, mandible, and skull was also done which showed osteoclastic lesions. Considering the clinical, radiographic, histopathological, and blood investigation findings, final diagnosis of brown tumour of maxilla was made. The patient underwent partial parathyroidectomy under general anaesthesia to control primary hyperparathyroidism. Surgical removal of the bony lesion was done by curettage. The patient has been followed up for 1 year with no postoperative complications and the lesion healed uneventfully. Central giant cell granuloma (CGCG) is a benign bone lesion which can be locally aggressive or may be asymptomatic in nature. In 1953, Jaffe described it as reparative granuloma of jaw bones . FemalesA 19-year-old young adult visited the outpatient clinic of the Department of Oral Surgery at Subharti Dental College with swelling over the right part of face from the last 6 months . The patTo confirm initial diagnosis, incisional biopsy was performed from maxillary lesion. Histopathology revealed numerous multinucleated giant cells within loose fibrillar connective tissue . MultinuTo assess the parathyroid gland, MRI of neck was advised which revealed small lobulated hyperintense lesion size 13.5 × 12.5 mm at the inferolateral aspect of the right lobe of parathyroid gland . ConsideThe central giant cell lesion of the jaw is a rare benign tumour with an unknown aetiology accounting for up to 7% of tumours in the mandible (lower jaw) and the maxilla (upper jaw) . As mentManagement of CGCG includes surgical curettage with or without medical management. Medical therapies include intralesional corticosteroids, calcitonin injections, and interferon-alpha therapy. They are alternatives as well as adjuncts to surgical treatment.In 1988, Jacoway et al.  first reIn 1993 Harris proposed the use of calcitonin. It increases influx of calcium into the bones and thus functions antagonistically to parathyroid hormone . SynthesAnother possible modality which is used for management of CGCG is interferon-alpha. Interferon is an antiviral and antiangiogenic agent that is used in conditions like infantile hemangiomas. It is produced by recombinant DNA technology or is purified from cultured human cells. Based on the assumption that this lesion may be vascular in origin, subcutaneous interferon-alpha has been used in the treatment of this lesion –19. It iThe giant cells in CGCG are osteoclasts. Their formation involves the interaction between stromal cells, which express RANKL which is also a potent stimulator of osteoclast bone resorbing activity. These processes are inhibited by osteoprotegerin (OPG), which is a decoy receptor for RANKL, and also by a monoclonal antibody to RANKL (AMG 162). This indicates that osteolytic expansion in CGCG can theoretically be controlled by therapeutic agents that inhibit the RANKL/RANK interaction, such as OPG and AMG162.At present, OPG and AMG162 are being evaluated for therapeutic use, mainly in studies in postmenopausal women. These reports indicate that both OPG and AMG 162 are rapidly effective and profoundly reduce bone turnover for a sustained period.In another study, the effect of imatinib on osteoclasts was examined and it was found that there was a dose dependent decrease in RANK. This finding strongly suggests that imatinib may be an effective antiosteolytic agent and could therefore be useful in the treatment of skeletal diseases involving excessive osteoclast activity, such as CGCG .Surgical curettage is the treatment of choice for CGCG. For aggressive lesions, en bloc resection is the treatment of choice. Even though radical resection is effective modality for aggressive lesions, it leads to functional disturbances .CGCG in the upper jaw in the event of endocrine disorders is termed “brown tumour.”In our case CGCG was the first manifestation with primary HPT which was confirmed by biochemical and radiological findings. Therefore, apart from CGCG, primary HPT was needed to be addressed.There are different schools of thoughts according to literature for management of CGCG associated with HPT. Regression and healing of the lesions are expected after the correction of HPT. The proper timing of parathyroidectomy and its favourable effect on regression of the brown tumour made it possible to avoid a potentially disfiguring surgical removal of the brown tumour as reported by Di Daniele et al. .There are many reasons why parathyroidectomy should be recommended for all patients with asymptomatic mild primary hyperparathyroidism. Untreated patients have a small risk of developing renal failure in the long term and an increased risk of bone loss particularly in older women. Palmer et al. conducted a cohort study and during 14-year follow-up found higher mortality rate with untreated hypercalcaemia than in age and sex matched controls. In primary HPT, there is an increased incidence of cardiovascular disease and it is predominant cause of death. Thus increased mortality can be reduced eventually by parathyroidectomy .But several cases have been reported of brown tumours that grow and do not resolve after parathyroidectomy or normalization of PTH levels that further require resection of brown tumour .Kennett and Pollick reported the need of surgical intervention for brown tumour even after parathyroidectomy because of slow regression which may take longer duration, even more than 5 years .Cases have been reported where brown tumours continued to grow although patients had been normocalcemic 2-3 months and 2 years after parathyroidectomy. It is therefore recommended that, in such cases where resolution is slow or growth continues, local curettage or enucleation should be undertaken .Both aspects of management were discussed with the parents of our patient; they agreed for simultaneous curettage along with parathyroidectomy under general anaesthesia due to financial constraint. Finally it was decided to perform surgical curettage via intraoral (I/O) approach along with parathyroidectomy, assuming that I/O approach will not cause much of functional and cosmetic deformity compared to aggressive resection via extraoral approach. Although the patient had existing missing upper right posterior teeth, later prosthetic rehabilitation for functional correction will be planned once the lesion healed satisfactorily. The patient was kept under regular follow-up and it was noticed that complete regression of swelling occurred after 1 year without any cosmetic deformity.Parathyroid adenomas are usually small and almost never palpable in the neck. When clinical manifestations are present, the signs and symptoms can be divided into two types: urologic changes secondary to hypercalcemia and skeletal changes. Urologic changes include polyuria, polydipsia, and the development of kidney stones. In severe cases it may cause anorexia, vomiting, constipation, fatigue, weight loss, muscle weakness, psychiatric symptoms, and pancreatitis . Except Early diagnosis of HPT can be done with assessment of all the radiographic, biochemical, and histopathological parameters. Giant cell lesions like CGCG must be suspected and investigated to rule out hyperparathyroidism. Although the lesion resolves spontaneously after parathyroidectomy, surgical curettage should be well thought out for large lesions irrespective of site of lesion.


Ependymomas are common spinal lesions, with the vast majority arising in an intramedullary location. Several cases have been described in the literature of ependymomas in an intradural, extramedullary location. The authors present a case of a 56-year-old female who presented with several weeks of lower back pain and weakness. MRI revealed an intradural, extramedullary enhancing mass at L1-L2. The mass was successfully resected surgically. Pathologic evaluation revealed a low grade glioma with components of both ependymoma and pilocytic astrocytoma with MUTYH G382D mutation. Extramedullary ependymomas are very rare tumors. To the authors' knowledge, this is the first case of ependymoma/astrocytoma collision tumors described in an extramedullary location.  Most adult ependymomas are spinal lesions, with the vast majority arising in an intradural, intramedullary location . IntraduA 56-year-old female presented to an outside hospital for several months of low back pain, bilateral hip pain, and leg pain/weakness. She subsequently had an episode of severe low back pain that resulted in difficulty breathing and loss of consciousness. Review of systems revealed several episodes of incontinence. Physical exam was remarkable for 5/5 strength in all extremities, 2+ reflexes in the lower extremities, and a negative Babinski sign.Magnetic resonance imaging of the lumbar spine performed with and without intravenous contrast revealed a 2.1 cm × 1.5 cm × 1.3 cm intradural, extramedullary mass at L1-L2 which compressed the filum terminale. Precontrast MRI showed intrinsic T1 hypointensity and heterogeneous T2 hyperinstensity Figures . PostconGiven the patient's symptoms, she underwent elective operative resection of the mass. A posterior L1-L2 laminectomy was performed. A tan, fleshy intradural tumor was identified . Most ofGross examination revealed an oval shape soft nodule with a smooth surface, which appeared encapsulated with a delicate membrane. Permanent sections showed cellular biphasic glial neoplasm: moderately cellular tumor with numerous perivascular pseudorosettes and less cellular areas with prominent eosinophilic fascicular component . The celThe lesional cells, both components, showed strong, diffuse immunopositivity with glial fibrillary acidic protein (GFAP) and S-100 (more in fascicular component). Synaptophysin was staining stronger in the ependymal component . The Ki-In summary, the microscopic examination revealed low grade glial neoplasm, with histologic features of ependymoma and pilocytic astrocytoma, closely intermixed, and represented collision tumor.The tumor was analyzed for mutation using a massively parallel (“next generation” or NGS) sequencing approach, performed by Foundation Medicine, INC, Cambridge, MA. This test assesses genomic alterations including base pair substitutions, insertions and deletions, and copy number alterations and select gene rearrangements for 315 cancer-related genes and introns from 28 genes often rearranged or altered in cancer, including NF1, NF2, IDH1, and TP53 . The mutThe patient had no postoperative complications. At one-month follow-up, she had resolution of lower extremity pain and tingling. At one-year imaging and clinical follow-up, patient had no evidence of tumor recurrence.Ependymoma (WHO grade II) is the most common intramedullary intradural spinal tumor followed by pilocytic astrocytoma. Although uncommon, spinal ependymomas (WHO grade II) have been described in an extramedullary, intradural location. To our knowledge, 23 such cases have been described –13. The Other extramedullary tumors of glial origin have been reported. Singh et al. described an astrocytoma in the conus region . Kumar eOur case showed an extramedullary low grade glial tumor with histologic characteristics of an ependymoma and astrocytic lesion. Additionally, a fascicular component with Rosenthal fibers and cystic change was seen, consistent with pilocytic astrocytoma, and was intermixed with ependymoma component. This leads us to speculate the possibility that the lesion has dual origin: ependymal and astrocytic. We considered the option that the astrocytic component might represent piloid gliosis within a WHO grade II ependymoma. However, this was discounted as the astrocytic component was not focal or at periphery of the lesion, which one would expect for reactive change/piloid gliosis, but rather interspersed throughout and comprised at least half of the entire lesion. In addition, the presence of ependymal and fibrillary differentiation within the same pseudorosette further supports the hypothesis of compound tumor.There are no absolute histologic criteria or immunohistochemical markers to differentiate piloid gliosis from pilocytic astrocytoma. Recent studies have shown that some pilocytic astrocytomas carry duplication at chromosome band 7q34 containing a BRAF-KIAA1549-gene fusion . AlthougSeveral cases of mixed astrocytoma and ependymoma have been described, though none have been identified in the spine. Molnár and Hegedüs encountered an intraventricular ependymoma in the setting of a partially removed infiltrating astrocytoma in the frontal lobe . HoweverThere are case reports of ependymomas to have mesenchymal differentiation, for example, cartilage formation, which can be seen in adults and children , 24. It To our knowledge, no cases of mixed ependymoma-astrocytoma have been reported. A tumor of glial origin should be included in the differential of an extramedullary, intradural mass, and based on our findings, a composite tumor should be considered in such a case.


We report three cases of dysembryoplastic neuroepithelial tumor (DNT) with intractable epilepsy which were successfully treated with surgery.18F-fluorodeoxyglucose positron emission tomography (PET), 11C-methionine-PET, interictal electroencephalography, and intraoperative electrocorticography were utilized in all patients. In individual cases, however, additional procedures such as preoperative magnetoencephalography (Case 1), diffusion tensor fiber tractography, a neuronavigation system, and intraoperative somatosensory-evoked potential (Case 2), and fiber tractography and the neuronavigation-guided fence-post tube technique (Case 3) were instrumental.In all cases, technology beyond the routine workup was critical to success. Preoperative magnetic resonance imaging, In all the cases, the objectives of total tumor resection, resection of the epileptogenic zone, and complete postoperative seizure control and the avoidance of surgical complications were achieved.Dysembryoplastic neuroepithelial tumor is commonly associated with medically intractable epilepsy, and surgery is frequently utilized. As DNT may arise in any supratentorial and intracortical locations within or near the critical area of the brain, meticulous surgical strategies are necessary to avoid neurological deficits. We demonstrate in the following three cases how adjunct procedures using advanced multitechnologies with neuroimaging and electrophysiological examinations may be utilized to ensure success in DNT surgery. Additionally, electrocorticography (ECoG) is also used intraoperatively to detect the epileptogenic zone (EZ).Dysembryoplastic neuroepithelial tumor (DNT), first described by Daumas-Duport et al. We report three cases of DNT with intractable epilepsy, successfully treated with surgery, in which not only imaging functional studies but also advanced neurosurgical technologies were critical for planning and supported the role for surgery. These interventions included preoperative magnetoencephalography (MEG) (Case 1), fiber tractography obtained from diffusion tensor imaging (DTI), a neuronavigation system, and intraoperative somatosensory evoked potential (SEP) (Case 2) and fiber tractography and the neuronavigation-guided fence-post tube technique (Case 3) .Table 1CThe medical equipment used in these cases were MRI , PET , neuronavigation system , SEP/EEG/ECoG , and MEG . Magnetoencephalography in Case 1 was performed at Komaki City Hospital.22.1The patient, a 43-year-old woman, presented with a 20-year history of complex partial seizures occasionally followed by generalized tonic–clonic convulsive seizures. Despite treatment for over two years with multiple anticonvulsants, she continued to have seizures several times a month. Her MRI showed a relatively well-demarcated, small, mass lesion (1.8 × 1.8 × 1.8 cm) in the left medial temporal lobe, which presented with hypointensity on a T1-weighted image (T1WI), hyperintensity on T2WI, hypointensity with a surrounding high intensity ring in fluid attenuated inversion recovery (FLAIR), no gadolinium (Gd) enhancement, low uptake in MET-PET, and hypo-uptake in FDG-PET E and F. 2.2The patient, a five-year-old girl, presented a four-month history of complex partial seizures occurring a few times a day. Anticonvulsants reduced the seizure frequency to a few times a week. Her MRI showed a relatively well-demarcated mass lesion (3.0 × 3.0 × 4.0 cm) in the left frontal lobe extending to the left lateral ventricle wall, which presented with hypointensity on T1WI, hyperintensity on T2WI, hypointensity with a surrounding high intensity irregular ring on FLAIR, no Gd enhancement, low uptake in MET-PET, and hypo-uptake in FDG-PET D and E a2.3The patient, a 10-year-old girl, presented with a history of surgery for DNT in the left posterior temporal lobe. She had developed complex partial seizures refractory to anticonvulsants and had seizures a few times a month before the surgery. She underwent a partial resection of the tumor in a community hospital at the age of eight years, and the tumor was diagnosed as a DNT histologically A. After 3The goals of surgery for DNT include not only complete tumor resection but also complete seizure control by resecting the EZ, while avoiding surgical sequela 11C-methionine positron emission tomography has improved the radiological diagnosis of brain tumors Characteristics of DNT, such as a well-demarcated margin, benign clinical course, indolent biology, and the frequent association of a peritumoral EZ, are conducive to surgical management. The preoperative diagnosis of a DNT facilitates the assemblage of intraoperative monitoring equipment necessary for precisely excising the EZ. Epileptogenic brain tumors which should be differentiated from DNT include diffuse astrocytoma, oligodendroglioma, pleomorphic xanthoastrocytoma, pilocytic astrocytoma, gangliocytoma, and ganglioglioma. Some may be easily differentiated from DNT using a conventional CT and/or MRI; however, others cannot precisely be differentiated because of their similar radiological features. Intraoperative ECoG is an essential tool for DNT surgery and is utilized in most surgical procedures for epilepsy For some DNT, preoperative CT, MRI, interictal EEG, PET studies, and intraoperative ECoG are usually sufficient to achieve seizure control with surgery; however, for others, these are not sufficient. In Case 1, the preoperative interictal EEG did not show any significant epileptogenic activity. Dysembryoplastic neuroepithelial tumor in the temporal lobe, like other epileptic lesions in deep brain structures, often presents with no significant epileptogenic activity in preoperative EEG. In these cases, preoperative chronic subdural electrode recording (CSDER) is strongly recommended to confirm whether the tumor truly has epileptogenicity and to evaluate the extent of the epileptogenic lesion The DNT in Case 2 was adjacent to the motor cortex and the language pathway. Epileptogenicity was detectable in the tumor by preoperative EEG. Complex partial seizures observed in this case were likely attributable to tumor extension to the left cingulate gyrus. In this case, treatment planning focused on avoiding damage to the motor cortex and the language pathway while aiming for total resection of the tumor with the EZ. In such a case, the combination of fiber tractography, neuronavigation system, and SEP is a valuable adjunct to ECoG and imaging studies Case 3 had a recurrent large DNT adjacent to the left visual tract, in which epileptogenicity was detectable near the tumor by preoperative EEG. The patient had already had a right upper quadrant hemianopsia from the previous surgery. Another clinicopathological concern in this case was the possibility of malignant transformation of the tumor in terms of its rapid regrowth. Dysembryoplastic neuroepithelial tumor is considered benign and tends to not recur even after a partial resection 4Although DNT frequently results in epilepsy that is unresponsive to medical therapy, DNT-associated epilepsy is highly curable with surgery. Dysembryoplastic neuroepithelial tumor may arise in any supratentorial or intracortical location within or near critical areas of the brain. Resection, therefore, must be carefully planned both pre- and intraoperatively and requires meticulous technique. Goals of therapy include not only completely resecting the tumor to avoid recurrence or progression but also excising the entire associated EZ. We have shown that when imaging and functional studies are utilized concurrently with advanced neurosurgical technologies for operative planning, excellent surgical outcomes result.None of the authors has any conflict of interest to disclosure.


Gastroenteropancreatic neuroendocrine tumors have occasionally been described in association with neurofibromatosis type 1, whereas an association with neurofibromatosis type 2 has never been reported.This report refers to an Italian 69 year old woman with neurofibromatosis type 2 and a pancreatic gastrinoma. In the past she had encephalic meningiomas, a tongue schwannoma and bilateral acoustic neurinomas. She presented with weight loss and a long-term history of diarrhea, responsive to proton pump inhibitors. Upper gastrointestinal endoscopy revealed peptic ulcer of the duodenal bulb. Blood tests were normal, except for the elevation of plasma gastrin  and chromogranin A . After secretin stimulation testing, the plasma gastrin level rose to 3789 pg/ml. The abdomen magnetic resonance imaging and gallium68-DOTATOC positron emission tomography scan demonstrated the presence of a 1.2 x 2 cm lesion in the pancreatic head and a liver metastatis. Pancreatic endoscopic ultrasound with fine needle aspiration revealed cytomorphologic features suggestive of pancreatic gastrinoma. Brain magnetic resonance showed a pituitary microadenoma. There was no evidence of hyperparathyroidism. The genetic test for multiple endocrine neoplasia type 1 syndrome mutation was negative.This report focuses on the first case of coexistence of gastrinoma with neurofibromatosis type 2. Although the clinical relevance of this association remains to be determined, our case report will surely give cause for due consideration. Both gastroenteropancreatic neuroendocrine tumors (GEP NET) and neurofibromatosis type 2 (NF2) are fairly rare diseases,2. GEP NGastrinoma is a gastrin-secreting tumor usually located in the duodenal wall or in the pancreas. ChronicAccording to available data, an association between GEP NET and NF1 has already been reported and it is well recognized that NF1 patients may develop NET, usually in the duodenum or periampullary region-14 and oNF2 is an autosomal dominant inherited disease characterized by the predisposition to develop tumors of the central nervous system (CNS). A commoAt present an association between GEP NET and NF2 has never been reported. The present case report refers to a woman with NF2 and a pancreatic gastrinoma.In 2011 an Italian 69 years old woman was referred to our Gastrointestinal Unit due to an 8 years long history of diarrhea, dyspepsia and weight loss. Diarrhea was responsive to PPI. Two years before (2009) she was diagnosed as having NF2 syndrome, according to internationally accepted clinical criteria, based oWe have reported the first case report of the association between NF2 and a pancreatic gastrinoma, although it remains to be determined whether this association is merely casual or not. The possible underlying pathogenetic mechanisms need also elucidation.The association between NF1 and GEP NET has already been established. Several case reports and reviews of the literature describe the coexistence of NF1 and neuroendocrine neoplasms, such as somatostatinomas, gastrinomas, insulinAs concerns patients with NF2, schwannomas of the intestinal tract are a possible complication, whereasAn increased occurrence of endocrine tumors, as well as of other non endocrine tumors, including collagenomas, ependimomas, schwannomas and meningiomas has been recently reported in patients with MEN1-29. MeniThe present report focuses on the first case of gastrinoma in a NF2 patient. This patient might have a MEN1 like phenotype, as she showed the clinical features typical of MEN1 syndrome , despite the absence of hyperparathyroidism and MEN1 gene mutations. The phenotype of MEN1 is broad and different combinations of endocrine and non endocrine manifestations have been described. About 30% MEN1 patients do not carry detectable MEN1 gene mutations. PatientOverall, the possible pathogenic mechanisms underlying the occurrence of both gastrinoma and NF2, which would allow to discriminate between an actual connection and a merely casual association, remain to be elucidated.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.GEP NET: Gastroenteropancreatic neuroendocrine tumors; NF2: Neurofibromatosis type 2; NF1: Neurofibromatosis type 1; ZES: Zollinger Ellison syndrome; PPI: Proton pump inhibitor; MEN1: Multiple endocrine neoplasia type 1; CNS: Central nervous system; MR: Magnetic resonance; PET: Positron emission tomography.We have no conflict of interest to declare.SM planned the work. SM, AZ and RER wrote the paper and subsequently performed its critical review, contributing equally to this work. AZ and FC carried out the literature research; MP contributed to the acquisition of data and their interpretation. DC and MP revised all the materials and manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/14/110/prepub


Hemangiomas account for 0.4-0.6% of all tumors of the parotid gland and most of them occur in children, nevertheless in adults hemangiomas are very rare. We report the case of a 62 year old woman with a mass in the parotid right tail associated with fluctuating swelling episodes unrelated to meals and with a slowly progressive growth. The provisional diagnosis was a pleomorphic adenoma, so a right superficial parotidectomy was performed. During surgery, the macroscopic appearance makes suspect a vascular lesion. The histopathological result was a cavernous hemangioma. The classic clinical presentation of a parotid hemangioma is an intraglandular mass associated or not with skin lesions characterized by reddish macules and/or papules, and a vibration or pulsation when palpating the parotid region. In imaging tests, phleboliths could be observed which are very suggestive of a hemangioma or a vascular malformation. In the absence of these signs, the diagnosis could be difficult, particularly in an adult due to its low prevalence, with about 50 cases reported worldwide. However a hemangioma should be considered in the differential diagnosis of parotid tumors in adults. Key words:Cavernous hemangioma, parotid gland, superficial parotidectomy, pleomorphic adenoma. Hemangiomas are vascular abnormalities that are characterized by increased proliferation and renewal of endothelial cells. They are classified as cavernous, capillary and mixed hemangiomas . The 65%A 62 years old female with no family history of interest has a right parotid mass with a slowly progressive growth of 4 years of evolution. Initially, it was asymptomatic and subsequently, fluctuating swelling episodes, not associated with meals, in the parotid region appeared.On physical examination, a soft, elastic, painless, non-fluctuating, non-pulsatile mass without trophic skin changes and of 3x2cm of maximum diameter, was palpated in the tail of the right parotid gland. The parotid duct was permeable with transparent production of saliva. Cervical lymphadenopathy or other masses were not palpable. Fine needle aspiration (FNA) and magnetic resonance imaging (MRI) was made to guide the diagnosis. The result of FNA was inconclusive. In the MRI, at the superficial lobe of the parotid gland, posterolateral to the retromandibular vein, a well-defined mass 3 x 2.6 x 2.3 cm was revealed. It appeared as a lobulated tumor, hyperintense on T2, hypointense on T1, and with intense and homogeneous enhancement after intravenous contrast administration but without extension to adjacent structures  is useful in demonstrating lesions of the parotid region and its extension. Hemangiomas usua-lly appear as a lobulated lesion with intermediate signal on T1, hyperintense on T2 and homogeneous enhancement with contrast. MRI also helps determine the surgical approach for the tumor and to reveal the relationship with adjacent structures. The fine needle aspiration (FNA) is useful in the preoperative diagnosis of tumors of the head and neck. It is considered unnecessary in a hemangioma because of the probability of generating a hematoma and when MRI is highly suggestive of the diagnosis. Therefore, a typical clinical presentation and characteristic radiologic findings are sufficient for the diagnosis ,8.Currently, the treatment of choice in the cavernous intraparotid hemangioma is surgery, taking into consideration a pre-surgical embolization. However, infantile hemangiomas have other treatment options such as endovascular sclerotherapy, intralesional or systemic corticosteroids, vincristine, and propanolol .Researching studies have found recently, the expression of the cyclo-oxygenase 2 (COX2) protein on endothelial cells of various vascular spaces of cavernous hemangiomas. There is little evidence of the relation of vascular tumors with the expression of COX2. However, there has been reported that high doses of celecoxib have inhibited the cell proliferation of angiosarcomas cell lines. So it could be considered as a new therapeutic line research for tumors of vascular origin .


Angiomyolipoma and PEComa are rare tumors descending from perivascular epithelial cells (PECs), with distinctive IHC, morphological, and ultrastructural features. The kidney is the most frequent site of origin, but not the only one; however, adrenal gland angiomyolipomas are extremely rare. We describe two cases being found in the adrenal glands. Given the paucity of literature on the subject, more information on this disease is necessary for diagnosis and treatment. Here, we describe two complete case reports, from presentation to treatment and follow-up, along with imaging and microscopic pathology samples, and provide a comprehensive review as to the history and current literature available regarding these extremely rare tumors. Angiomyolipomas are mesenchymal tumors made up of abnormal blood vessels, spindle cells, and mature adipocytes, believed to be derived from perivascular epithelioid cells (PEC) . PEComas65 y/o Caucasian female with hypothyroidism and coronary artery disease presented with intermittent right upper quadrant abdominal pain for one year, worsening over a month. She denied fevers and weight changes, was tolerating her diet without difficulty, and had noticed no change in bowel movements. She reported no history of nephrolithiasis, hematuria, or dysuria. Physical exam was unremarkable except for mild right flank tenderness.Abdominal U/S revealed a large heterogeneous mass, appearing to arise from the right adrenal gland. CT scan confirmed an 11.3 × 9.4 cm right adrenal mass with scattered calcifications consistent with adrenal CA . 24-hourGiven the suspicion for adrenal CA, the patient underwent an open right adrenalectomy. The mass displaced surrounding structures with no evidence of invasion. Intraoperative frozen sections demonstrated necrotic tissue without signs of malignancy. The 626 g, 13 × 12 × 9 cm specimen contained adipose tissue, thick walled blood vessels, and scattered foci of spindle cells with areas of hemorrhage and calcifications on H&E stain . Immunoh63 y/o Caucasian male without past medical history presented with chronic cough, for which a CT scan of the sinuses and chest was performed. The lower images of the CT showed a possible right kidney lesion. CT of the abdomen and pelvis demonstrated a 13 cm right adrenal mass, consistent with adrenal CA. MRI clarified the lesion as adrenal with no invasion of surrounding structures . The patThe tumor replaced the adrenal gland in its entirety but the capsule was intact, with various growth patterns Figures  and 6. HIn 2002, the World Health Organization (WHO) recognized a family of neoplasms showing morphologic and immunohistochemical evidence of perivascular epithelioid cell (PEC) differentiation, including angiomyolipoma (AML), lymphangioleiomyomatosis (LAM), and other clear cell tumors [The term “angiomyolipoma” was first coined in 1951 by Morgan to describe a benign renal tumor composed of blood vessels, muscle, and fat , 7. The The diagnosis of angiomyolipoma is made by the presence of components listed above, as well as using immunohistochemistry. In most cases, the tumors stain positive for desmin, muscle specific actin, and HMB45. Markers for epithelial cells, cytokeratins, and epithelial membrane antigen are generally absent .Angiomyolipoma is the most common benign resectable tumor of the kidney and is rare in other locations. As few as 40 documented cases of extrarenal angiomyolipoma exist and only three cases of adrenal angiomyolipoma have been reported. The first two cases of adrenal angiomyolipoma were published in 2001 in the Journal of Clinical Pathology by Lam and Lo. They described a 46 y/o female incidentally found to have an 8 cm left adrenal mass. The second case was a 20 y/o male with tuberous sclerosis who noted hematuria and left groin pain. He was found to have multiple angiomyolipomas in both kidneys, the liver, and the left adrenal gland . In 2005Imaging of angiomyolipomas demonstrates characteristic aneurysmal dilation, representing thick-walled vessels , 9. It cThe first perivascular epithelioid cell (PEC) was noted by Apitz in 1943 in renal angiomyolipomas. The term PEComa was introduced in 1996 by Zamboni to describe a tumor arising in the pancreas . Since tAs with angiomyolipomas, with PEComas, there is an association with tuberous sclerosis, an autosomal dominant syndrome. This rare, multisystem genetic disease results from mutations from one of two genes, TSC1 or TSC2. These genes encode tumor growth suppressor proteins hamartin and tuberin, causing tumors to grow in the brain, kidney, heart, lungs, eyes, and skin. Historically, neurological issues were the primary cause of death for these patients, but as medical care has progressed, renal disease has become the primary cause of death. Given that these patients develop masses at multiple sites, including the kidneys, it was observed that these patients develop renal cysts and masses in childhood, and the incidence only increases with age. 60–80% of these patients will have angiomyolipomas, of which PEComas are a modulated cell type .There is a marked increase in incidence in patients of female sex (6-7 : 1), and age at diagnosis ranges within 8–89 years. There is a high level of association (60–80%) with tuberous sclerosis. The most frequent sites of PEComas are the uterus and retroperitoneum, though the tumor can be found virtually anywhere –24. The A small subset of PEComas show aggressive behavior and can be malignant. Folpe described high-risk criteria for malignancy, including size > 5 cm, mitotic rate > 1/50 hpf, necrosis, high nuclear grade and cellularity, vascular invasion, and an infiltrative growth pattern  Figure . If a tuThis is one of the first noted cases of an adrenal malignant PEComa, and the fourth adrenal angiomyolipoma. These tumors appear to represent a spectrum of diseases derived from the perivascular epithelioid cell (PEC). Both of these patients have been followed up for over 36 months without evidence of recurrence or metastatic spread from the malignant PEComa. As more of these tumors are described, open questions will likely be answered.