We present the clinical course of a pediatric patient with large rhabdomyosarcoma of the body trunk who received proton beam therapy (PBT).A 1-year-old girl was diagnosed with stage IV alveolar rhabdomyosarcoma in 2008. A large tumor was located in the central diaphragm and had infiltrated the liver and pericardium with peritoneal dissemination. Chemotherapy was immediately started with six courses of vincristine, actinomycin-D and cyclophosphamide (VAC) firstly, and secondly followed by 2 courses of ifosfamide, carboplatin and etoposide (ICE), but a large tumor of 15 cm in size remained. The tumor was inoperable because of its location, and photon radiotherapy could not be performed due to limited liver tolerance. The patient was referred to our hospital and received PBT at a dose of 54 GyE in 30 fractions in June 2009.The tumor quickly responded and 95 % of volume reduction was achieved at the end of PBT. However, marginal recurrence in the caudal part of the irradiated field, where we reduced the proton dose because of the presence of the intestine, was detected in August 2010. The recurrent tumor size was less than 1 cm. Chemotherapy with VAC followed by topotecan and carboplatin (TC) was again tried, but the tumor size was stable. Repeated PBT was not possible because of limited intestinal tolerance; therefore, intraoperative radiotherapy was conducted with 20 Gy of electron beams in April 2011. The tumor was subsequently well controlled, but secondary myelodysplastic syndrome developed and the patient died of hemophagocytic syndrome after umbilical cord blood transplantation in May 2012.PBT was performed safely and effectively for a 1-year-old girl with alveolar rhabdomyosarcoma with liver and cardiac invasion that was resistant to surgery and chemotherapy. This case illustrates that PBT can be useful in cases that are difficult to treat with conventional radiotherapy. Radiotherapy plays an important role in treatment of rhabdomyosarcoma (RMS), with a dose of 40–60 Gy typically required, and hyperfractionation (1.1-Gy twice daily fractions) or standard fractionation (1.8-Gy daily fractions) is used for definitive treatment , 3. HereA girl aged 1 year and 6 months initially presented with an abdominal mass with a maximum diameter of over 15 cm. The mass was located in the central diaphragm and was infiltrating the liver and pericardium with peritoneal dissemination. Ascites cytology showed class V in Papanicolaou’s classification. The patient was diagnosed as alveolar RMS IRS Stage 4, postoperative group IV, and classified in the high risk group. Chemotherapy was started, but the tumor did not shrink sufficiently: with disease progression after the first 2 courses of VAC every 3 weeks, including vincristine (VCR) + actinomycin-D (Act-D) + cyclophosphamide (CPA); and partial response (PR) after 2 courses of ICE, including ifosfamide (IFM) + carboplatin (CBDCA) + etoposide (VP-16). Surgical resection was not possible for broad tumor’s invasion of the surrounding diaphragm and radical photon radiotherapy could not be performed because the liver could not tolerate the definitive treatment dose. Palliative care was initially recommended from local pediatric oncologists, but subsequently the patient and her family visited our hospital to receive PBT, which was started in June 2009 at age 1 year and 11 months. Peritoneal dissemination disappeared after the chemotherapy, but the tumor was still located in the central diaphragm with infiltration of the liver and pericardium, and the maximum diameter was still over 8 cm at the point (Fig. The incidence of soft tissue sarcomas in children and adolescents younger than 20 years of age is 11.0 per million, representing 7.4 % of cancer cases for this age group . RhabdomIn this case, PBT was performed safely and effectively with alveolar rhabdomyosarcoma with liver and cardiac invasion that was unable to be removed with surgery and was resistant to chemotherapy. We supposed that side effect and tumor’s local control turned worse, if we used conventional radiotherapy not PBT. This case illustrates that PBT can be useful in cases that are difficult to treat with conventional radiotherapy.Written informed consent was obtained from the patient’s legal guardians for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


We report the case of a 60-year-old man with an accessory spleen in the pelvis. He visited our outpatient clinic because of abdominal discomfort. Computed tomography (CT) showed an enhanced mass (40 mm in diameter) in the pelvis. Preoperative diagnosis was difficult even after magnetic resonance imaging and colonoscopy. The patient underwent surgery for suspicion of a gastrointestinal stromal tumor or malignant lymphoma of the rectum. Intraoperative findings showed a mass in the pelvis and a long cord-like tissue reaching the mass and arising from the great omentum; the mass was excised. Histopathologic examination indicated that the mass was splenic tissue, and feeding vessels were found in the cord-like tissue, which were determined to be derived from the left gastroepiploic artery and vein. Thus, we diagnosed it as an accessory spleen in the pelvis. An accessory spleen is not rare and can occur anywhere in the abdominal cavity. However, an accessory spleen in the pelvis is an infrequent finding, and only 9 other cases of an accessory spleen in the pelvis have been reported. Therefore, it is very difficult to make a correct diagnosis preoperatively. However, 7 of the 9 cases (77.8 %) of a pelvic accessory spleen had vascular pedicles from the great omentum or splenic hilum as feeding vessels; hence, determining the feeding blood vessels on dynamic CT may be useful for diagnosing an accessory spleen in the pelvis. Additionally, if the accessory spleen is symptomatic or has a vascular pedicle, surgeons should attempt to resect the accessory spleen in the pelvis using minimally invasive laparoscopy. Accessory spleens are not rare and are commonly found in the splenic hilum, great omentum, and pancreas . HoweverA 60-year-old man visited our outpatient clinic because of lower abdominal discomfort in the 2 months preceding his visit. He had a history of chronic hepatitis C infection and cirrhosis. There was no history of abdominal trauma. The abdomen was soft, and no mass was palpable. The hemoglobin concentration, white blood cell count, platelet count, and C-reactive protein level were within normal limits. The tumor marker levels were also within normal ranges, such as CEA 2.9 ng/mL and CA19-9 25 U/mL. Dynamic computed tomography (CT) showed a 40-mm solid pelvic mass anterior to the rectum. The mass was enhanced homogeneously with no infiltration into the organs surrounding the mass Fig. . Since tIntraoperative findings showed a spherical firm mass with a well-defined capsule, measuring approximately 4 cm in diameter. The mass had a long cord-like tissue arising from the great omentum Fig.  and was Dogan et al.  reportedA PubMed search of the literature using the keywords “accessory spleen” and “pelvis” resulted in the identification of only 9 cases of an accessory spleen in the pelvis –9, incluAdditionally, we found that in 7 of the 9 cases 77.8 %) in the previous studies, the feeding vessels originated from the vascular pedicles from the great omentum or splenic hilum –4, 6–8.  % in theSurgery is recommended in cases of a pelvic accessory spleen with a vascular pedicle, because the pedicle may cause abdominal symptoms due to torsion, as reported by Vural et al.  that torSurgeons should confirm the presence or absence of a vascular pedicle on dynamic CT upon incidental identification of a pelvic mass. Additionally, laparoscopic surgery should be considered for the resection of an accessory spleen in the pelvis with a vascular pedicle.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.


Splenic “incidentalomas” are focal lesions of the spleen that are discovered on imaging studies that were performed for the evaluation of non-spleen related pathology. These lesions are frequently encountered on CT and ultrasound examinations. Most incidental focal splenic lesions are benign, however a systematic approach to evaluating these lesions is important, because many benign and malignant splenic masses share common imaging features ,2.Small studies report the prevalence of incidental splenic lesions on CT scans performed on trauma patients to be less than 1.5% . MetastaAsymptomatic simple cysts are almost always benign; these are most commonly false cysts due to prior hematoma or infarct, as well as true congenital epidermoid cysts and pancreatic pseudocysts. Abscesses, echinococcal cysts, cystic metastases, and lymphoma can manifest as simple cysts, but these are not usually incidental; there is usually a typical history, and there are usually other clues on the study. In any event, these more commonly cause complex appearing cysts. Patients with splenic abscesses are almost always febrile and symptomatic. Abnormal enhancement patterns suggest malignant etiologies such as metastases, lymphoma, and angiosarcoma. Hypervascular masses are frequently due to benign masses such as hemangiomas, hamartomas, littoral cell angiomas, and lymphangiomas, but hypervascular metastases and lymphoma must be considered. Angiosarcomas are rare, and cause irregular hypervascular masses. The differential diagnosis of multiple small hypovascular masses includes granulomatous disease, metastases, and lymphoma.Most calcifications are benign, but several morphological forms may be associated with malignancy or infection.Lesions with specific benign features, such as a simple cyst in an asymptomatic patient may be dismissed. Prior imaging and history are the key to evaluating indeterminate lesions such as small hypovascular masses. Stable lesions for one year or greater in patients without cancer are benign; if no prior examinations are available then indeterminate lesions in asymptomatic patients should be followed. If there is a history of lymphoma or other cancer, MRI or PET scan should be considered. The most common indication for percutaneous splenic biopsy is an indeterminate splenic mass in a patient with history of lymphoma or other malignancy . Publish


A full termed local ewe with the history of continuous straining with labored breathing for last 24 hours was presented. The animal was disinclined to move with tense and round abdomen which developed rapidly during last two weeks. Caesarean section revealed hydrallantois accompanied by multiple fetal congenital abnormalities. The ewe was under observation for four weeks. Metritis developed 12 days post-operation and was treated successfully. The ewe was found active on 25 days post-surgery with gain of extra 3 kg bodyweight.  The present report documents a rare case of hydrallantois along with fetal monstrosity in a local sheep of Kashmir. Hydrallantois is frequently reported in bovines. It is rarely reported in small ruminants. In such a condition, the fetus is either normal or slightly smaller in occasional cases.Case history observation and treatmentA full termed local ewe, aged 3 years and weighing 48 kg, at her first parity was presented with the history of labor started 24 hours earlier. As per history there was rapid abdominal enlargement in last 15-20 days which was suspected as a probable cause of twin pregnancy by the owner. At the time of presentation the animal was in sternal recumbency. Abdominal wall was bilaterally distended, round and tense. Pulse rate was elevated (100 bpm) with normal temperature (38.6 ˚C) and deep abdominal respiration. Vaginal examination revealed closed os-cervix. Ultrasonography revealed heavy fluid accumulation in the allantoic cavity. The case was initially diagnosed to be a case of hydrallantois. Because of the condition of the patient it was decided to perform immediate cesarean section. Pre-operatively animal was administered with analgesic, systemic antibiotic and intravenous fluid therapy as per recommended therapeutic dose. -1, IM for 3 days) were administered with regular antiseptic dressing. Information of the patient was taken by phone at every two days interval. Skin sutures were removed 12 days following surgery. However, metritis was developed and was treated with both systemic and intrauterine antibiotic preparations with a single dose of PGF2α. The animal was found active and alert 25 days post-operation with 3 kg extra body weight gain. The C-section was conducted under local infiltration of 2% lignocaine hydrochloride using left paramedian approach as per standard procedure. Fluid filled gravid uterus was very cautiously exteriorized with utmost care to prevent peritoneal contamination. After incising the uterine horn, more than 15 liters of amber colored allantoic fluid resembling transudate were drained out. Following removing of fluids a male monster  was takeDescription of the monster. The monster weighing 2.5 kg was alive up to 2-3 min and died thereafter. Before death heart beat could be heard very prominently as part of it was extrathoracic. The head of the monster was connected with blood vessels and tissues to umbilicus. Whole intestines and liver were herniated through a big opening at the site of attachment of the absent left (Amelia) fore leg to the body. There was cleft palate and both the mandibles were separated up to canthus. Left sided ribs were also absent (o absent . On secto absent . 2 In cattle it may also be due to infection caused by bovine viral diarrhea virus or BVDV.3 Hydrallantois also occurs sporadically in dairy and beef cattle; its occurrence is very rare in small ruminants. It has been reported in cattle carrying twin fetuses and is associated with diseased uterus in which caruncles are not functional and rests of the placentomes are enlarged and diseased. However, in these cases fetuses are usually slightly smaller than normal and show some edema and ascities.1 Enlargement of some of the placentomes were also noticed in the present case. Hydrallantois with fetal defects is not reported in the available literature. Instead normal fetus was reported in these findings.1,4Hydrallantois is excessive accumulation of fluid in the allantoic sac. It may result from abnormal functioning of the placentomes due to inadequate numbers of caruncles and development of adventitial placentation.2α preparation and estrogen preparations.5,6 However, C-section is best choice when it is felt that the animal may not withstand the stress imposed by normal delivery provided that prognosis of the animals is not grave. When prognosis of the animal is grave caesarean is not recommended but may be tried to save the life of the valuable animals. Delivery of two lambs by caesarean from a ewe suffering from hydrallantois is also reported.6Various methods to induce parturition in cattle suffering from hydrallantois is reported in the literature including use of cloprostenol or other PGF1 Ultrasonography can be a very useful alternative to diagnose the condition. Moreover, rapid abdominal enlargement in last 15-20 days with round distended and tense abdominal wall are characteristic features of hydrallantois which was also noticed in the present case. Acute, progressive, bilateral abdominal distention was also observed in a ewe suffering from hydrallantois.6 In the present case it was confirmed on exploratory surgery with the presence of amber colored huge amount of allantoic fluid. Occurrence of metritis following removal of fetus and allantoic fluid was also reported earlier in cattle.1 Previous report indicated nonoccurrence of retained fetal membrane and septic metritis in ewe suffering from hydrallantois.6Hydrallantois can easily be diagnosed by rectal palpation in bovine with the findings of distorted uterine horn and no palpation/ballottement of fetus and placentomes but this is not possible in small ruminants.7 In routine handling of dystocia cases, schistosomus reflexus can easily be diagnosed by either feeling the viscera, which might be mistaken for dam’s viscera by the operator’s hand or even a beating heart. This misjudgment can be avoided by careful examination of the fetus. Characteristic findings of the monster recorded in this study were somewhat similar to that of schistosomus reflexus. However, classical schistosomus reflexus is characterized by marked ventral curvature of spine, lateral bending of fetal body and chest wall exposing thoracic and abdominal viscera with deformed pelvis, ankylosis of limbs and enclosure of head in a complete sac of skin.


We report an unusual case of a patient with multiple hepatic angiomyolipomas exhibiting high 18 F-FDG uptake on PET imaging with SUVmax ranging from 3.3–4.0. In this case, increased 18 F-FDG uptake is more likely to appear, particularly if the inflammation is predominant.A 29-year-old man with a medical history of tuberous sclerosis was admitted to our hospital for fever, vomiting, and weight loss. Abdominal dynamic computed tomography revealed faint hypervascular hepatic tumors in segments 5 (67 mm) and 6 (10 mm), with rapid washout and clear borders; however, the tumors exhibited no definite fatty density. Abdominal magnetic resonance imaging revealed that the hepatic lesions were slightly hypointense on T1-weighted imaging, slightly hyperintense on T2-weighted imaging, and hyperintense with no apparent fat component on diffusion-weighted imaging. FDG-positron emission tomography (PET) imaging revealed high maximum standardized uptake values (SUVmax) of 6.27 (Segment 5) and 3.22 (Segment 6) in the hepatic tumors. A right hepatic lobectomy was performed, and part of the middle hepatic vein was also excised. Histological examination revealed that these tumors were characterized by the background infiltration of numerous inflammatory cells, including spindle-shaped cells, and a resemblance to an inflammatory pseudotumor. Immunohistochemical evaluation revealed that the tumor stained positively for human melanoma black-45. The tumor was therefore considered an inflammatory pseudotumor-like angiomyolipoma. Although several case reports of hepatic angiomyolipoma have been described or reviewed in the literature, only 3 have exhibited high 18 F-FDG-PET in hepatic angiomyolipoma diagnosis is limited and the diagnostic value of 18 F-FDG-PET has not yet been clearly defined, the possibility that hepatic angiomyolipoma might exhibit high 18 F-FDG uptake should be considered.Although literature regarding the role of  Similar to benign tumors, which tend to exhibit low FDG uptake, hepatic AMLs have been reported to exhibit similarly low uptake levels in the absence of hemorrhage [Angiomyolipoma (AML) of the liver is a rare benign mesenchymal tumor with positive human melanoma black-45 (HMB-45) expression and a heterogeneous composition of blood vessels, and smooth muscle cells, and adipose tissue cells, which account for the tumors varying morphological features . Most AMA 29-year-old man with a medical history of tuberous sclerosis was admitted to our hospital for fever, vomiting, and weight loss (4 kg/month). His serum C-reactive protein and hepatic enzyme levels were increased. Tests for all evaluated markers of hepatic viruses and tumors such as alpha-fetoprotein, carcinoembryonic antigen, protein induced by vitamin K absence-2, and cancer antigen 19–9 were negative.Abdominal dynamic CT revealed faint hypervascular hepatic tumors in segments 5  and 6 S6; 10 mm), with rapid washout and clear borders but without definite fatty densities. CT also revealed multiple tumors with fat components in both kidneys, but these did not show definite enhancements  revealed that the hepatic lesions were slightly hypointense on T1-weighted imaging (T1WI), slightly hyperintense on T2-weighted imaging (T2WI), and hyperintense on diffusion-weighted imaging (DWI) with no apparent fat component  imaging revealed that the hepatic tumors exhibited high maximum standard uptake values (SUVmax) of 6.27 (S5) and 3.22 , and this was true for our patient. The first case of hepatic AML was described by Ishak in 1976 ,5,6,10. 18 F-FDG PET/CT is useful for detecting malignant transformation [18 F-FDG-uptake [18 F-FDG PET/CT might be highly valuable for excluding malignancies among hepatic lesions suspected to be AML.Although AMLs are considered benign, malignant transformation is not a rare complication associated with renal AML . In receormation . Moreove18 F-FDG uptake on PET imaging, with SUVmax ranging from 3.3–4.0 [We performed a Medline search using the search terms “liver”, “angiomyolipoma”, and “FDG”. Although several case reports of hepatic AML have been described or reviewed in the literature, only 3 cases exhibited high  3.3–4.0 ,15-20 (Tet al. [18 F-FDG uptake. Takanami et al.[18 F-FDG uptake in the presence of hemorrhage or an inflammatory response. In such cases, increased 18 F-FDG uptake is more likely to appear, particularly if the inflammatory tissue is predominant. Because the average mean SUV of the normal liver is approximately 3.5 ± 3.1 and the malignant lesion-to-liver SUV ratio is approximately 1.9 ± 1.4 [Kinugasa et al.  suggeste.9 ± 1.4 , the FDGRecently, some hepatic epithelioid AMLs have been reported to have features similar to those of renal AMLs reported previously. Epithelioid AMLs either lack or contain only a minimal amount of adipose tissue and are thus more difficult to distinguish from other hypervascular tumors. The imaging features of epithelioid AMLs include an absent capsule and hypervascularity with central punctiform or filiform vessels that exhibit characteristic enhancement ,23. No l18 F-FDG uptake in the lesions.The presence of HMB-45-positive smooth muscle cells in an immunostaining analysis is the definitive criterion for diagnosing hepatic AML . Althoug18 F-FDG uptake. Although the literature reports on the role of 18 F-FDG PET/CT in hepatic AML diagnosis are limited and the diagnostic value of 18 F-FDG PET/CT has not yet been clearly defined, the possibility that hepatic AML might exhibit high 18 F-FDG uptake should be considered.We report herein a case of multiple hepatic angiomyolipomas with high Written informed consent for the publication of this case report and any accompanying images was obtained from the patient. A copy of this written consent is available for review by this journal.PET: Positron emission tomography; CT: Computed tomography; FDG: Fluorodeoxyglucose; MRI: Magnetic resonance imaging; SUV: Standardized uptake values; SUVmax: Maximum standardized uptake values; T1WI: T1-weighted imaging; T2WI: T2-weighted imaging; DWI: Diffusion-weighted imaging; AML: Angiomyolipoma; HMB-45: Human melanoma black-45.The authors declare that they have no competing interests.SK, YA, and TN drafted the manuscript. SK, YA, AT, and TH contributed to the diagnosis. YA and TN reviewed the radiological findings and interpreted the data. YA conceived the study. YA, TN, and YT reviewed the manuscript. All authors approved the final version of the manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2342/14/17/prepub


The finding of an adrenal mass should induce a diagnostic work-up aimed at assessing autonomous hormone production and differentiating between benign and  malignant lesions. The common differential diagnosis in adrenal incidentaloma consists of (non-)functioning adenoma, pheochromocytoma, myelolipoma, metastasis, and primary carcinoma. There remains a category of lesions that are hormonally inactive and display nonspecific imaging characteristics. We provide a succinct literature review regarding pathologies from this category. Imaging and histological characteristics are discussed, as well as clinical management. In conclusion, an adrenal mass may present a diagnostic challenge. After exclusion of most common diagnoses, it can be difficult to differentiate between possible pathologies based on preoperative diagnostic tests. Surgical resection of possibly harmful tumors is indicated, for example, lesions with malignant potential or risk of spontaneous hemorrhage. Resection of an obviously benign lesion is not necessary, unless problems due to tumor size are expected. Clinicians may be confronted with adrenal masses in four different scenarios. The first category comprises patients presenting with endocrinological symptoms suggesting adrenal origin, such as virilization or Cushing's syndrome as seen in selected adrenocortical adenomas and carcinomas. Hypertension, flushes, and headache may be signs of pheochromocytoma or aldosterone-producing adenoma (Conn's syndrome). Secondly, patients may present with nonspecific symptoms that turn out to be caused by an adrenal tumor such as pain, fatigue, weight loss, or the sensation of an abdominal mass. Thirdly, adrenal metastases might be found in the work-up of another malignancy, for example, lung cancer. Finally, an adrenal mass may be found incidentally during evaluation for nonrelated complaints: a so-called adrenal incidentaloma.The common differential diagnosis includes six entities which account for the large majority of all adrenal masses. This will be discussed first. Secondly, we discuss a remaining category that consists of ten entities that are hormonally inactive and display nonspecific imaging characteristics.The common differential diagnosis in adrenal incidentaloma consists of nonfunctioning adenoma, functioning adenoma, pheochromocytoma, and adrenocortical carcinoma. Myelolipomas and metastases from various malignancies are also common and should be included , 2. The The diagnostic work-up should be aimed at assessing autonomous hormone production and differentiating between benign and  malignant lesions.Evaluation of cortisol and (nor)metanephrine secretion should be performed in all patients presenting with an adrenal mass, even in absence of clinical signs of Cushing's syndrome or pheochromocytoma , 58, 59.Percutaneous adrenal biopsy has high false negative rates and there is a risk of complications. Therefore, the only role of percutaneous biopsy in the evaluation of an adrenal mass is confirming metastatic disease in patients with known extra-adrenal malignancy and confirming the diagnosis of ACC when radical resection is deemed not possible , 58.There remains a category of lesions that are hormonally inactive and display nonspecific imaging characteristics, which poses a diagnostic challenge. Here we discuss individual entities from this group. A summary of imaging and pathological characteristics of these lesions is provided in Primary adrenal lymphoma (PAL) is a rare finding with less than 200 cases described in the literature. In 70–80% of cases both adrenal glands are affected , 62. On Liposarcomas account for 45% of all retroperitoneal soft tissue sarcomas. Five histological subtypes are known, of which well-differentiated liposarcomas (WDLS) and dedifferentiated liposarcomas (DDLS) are most commonly found retroperitoneally . DDLS isSchwannomas originate from Schwann cells in peripheral nerve sheaths. Approximately 3% of schwannomas are located in the retroperitoneal space, where it may involve the adrenal gland and/or mimic an adrenal mass , 24. AllTo our knowledge, there are no reports on recurrent retroperitoneal schwannoma after radical resection.Ganglioneuromas typically arise from primordial neural crest cells present in the adrenal medulla –30. CalcIdiopathic adrenal haematomas may be discovered as incidentaloma, due to abdominal complaints or due to adrenal insufficiency. Imaging characteristics vary from well-demarcated homogeneous masses to heterogeneous lesions suspect for periadrenal infiltration . AdrenalAdrenal cavernous haemangiomas are very rare and have only been described in individual case reports –39. RecuAdrenal angiomyolipomas are extremely rare with only five cases reported –44. ThesAdrenal leiomyosarcomas and epithelioid angiosarcomas are also exceptionally rare. Concise histomorphological examination combined with positive staining of specific immunohistochemical markers is necessary to confirm the diagnosis , 50. InvAdrenal cysts form a subcategory which can be divided into pseudocysts, endothelial cysts, epithelial cysts, and parasitic cysts . On CT iAn adrenal mass may present a diagnostic challenge. If a diagnosis is not established after exclusion of the most common diagnoses, a category remains that consists of rare entities. It may be difficult or even impossible to differentiate between these pathologies based on preoperative diagnostic tests. Radical surgical resection is indicated in case of possibly harmful tumors, for example, lesions with malignant potential, risk of spontaneous hemorrhage, or increase in size over time. Clinicians should assess these issues using clinical judgment complemented with radiological evaluation of the lesion, aimed at characteristics summarized in the present study. This will result in resection of benign lesions, but this is inevitable given the uncertainty that may remain after complete diagnostic work-up. Surgical resection is not necessary if a lesion is judged to be certainly benign unless the size of the lesion causes problems, for example, due to a mass effect on other abdominal organs.


Introduction. Myopericytoma is a very rare perivascular tumor that can be presented with painful mass in lower extremities. We aimed to present an atypical presentation and location of myopericytoma. Presentation of Case. An 18-year-old otherwise healthy individual was admitted to outpatient clinic with complaints of numbness and pain in his right hand. He has had no trauma. On volar aspect of his right hand, a well-circumscribed, painful mass was palpated. MRI results were related to hemangioma. Surgical excision was planned and performed. Pathological investigation revealed the mass is myopericytoma. Discussion. This case demonstrates a rare location and presentation of myopericytoma. Reviewing the literature, discussion was made to expand the horizon for diagnosis and treatment of patients with similar symptoms. Conclusion. Myopericytoma can rarely present with numbness and pain in affected region. Surgical excision is helpful for definitive diagnosis and symptom relief. Myopericytomas are perivascular benign neoplasms. These uncommon lesions are believed to arise from perivascular myoid cells . They arIn this paper, we present 18-year-old male patient with a painful mass in thenar region of his right hand. He had also numbness in ulnar side of his thumb. We aimed to discuss this atypical presentation of this rare entity in the light of the literature.An 18-year-old male patient was admitted to our outpatient clinic with complaints of ongoing pain in his thenar region and numbness of 6-month duration in ulnar side of his right thumb. In the physical examination, a 3 × 2 cm well-circumscribed, painful mass in his thenar region was seen. His past medical history and family history were unremarkable. Preoperative complete blood count, erythrocyte sedimentation rate, and C-reactive protein values were within normal ranges. Preoperative X-ray examinations were normal. Preoperative MRI demonstrated a lobulated, well-circumscribed, contrast enhanced mass, which was 24 × 20 × 16 mm in size and had low signal intensity in T1A  and highUnder tourniquet control, we perform a curved incision over the mass in the thenar region of the right hand. With meticulous dissection of soft tissues, the margins of the lesion were exposed between adductor pollicis and flexor pollicis brevis muscles. Superficial palmar arch was also exposed during the dissection. Under the lesion, we observed the compression of the lesion to the ulnar side of palmar digital nerve with some adhesions to the lesion. We excised the lesion from the nerve under microscopic dissection .The excised lesion was prepared for histopathological evaluation.Patient reported relief of pain and numbness symptoms at the 4th week of the surgery. At the last follow-up of the 14th month after surgery, the patient was well with no recurrence or symptoms.Myopericytomas are uncommon benign perivascular neoplasms that show a hemangiopericytoma-like vascular pattern , 2, 4, 5These lesions frequently arise in lower extremities, whereas they can be found in upper extremities, head and neck region, and the trunk , 4. To tAccording to the literature, preoperative MR and ultrasound investigations are found to be insufficient or misleading , 8. ExciThe histopathological examination revealed CD34 expression in endothelial cells and thin-walled vessels with a concentric, perivascular gathering of spindled myoid tumor cells as similarly described in the literature . These cRecurrent and/or malignant cases of myopericytomas are rarely reported, even though they are excised marginally or incompletely. Additionally, some local recurrences are reported in a few numbers , 10–12. Surgical excision is the preferred treatment method. Several studies show good-to-excellent results with low rate of local recurrence , 7. In oMyopericytomas are rare tumors that can show atypical presentations. Surgical excision is the gold standard for diagnosis and treatment.


We report a case of eosinophilic cystitis in a 7-year-old boy with a history of atopic symptoms, with focus on the radiological findings. He presented with hematuria and dysuria and ultrasonography (US) showed irregular bladder wall thickening resembling a bladder mass. CT urography did not characterize the lesion any further and showed no local or distant spread. Biopsies revealed eosinophilic cystitis, a benign inflammatory condition. We found that US characterized the lesion at least as well as CT and should be the first choice of imaging. When staging is considered before biopsy, MRI should be preferred to CT. There are no specific radiological signs of eosinophilic cystitis. On follow-up, US was a safe, cost-effective imaging modality, but findings should be interpreted in a clinical context. In a child with hematuria and a bladder mass, eosinophilic cystitis is a relevant but rare differential diagnosis, especially when there is a known atopic history. The aim of this paper is to draw attention to the presenting symptoms and the radiologic evaluation of eosinophilic cystitis in childhood. Eosinophilic cystitis is an inflammatory condition characterized by eosinophilic infiltration of the bladder wall. It is rare amongst children with only 59 cases in the literature to date –5. In a A 7-year-old boy was referred to a secondary pediatric centre with gross hematuria and dysuria.μmol/L (26–49 μmol/L); infection parameters were normal. It was suspected that he suffered from urinary tract infection and antibiotic treatment was initiated, but the urine culture turned out to be negative. He had elevated IgE 665 ie (<400 ie) and slight eosinophilia in the blood 0.84 109/L (<50 109/L). All other hematologic parameters were normal: hemoglobin 7.8 mmol/L (6.5–8.9 mmol/L), white blood cell count 8.5 109/L (4.5–12.5 109/L), neutrophils 4.43 109/L (1.8–8.9 109/L), lymphocytes 2.5 109/L (1.0–4.7 109/L), and platelets 337 109/L (165–435 109/L).His previous medical history included atopic symptoms , but, despite diagnostic evaluation, no asthma or allergies had been diagnosed. Physical examination revealed mild tenderness to palpation over the bladder. Urinalysis was positive for blood and protein. Blood creatinine was elevated to 56 One week later, he was readmitted due to recurrent terminal hematuria. Ultrasonography (US) showed right-sided hydronephrosis and hydroureter with distal occlusion by thickened ureter wall. Extending from the right ureter ostium, there was very irregular thickening of the posterior bladder wall, up to 1 cm, and Doppler flow within the wall . US diagHe was then transferred to a tertiary centre. A cystoscopy showed edematous mucosa with bullous excrescences from the trigonum to the right ostium and findings were very suspicious of neoplasia. A JJ catheter was inserted.The biopsies showed eosinophilic cystitis and no sign of malignancy .The patient was treated empirically with antihistamine (Desloratadine 5 mg bd) and NSAID (Naproxen 5 mg/kg bd) and recovered clinically and paraclinically within 6–8 weeks. Before that he was treated with anticholinergica (Oxybutynin 2.5 mg bd) without effect. MRI after one month showed thickening of the right ureter wall and discrete bladder wall thickening. T2-weighted sequences showed homogeneous low signal intensity of the bladder wall .US and cystoscopy after 3 months showed marked regression and biopsies showed nonspecific reactive changes. At removal of the JJ catheter after 7 months, the bladder looked near normal at cystoscopy. US showed slight irregular bladder wall thickening. One year later, US showed low bladder volume; thus, wall thickness could not be evaluated.The patient was rereferred at the age of 9 years with gross hematuria and dysuria, frequent throat clearing, epigastric stomach pain, and acid reflux. The eosinophilic blood cell count was normal and US showed slight thickening of the bladder wall of up to 5 mm . TreatmeOur patient presented with macroscopic hematuria and symptoms typical of cystitis. Urinalysis was not consistent with urinary tract infection, though. This fact should lead to the consideration of other diagnoses. Eosinophilic cystitis, though rare, is a relevant differential diagnosis, especially in a patient with a known atopic history. Macroscopic hematuria in a child can be a symptom of severe renal or urinary tract disease, and immediate evaluation is recommended by the Danish Pediatric Society . The evaA pseudotumoral appearance of (hemorrhagic) cystitis has been described in viral cystitis, being caused by chemotherapy or indwelling catheters, granulomatous cystitis, inflammatory myofibroblastic tumor, and eosinophilic cystitis , 9, 10.Benign bladder lesions in children include several rare entities such as fibromas, hemangiomas, schwannomas, hematomas, leiomyomas, endometriomas, and transitional cell papillomas.Malignant lesions include rhabdomyosarcomas (RMS), leiomyosarcomas, urothelial carcinomas, and secondary involvement such as lymphomas, with RMS being by far the most common , 11, 12.In a study of 65 pediatric patients with polyps and masses in the urinary bladder, reactive/inflammatory masses comprised 20 (31%), RMS comprised 15 (23%), and other neoplasms  22 (33%) .Sonographically, eosinophilic cystitis can present as concentric bladder wall thickening or a more focal, tumor-like appearance as in our case. Hydroureteronephrosis was seen in other cases too , 5.To our knowledge, no specific US signs of eosinophilic cystitis have been reported.Rosenberg et al. reported 17 children with benign cystitis in whom imaging mimicked RMS. They concluded that sonographic findings of isoechoic bladder wall thickening , intact mucosa, and bullous lesions strongly suggested inflammation and not malignancy. Also, changing mass contour and thickness with increasing bladder filling should be signs of inflammatory thickening .RMS has a highly variable appearance on US, but polypoid projection into the bladder lumen makes this diagnosis more likely , 9.Color Doppler US can be useful, as some differential diagnoses such as hematomas can be excluded if Doppler flow is missing.In our case, CT did not characterize the bladder lesion any further. As RMS is a potential and common differential diagnosis, however, a CT scan can be useful to assess any local or distant spread (staging). This is also well examined with MRI though , and wheTamai et al. report a case of eosinophilic cystitis in an 8-year-old girl where MRI showed distinct low signal intensity on T2-weighted images, which was suggested to represent high cellularity due to massive eosinophilic infiltration . EosinopThe normal bladder wall thickness in children depends on bladder volume. A normal bladder wall thickness of up to 1.5–3 mm (full bladder) has been found , 17. OurIn a child with hematuria and clinical cystitis, but sterile urine, eosinophilic cystitis is a relevant but rare differential diagnosis, especially when there is a known atopic history. US should be the first choice of imaging and MRI should be preferred to CT when staging is considered before biopsy. Eosinophilic cystitis can present as a bladder tumor radiologically and macroscopically and since there are no specific radiological signs, biopsy remains required. US is a safe, cost-effective imaging modality on follow-up, but findings should be interpreted in a clinical context.


Helicobacter pylori (H. pylori) infection. Primary ileal MALT lymphoma is rare, and has not been associated with a specific infectious disease. We report a case of a 58-year-old man who presented to the emergency department with constipation and abdominal distension, and signs of an obstructing mass on computed tomography scan. A small bowel resection was performed which revealed an 8 cm saccular dilatation with thickened bowel wall and subjacent thickened tan-yellow tissue extending into the mesentery. Histologically, there was a diffuse lymphoid infiltrate consisting of small atypical cells with monocytoid features. These cells were CD20-positive B-lymphocytes that co-expressed BCL-2 and were negative for CD5, CD10, CD43, and cyclin D1 on immunohistochemical studies. Kappa-restricted plasma cells were also identified by in situ hybridization. The overall proliferation index was low with Ki-67 immunoreactivity in approximately 10 % of cells. No areas suspicious for large cell or high grade transformation were identified. The pathologic findings were diagnostic of an extranodal marginal zone lymphoma involving the ileum, with early involvement of mesenteric lymph nodes. Small hypermetabolic right mesenteric and bilateral hilar lymph nodes were identified by imaging. The bone marrow biopsy showed no evidence of involvement by lymphoma. The patient was clinically considered advanced stage and opted for therapy with rituximab infusions. After six months of therapy, follow-up radiologic studies demonstrated significant decrease in size of the mesenteric lymph nodes.Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT Lymphoma) of the gastrointestinal tract commonly involves the stomach in the setting of concurrent  Helicobacter pylori (H.pylori) infection [The gastrointestinal tract is involved by 30-40 % of all extranodal non-Hodgkin lymphomas . The majnfection –3. Involnfection , 2. Whennfection . A varianfection , 7. OtheH. pylori gastritis or autoimmune process such as celiac disease, Sjogren syndrome, or Hashimoto’s thyroiditis [Helicobacter pylori infection may regress with antibiotic therapy [Campylobacter jejuni in the proximal small bowel, Borrelia burgdorferi in the skin, and Chlamydia psittaci in ocular adnexa, have also been described [Extranodal marginal zone lymphoma typically arises in areas where mucosa-associated lymphoid tissue (MALT) has been acquired, but not necessarily where it is found under normal circumstances, such as the Peyer’s patches. In these acquired areas, lymphoma is believed to arise as a result of some chronic antigenic or inflammatory stimulus such as roiditis . Gastric therapy , 8. Howe therapy . Risk fa therapy . In otheescribed . MALT lyescribed . It is aescribed .MALT lymphomas are thought to arise from the post-germinal center B-cells, normally found in the marginal zone of lymphoid follicles . These mH. pylori. Other translocations associated with extranodal marginal zone lymphoma include t , t(3:14) , and t  [The most common associated chromosomal abnormality in MALT lymphoma (particularly of gastric and pulmonary origin) is the t(11:18)  resulting in fusion of the API2 and MALT1 genes, occurring in approximately 50 % of cases , 12. Casq32;q21) . Other nq32;q21) .While extranodal marginal zone lymphoma of mucosa-associated lymphoma tissue  is one of the more common primary gastrointestinal lymphomas, a review of the literature shows very few reported cases of primary MALT lymphoma involving the ileum, occurring predominantly in Asian patients , 4, 10. A 58-year-old Caucasian male presented to the emergency department with complaints of constipation and abdominal distension. He had experienced nausea and decreased bowel movements for a week, which were not alleviated by stool softeners or enemas. There was no history of diarrhea, fever, chills, weight changes, night sweats, hematochezia, or melena. A colonoscopy 4 years prior had only revealed a small benign polyp. The patient otherwise had no significant past medical history and was not taking any medications. No history of abdominal surgeries or significant family history of cancer was elicited. He denied the use of alcohol or smoking, but admitted to using smokeless tobacco. Vital signs and physical exam were essentially unremarkable. The abdomen was not visibly distended and normal bowel sounds were present. No hepatosplenomegaly or lymphadenopathy was appreciated. Laboratory results showed normal complete blood count, coagulation parameters, electrolytes, liver function tests, pancreatic enzymes, and kidney function. Alpha fetoprotein (AFP) and carcinoembryonic antigen (CEA) were within normal limits. An abdominal computed tomography scan (CT scan) was concerning for a neoplasm causing small bowel obstruction, showing mid to distal small bowel dilatation with abnormal wall thickening and significant retained debris. The patient underwent a laparotomy and resection of a distal segment of ileum was performed.On gross examination, a 39 cm portion of distal ileum was received and the central portion contained an 8 cm length saccular dilatation that had a hemorrhagic and nodular mucosa Fig.  and b. TThe large ileal mass in the setting of small mesenteric and hilar lymph nodes was clinically consistent with a diagnosis of extranodal marginal zone lymphoma arising from the ileum. A complete staging evaluation including PET and CT imaging studies revealed additional small hypermetabolic mesenteric and bilateral hilar lymph nodes. No other lesions were noted in the stomach, thyroid, liver, pancreas or other organs. A staging bone marrow biopsy was negative for involvement by lymphoma, by morphologic and flow cytometric analysis. The patient was clinically staged as 3E (lymphadenopathy above and below the diaphragm). Treatment was initiated with single agent rituximab  every other month, and is planned to continue for 2 years. After six months of treatment, the patient was asymptomatic and radiologic studies demonstrated significant decrease in size of the mesenteric lymph nodes.Small bowel obstruction in industrialized countries is usually due to adhesions, which account for approximately 70 % of cases . Other cCampylobacter jejuni infection [Helicobacter pylori infections seen in gastric MALT lymphomas. This is an unusual case in which extranodal marginal zone lymphoma presented as ileal obstruction, in a patient with no known history of a chronic inflammatory or autoimmune disease.Diffuse large B-cell lymphoma is the most common primary gastrointestinal tract lymphoma, with the small bowel as the site of origin 15–30 % of the time , 7. IPSIIn conclusion, this case represents a rare case of extranodal marginal zone lymphoma involving the ileum presenting as small bowel obstruction. Further studies are required to understand the etiology of ileal MALT lymphomas, with possible identification of causative agents and better understanding of pathogenesis of this malignancy. Eradication of treatable infectious organisms may help in preventing the occurrence of ileal MALT lymphomas in the future. Appropriate identification of these cases is important for early intervention, and proper management that includes targeted agents and chemotherapy, with possible avoidance of surgical procedures.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in –chief of this journal.


Cluster headache belongs to a group of primary headache entities: the trigeminal autonomic cephalalgias. Cluster headache is the most common variant. The headache is usually severe and it is also associated with autonomic symptoms. Secondary causes of cluster headache have been reported, such as intracranial artery aneurysms and tumors. The question of when to carry out neuroimaging in patients with cluster headache is yet unsettled. To the best of the author's knowledge, cluster headache associated with a clinically non-functioning pituitary adenoma (chromophobe adenoma) has not been described. This case report describes the case of a man with cluster headache where the evaluation showed a clinically non-functioning pituitary adenoma.This case involved a 49-year-old Caucasian man who presented with a one-month history of side-locked attacks of pain located in the right orbit. His symptoms fulfilled the criteria for cluster headache and a diagnosis of cluster headache was made. The patient responded to symptomatic treatment. Enhanced magnetic resonance imaging showed a pituitary adenoma. Further evaluations including hormonal screening revealed a clinically non-functioning pituitary adenoma (chromophobe adenoma). After surgery to remove the tumor, his headache attacks resolved totally.Tumors have been reported in patients with cluster headache whose clinical attacks are identical to genuine cluster headache. A clinically non-functioning pituitary adenoma can present as cluster headache. This case emphasizes the need of imaging procedures in patients with cluster headache. Contrast-enhanced magnetic resonance imaging including the sella turcica should always be done in patients with cluster headache. Cluster headache (CH) belongs to a group of primary headache entities, the trigeminal autonomic cephalalgias. CH is the most common variant. Studies have demonstrated that CH has a lifetime prevalence of 0.12%. The headache is usually severe and is associated with autonomic symptoms. CH is more common in men and typically manifests itself between the third and fifth decades of life. The question of when to carry out neuroimaging in patients with CH is yet unsettled. Secondary causes of CH have been reported in many cases, such as intracranial artery aneurysms and tumors (symptomatic CH) [A 49-year-old Caucasian man presented with a one-month history of side-locked attacks of excruciatingly severe stabbing and boring right-sided pain located in his orbit. The attacks were associated with nasal obstruction, conjunctival injection, restlessness and migrainous features such as nausea, photophobia and phonophobia. No continuous background pain was identified. The duration of the attacks was about 20 to 30 minutes with a frequency of two to three per 24 hours. The attacks were stereotypical, being exclusively right-sided with severe agitation during the pain. He had no previous history of headache. His medical and family history was otherwise unremarkable. He was not on any medications and used no drugs. His vital signs, a physical examination and a neurological examination were normal. Laboratory testing was normal. His symptoms fulfilled the revised International Classification of Headache Disorders criteria for CH.A diagnosis of CH was made and subcutaneous sumatriptan as well as high-flow oxygen were prescribed. A prophylactic treatment with verapamil was prescribed. He responded to the symptomatic treatment, with relief within 15 minutes. A follow-up was planned. Because the headache attacks continued, the patient was seen again after two weeks. On arrival, a neurological examination was normal. His visual fields were normal. He was taking subcutaneous sumatriptan when required and verapamil 600mg daily. Owing to the relatively late onset of CH, enhanced magnetic resonance imaging (MRI) was ordered to rule out an underlying lesion. It showed a rather large pituitary tumor (20×21×17mm). No clear cavernous sinus invasion could be demonstrated . Hormonal screening showed no abnormalities: pituitary hormones such as prolactin, insulin-like growth factor 1 and growth hormone, thyroid-stimulating hormone and others were within the normal ranges. His headache attacks resolved totally after surgery. He remained headache-free and had not experienced any headache attacks at follow-up after 17 months. The patient is regularly followed up in our endocrine clinic.This case study involved a patient with CH responding to treatment. An assessment revealed a clinically non-functioning pituitary adenoma. The patient became headache-free after treatment and remained so at follow-up.et al. [et al. [et al. [Tumors have been documented in patients with CH. The symptoms may be in line with the criteria for CH , 4. Pituet al.  observed [et al.  describe [et al.  found thet al. [The etiology and pathophysiology of CH are unknown. The current hypothesis proposes that primary CH is characterized by hypothalamic activation with secondary activation of the trigeminal-autonomic reflex, probably by a trigeminal-hypothalamic pathway . In thiset al.  point toet al. . There aet al. , 10. ThiHowever, there are studies that contradict this explanation. These studies have failed to show an association between cavernous sinus invasion or tumor volume and headache , 11. ThuSymptomatic CH should be suspected when the clinical features of the headache are atypical and in patients with pathological findings on neurological examination . CittadiTo the best of the author's knowledge, this is the first report showing an association between CH and a clinically non-functioning pituitary adenoma (chromophobe adenoma). All previous reports have shown an association between CH and prolactinomas or a growth hormone-producing adenoma. CH may be the presenting symptom of a chromophobe adenoma of the pituitary. Symptoms and objective signs of pituitary gland or pituitary region disease are not always present, as in this case. Consequently, the absence of pituitary symptoms or disease does not exclude a symptomatic CH. Tumors have been reported in patients with CH with clinical attacks identical to genuine CH. This case emphasizes the need for imaging procedures in patients with CH. Contrast-enhanced MRI including the sella turcica should always be done in patients with CH.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Congenital jejunal stenosis and gastric duplication cysts are very rare congenital anomalies of the gastrointestinal tract in the newborn. We present a case of congenital membranous jejunal stenosis associated with gastric duplication cysts, which was diagnosed by ultrasonography. To the best of our knowledge, this is the first report of ultrasonographic diagnosis of congenital membranous jejunal stenosis associated with a gastric duplication cyst in a newborn.A 1-month-old Chinese baby girl presented with projectile vomiting and hyperpyrexia for 3 days. An upper gastrointestinal contrast study showed incomplete duodenal obstruction; however, ultrasonography revealed congenital membranous jejunal stenosis associated with a gastric duplication cyst. After surgical excision of the jejunal membrane and gastric duplication cyst, she recovered well with no bilious vomiting at the 1-year follow-up.Ultrasonography is a useful tool for the evaluation of membranous jejunal stenosis and the identification of small, asymptomatic gastrointestinal duplication cysts. Congenital jejunal stenosis and gastric duplication cysts are very rare congenital anomalies of the gastrointestinal (GI) tract in the newborn; the prevalence rate of jejunoileal atresia and stenosis has been reported to be between 1 in 330 and 0.9 in 10,000 live births, and stenosis occurs in 7 % of cases , and gasA 1-month-old Chinese baby girl presented to our emergency department with vomiting and high-grade fever, which had occurred for 3 days. The fever was not associated with rigors and chills. She presented with a history of intermittent projectile and bilious vomiting after breastfeeding. Vomiting occurred approximately five times each day, at a volume of 10mL at each instance. A general physical examination revealed a temperature of 39 °C, pulse rate of 100/minute, and respiratory rate of 30/minute. On abdominal examination, no abdominal distention, regional tenderness, or rebound was observed, and no masses or viscera could be palpated. Bowel sounds were audible, and other physical examination results were normal. An upper GI contrast study in another hospital showed incomplete duodenal obstruction Fig. , and theIn general, the symptoms of patients with congenital anomalies of the alimentary tract present early after birth with recurrent, progressive vomiting. Bilious vomiting indicates obstruction distal to the ampulla of Vater , which rDuplication cysts are rare congenital anomalies that can occur in any part of the GI tract, although the most common site is the ileum . GastricUsing ultrasonography, we successfully diagnosed congenital membranous jejunal stenosis associated with a gastric duplication cyst in a newborn. This case emphasizes that ultrasonography in a baby with congenital jejunal stenosis was effective as a diagnostic modality. In addition, ultrasonography can be helpful for the accurate identification of small and asymptomatic GI duplication cysts. Ultrasonography is superior to radiography for revealing the cause of some intestinal obstructions, suggesting that babies with bilious vomiting should have a routine ultrasound for diagnosis.Written informed consent was obtained from the patient’s legal guardian for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Colonic solitary plasmacytoma is a rare disease, with few reports occurring in the literature. Solitary plasmacytoma is defined as a plasma cell tumour with no evidence of bone marrow infiltration. Plasmacytoma can present as a solitary tumour in bone or in other parts of the body. The gastrointestinal tract is rarely the site of the disease. We report on the case of a 51-year-old man presenting with a colonic symptomatic mass with unclear biopsy results. A resected specimen showed a solitary plasmacytoma. Surgical resection was an adequate treatment modality in this case. Endoscopic resection, radiotherapy, and chemotherapy are also preferred treatments in selected gastrointestinal plasmacytoma cases. Solitary plasmacytoma (SP), a monoclonal proliferation of plasma cells without evidence of significant bone marrow plasma cell infiltration, occurs in two main forms: bone SP (P-bone) and extramedullary SP (P-extramedullary). SP is a rare disease and accounts for about 10% of plasma cell tumours . InvolveA 51-year-old man presented with 6 months' history of abdominal pain, changes in bowel habits, bloody diarrhoea, and a 10 kg loss of weight. No palpable mass was found in the abdomen during physical examination. Liver and kidney function tests, the carcinoembryonic antigen (CEA) level, and the complete blood count were normal except for a measured haemoglobin (Hb) level of 9.3 gr/dL. His ultrasonography (USG) examination showed a 9.5 cm long annular mass at the cecum and the ascending colon. An abdominal computed tomography (CT) scan showed a 3 cm wide and 10 cm long annular mass with medial extension into the fatty tissue at the same localization. The colonoscopy revealed a constricting ulcerovegetative mass localized at the medial border of the cecum in close proximity to the ileocecal valve extending into the ascending colon . A biopsHistopathological examination of the resected colon showed atypical plasmacytoid cells staining Kappa light chain positive and Lambda light chain negative. The Ki-67 proliferation index was 40%. CD43, CD3, and CD20 staining were negative, while that for CD38 was positive. The tumour was reported as a malign plasma-cell neoplasia . None ofPlasmacytomas, first described by Schridde in 1905 , are cloThe incidence of P-bone is approximately 40% higher than that of P-extramedullary. The male-to-female ratio is somewhat higher for P-extramedullary than for P-bone. Nearly 30% of P-extramedullaries are of respiratory origin, and 24% occur in the mouth orthopharynx. P-extramedullaries rarely occur in the GI tract, stomach, small bowel, colon, or rectum. SP usually presents in the 5th or 6th decades [Diagnostic criteria for SP have varied over time. Both P-extramedullary and P-bone should be basically associated with a normal bone marrow . All of The presentation of gastrointestinal plasmacytoma (P-GI) is different from that occurring at other sites in the body. The most common presenting symptom is abdominal pain. Other symptoms can include rectal bleeding, a change in bowel habits, weight loss, nausea, vomiting, large bowel obstruction, and intussusception . P-GIs aIn general, P-extramedullaries are considered to be radiosensitive, with a local control rate of 90–100% . AllisonIn only one randomised trial of chemotherapy, patients receiving melphalan and prednisone showed an improved disease-free survival rate . SusnerwP-extramedullary patients characteristically present with localized disease, and the incidence of lymph node involvement is 10–20%. The overall 10-year survival rate is ~70% . SurvivaP-extramedullaries are rarely seen in the GI tract. It is important to consider this possibility during the evaluation of a mass in the GI tract because P-GIs have different treatment and follow-up modalities than adenocarcinomas and other tumours of GI. Surgical treatment is usually sufficient in localized P-C. In our case, with localized disease and no lymph node involvement, there has been no recurrence or MM progression in three years.


Pseudomyxoma peritonei is characterized by mucinous ascites originating from a mucin-producing neoplasm; however, even the definition is still under debate. Tumor deposits extend and ultimately engulf the entire cavity, causing death from cachexia due to limited intestinal movement. Here, we report a unique case of an 80-year-old woman with pseudomyxoma peritonei, which extended to the lower extremity mimicking infectious condition. The patient survived for a long time without bowel obstruction despite having the histologic subtype that has an unfavorable prognosis. The extremity lesion was treated with limited extensive surgery. The origin of the disease and the mechanism of extension to the extremity could not be clarified. Clinicians should be aware of the original disease entity and this unusual presentation and determine its mechanism and the best management strategy. Pseudomyxoma peritonei (PMP) is a clinical syndrome characterized by mucinous ascites that result from rupture of a mucin-producing neoplasm –3. HowevTo the best of our knowledge, no case of PMP extension to the lower extremity has ever been reported. Therefore, the presentation, mechanism, and complications are unknown, and the management strategy, including the appropriate surgical margin and adjuvant treatment for the extremity lesion, are undetermined. In this paper, we share our experience with this rare presentation.An 80-year-old woman presented to our institution with odorous discharge from an opening in her swollen right thigh. In the previous 4 years, her right thigh had swollen up. Three months prior to her visit, an opening formed spontaneously at the medial aspect of the thigh, and jelly-like material began to ooze steadily. Two months before her visit, an odorous discharge began to exude from the opening. Physical examination revealed fluctuation in the right lower quadrant of the abdomen, the medial aspect of the right thigh, and the posterior aspect of the right calf.Fourteen years previously, the patient had undergone an operation for an intra-abdominal mass at another institution. The operation record mentioned a bilateral salpingo-oophorectomy with massive adhesiolysis and excision of a large 20 × 19 × 18-cm mass in the retroperitoneal space. There was no mention of the appendix in the record, and the patient had not undergone appendectomy before the operation. The pathological record reported a mucinous borderline left ovarian tumor with extensive pseudomyxoma ovarii and severe tubo-ovarian adhesion. Although the patient had noted progressive abdominal expansion with dull pain in the right lower quadrant 8 years previously, diagnostic studies such as computed tomography (CT) and ultrasonography (US)  were not3, 87.5 % segmented neutrophil, an erythrocyte sediment rate of 96 mm/h, 5.89 mg/dL C-reactive protein, 178 mg/dL fasting blood sugar, 4.5 g/dL total protein, 2.4 g/dL albumin, and 50 U/L aspartate transaminase. The urine analysis and sediment examination noted abnormal results including leukocytosis, a positive occult blood, and WBC and red blood cell count of 30 to 49 and 20 to 29 per high-power field.A magnetic resonance image of the right thigh showed a fluid-containing lesion in contact with the surface and an opening in the medial compartment. In addition, there was a multiloculated, lobulated lesion mainly involving the anterior and medial compartment of the thigh and extending distally along the fascia of the hamstring muscles Fig , b. A maImmediately after admission, we started intravenous antibiotic administration, which included 2.25 g of piperacillin and tazobactam four times a day and 1 g vancomycin once every 96 h. Two weeks after admission, a suspected infected portion of the thigh lesion was excised, and tracks connecting from the thigh to the peritoneum were ligated. After the operation, administration of 2.25 g of piperacillin and tazobactam four times a day was continued. Staphylococcus epidermidis grew when mucinous material obtained intra-operatively from the lesion was cultured. Pathological examination of the excised specimen demonstrated acellular mucinous materials with a carcinoembryonic antigen-positive immunoreaction Fig .Fig 2Pat3, 51.8 %, 11 mm/h, 1.09 mg/dL, 112 mg/dL, 5.7 g/dL, 2.9 g/dL, and 24 U/L, respectively. On urine analysis and sediment examination, the WBC and red blood cell count were 1 to 3 and 4 to 9, respectively, per high-power field.Two weeks after the first surgery, follow-up blood test results demonstrated improvement of the infectious condition in the thigh. The WBC count, segmented neutrophil, erythrocyte sediment rate, C-reactive protein, fasting blood sugar, total protein, albumin, and aspartate transaminase were 4640 /mmA second operation was performed for all the lesions from the abdomen to the calf by a collaborative team of orthopedic surgeons and gynecologists. No bowel obstruction was observed, and the appendix was not seen. A large volume of mucinous material was suctioned from the abdominal lesion Fig . MultiplFive days after the second surgery, no bacterial growth was observed in a culture of intra-operatively obtained mucinous material. Pathological examination showed low-grade mucinous carcinoma peritonei with the lining epithelium in the intra-abdominal lesion Fig  and onlyThe origin of PMP is still controversial. PMP is currently thought to be associated mainly with mucinous epithelial neoplasms of the appendix –9. HowevPMP usually manifests with tumor deposits throughout the entire peritoneal cavity. This characteristic pattern of dissemination can be explained by the redistribution phenomenon, which is associated with the intra-peritoneal fluid current and gravity . In the CT and US have been reported to be useful in detecting PMP , 17, 18.In 1995, Ronnett et al  describeAlthough the intra-peritoneal lesion in this case was diagnosed as low-grade PMCA and optimal treatment was not performed for a long time, our patient did not report any symptoms associated with bowel obstruction. Despite a huge lesion, her clinical course was modest over a long period, and death by cachexia was avoided. We assume that the extension of the intra-peritoneal lesion to the lower extremity acted as an expansion of the peritoneal cavity and prevented such an outcome. When PMP is progressing, the characteristic visceral and mesenteric sparing of PMP becomes visible intra-operatively and is related to a favorable prognosis after surgery , as in oThe proper treatment for PMP remains uncertain; however, the combination of aggressive cytoreductive surgery and hyperthermic intra-peritoneal chemotherapy seems to improve the outcome . NeverthAlthough the cause of PMP extension to the lower extremity has an important clinical significance, we could not explain why the extension occurred only in this case. The fluidity of mucinous contents and the gravity might have contributed to the formation of the lower extremity lesions as an extension of tuberculous psoas abscess into the thigh. Further studies to elucidate the mechanism are required.We reported a rare case of PMP extension to the lower extremity. Clinicians should understand the original disease entity of PMP, be aware of this unusual presentation and complication, and determine the best management strategy by balancing the benefits against possible morbidity. We also expect further studies to elucidate the mechanism of PMP extension to the lower extremity.Written informed consent was obtained from a legal guardian of the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor in chief of this journal.


Renal cell carcinoma represents 3 % of all cancers. Around 4–10 % of cases present with inferior vena cava involvement, generally with tumor thrombus. Clinical and preoperative stage will be classified depending of the thrombus extension. A high quality preoperative workup is essential to properly plan surgical approach. Complete surgical resection of the tumor is potentially the only curative treatment, although it supposes a real challenge due to operative difficulty, potential for massive bleeding or tumor pulmonary thromboembolism. Surgery includes techniques derived from transplantation surgery and, in some cases, cardiovascular intervention with cardiopulmonary bypass. Long-term oncological outcomes after complete removal of the entire tumor burden are acceptable. In this report we describe step-by-step surgical maneuvers depending on the thrombus lever, and focusing in complete abdominal approach for the complete excision of the tumor. Moreover, a recent literature review about oncological results is reported. Renal cell carcinoma (RCC) represents around 3 % of all cancers, with an incidence of 84,400 new cases per year in Europe IV: right atriumThe most crucial element in preoperative workup is to determine the level of the tumor thrombus Fig. . In addiTransesophageal echocardiography (TEE) is widely recommended for a correct staging of the level of the thrombus, especially for cases level III or IV. Although its use is sometimes avoidable in the preoperative workup, it will play an essential role during surgery, allowing the anesthetist and surgeon to have an intraoperative control of the thrombus, overall during clamping and thrombectomy steps. Moreover, it can help to early diagnose a potential tumor thromboembolism.Patient is placed in supine with extension of the lumbar lordosis in order to get a correct access to both subdiaphragmatic spaces. To gain optimal surgical exposure, we perform a bilateral subcostal incision (chevron) 3–4 cm below the costal margin, which sometimes it is extended in the midline vertically up to xiphoid process (triradiate incision). Rochard retractor is placed  draining in IVC above renal vein, contradicting classical anatomic texts assertion, and this unexpected vein was an unrecognized source of massive bleeding during surgery . This technique is often feasible, especially when early ligation of renal artery was performed, because it reduces blood supply to the tumor thrombus. For level IIId thrombus, and for those cases when milking maneuver is not feasible, dissection continues until the supradiaphragmatic and intrapericardial IVC, which is dissected circumferentially.Once with IVC dissected, thrombectomy is performed previous correct sequential vascular clamping: Pringle maneuver, IVC below the thrombus, contralateral renal vein, right adrenal vein if needed, and IVC above the thrombus (below suprahepatic veins if milking maneuver was successful or around supradiaphragmatic IVC if not). Cavotomy starts through affected renal vein and is extended proximally as much as needed. Tumor thrombus is removed completely, including adherent thrombus to IVC wall with sharp dissection. Major hepatic veins and IVC wall can be directly visualized; it allows complete removing of thrombus in their ostia. IVC closure is done with 2 Prolene 4-0 running sutures.As previously commented, cases requiring hepatic ischemia and IVC clamping above hepatic veins have surgical disadvantages, due to intraoperative venous return decrease and to postoperative hepatic dysfunction. Thus, the less time the ischemia is established, the less risk of perioperative complications. That is the reason why we routinely recommend replacing clamps after thrombectomy. Once the thrombus is removed, a vascular clamp is positioned immediately above the proximal segment of the cavotomy, so the suprahepatic clamp and hepatic hilium clamp can be safely removed and then hepatic circulation reestablished.Early surgical approach of level IV thrombus relied on cardiopulmonary bypass (CPB). It can produce platelet dysfunction and coagulopathy . Median survival for patients with RCCIVCT not undergoing treatment is 5 months (Reese et al. Most controversial question regarding predicting factor of RCCIVCT is if the level of the thrombus is an independent factor of oncological outcomes. Several studies reported conflicting data. Recently, the largest multi-institutional and international cohort reported by Martinez-Salamanca  with morRadical surgery is the mainstay of treatment for patients with RCCIVCT. And adequate preoperative imaging is essential. Surgical maneuvers and techniques derived from liver transplantation provide excellent exposure and control of the IVC, avoiding the use of CPB in many cases. Level of the thrombus seems to be a survival independent predictor. Successful surgery may provide acceptable long-term survival rates and better quality of life. New targeted therapies may play a promising role as adjuvant therapy.


STK11 mutation. Immunohistochemistry analysis of the tumor showed decreased expression of STK-11 as compared to one of the patient’s hamartomatous polyps. Next generation sequencing of the tumor specimen failed to demonstrate a “second hit” somatic mutation in STK-11. This case represents the first case of endometrial stromal sarcoma associated with PJS and illustrates the importance of increased awareness of this condition among oncologists. PJS is associated with dysregulation of the mTOR pathway; treatment with an mTOR inhibitor was not effective in this case.A 46-year-old female presents with a pelvic mass and is diagnosed as having a high-grade endometrial stromal sarcoma. During surgery, she is noted to have areas of intussusception of the small bowel secondary to large hamartomatous polyps. The patient had a previous history of small bowel obstruction secondary to what had been thought to be hyperplastic polyps but represented hamartomatous polyps on further review. Additional examination revealed the presence of subtle hyperpigmented macules on the fingers leading to a diagnosis of Peutz-Jeghers Syndrome (PJS). The diagnosis was confirmed by the presence of a germ-line The online version of this article (doi:10.1186/s13053-015-0027-0) contains supplementary material, which is available to authorized users. Peutz-Jeghers syndrome (PJS) is a rare, inherited disease, with an estimated incidence of one in 100,000. It is an autosomal dominant condition characterized by the development of hamartomatous polyps throughout the gastrointestinal tract , characteristic mucocutaneous pigmented lesions and elevated cancer risk .Polyps may cause gastrointestinal bleeding, intussusception, obstruction, or infarction ,4]. The. The4].  ..7].PJS has not been associated with soft tissue sarcomas. In this article, we report the first case of a Peutz-Jeghers Syndrome patient with an endometrial stromal sarcoma.cul-de-sac and pelvic retroperitoneum. Pathology was most consistent with a high grade endometrial stromal sarcoma. Adequate surgical cytoreduction and staging with hysterectomy, bilateral salpingo-oophorectomy, omentectomy, nodal sampling, and peritoneal washings were performed. No additional areas of tumor involvement were noted; specifically, there was no gross evidence of malignancy involving the uterus, fallopian tubes, or ovaries.A 46-year-old woman presented with sensation of pressure in her pelvis. A CT scan of the abdomen and pelvis showed an 8 × 7 cm pelvic mass. Serum CA 125 level was 280 U/ml and serum CEA value was within normal limits. The patient underwent an exploratory laparotomy with findings of an 8 cm pelvic mass thought to be associated with an area of endometriosis with involvement of the pelvic During inspection of the small intestine, an area of intussusception was noted, with resection revealing the presence of two large pedunculated polyps (3.2 and 3.0 cm) as the cause of the intussusception. Suspicion of PJS was raised by intraoperative consultation with medical genetics based on the presence of pedunculated small bowel polyps and additional findings on physical examination of the patient, which included multiple hyperpigmented areas on her fingertips Figure A.Figure cul de sac peritoneum were involved by tumor, and foci of endometriosis were present. Lymph nodes were negative for neoplasm . In addition to the two larger polyps, six other small bowel polyps (0.4 – 2.2 cm) were found and resected via small bowel enterotomies; pathology on all polyps was significant for hamartomatous polyps of Peutz-Jeghers type. A Peutz-Jeghers type polyp is a hamartomatous polyp with smooth muscle arborization with occasional pseudo-invasion. This patient had three polyps removed from the large bowel a month before presenting to our institution; all three polyps were considered to be hyperplastic by an outside pathologist. Upon review of these polyps at our institution, it was determined that all three polyps were hamartomatous with one consistent with the Peutz-Jeghers type. Full gene sequencing and deletion/duplication analysis of the STK11 gene was completed so that predictive testing could be offered to the patient’s daughter; this genetic testing was not needed to make the diagnosis of PJS in our patient. The diagnosis of Peutz-Jeghers syndrome was further confirmed with the identification of a deleterious mutation in the STK11 gene, designated as 5’UTR_EX1del.Pathological examination showed a 9.5 cm malignant neoplasm consistent with high-grade endometrioid stromal sarcoma with a predominant epithelioid component Figure A-F and aThe patient received radiation therapy to the pelvis followed by chemotherapy consisting of ifosfamide and doxorubicin for 4 cycles. A repeat CT scan at the end of chemotherapy showed subcapsular liver nodules suspicious for metastasis. The patient was then started on letrozole, with development of progressive disease on this treatment. She developed small bowel obstruction requiring surgery with findings of high-grade small bowel obstruction requiring small bowel resection and multiple metastatic nodules involving the right hemi-diaphragm largest 3 cm in size and the transverse colon mesentery, largest 5 mm in size. Pathology showed a poorly differentiated neoplasm with a predominant epithelioid component and a minimal spindle cell component. The epithelioid component was pankeratin positive, with patchy EMA positivity, patchy CK8/18 positivity and patchy membranous CD99 positivity. Tumor cells were ER positive (60%) and PR positive (5-10%) and were negative for inhibin, melan A 103, desmin and SMA. The spindle cell component had a myxoid morphology with patchy faint positivity for SMMS-1. Subsequent treatment with everolimus and anastrozole and later on with pazopanib and everolimus, and gemcitabine and docetaxel, was not associated with a clinical response. The patient had progressive disease leading to her death.Additional genetic and immunohistochemical (IHC) analysis of the tumor specimens were performed. Tumor DNA was extracted from formalin fixed paraffin embedded tissue. STK11 was sequenced using custom sequence capture with targeted next generation sequencing. IHC of the hamartomatous polyps and the sarcoma tumor from paraffin embedded tissue was done utilizing human LKB1/STK11 Affinity Purified Polyclonal Antibody (R & D Systems).STK11 tumor sequencing findings were consistent with the patient's known germline deletion of exon 1. A "second hit" somatic tumor mutation was not identified. IHC studies demonstrated STK-11 expression in the hamartomatous polyp with decreased expression of STK-11 in the sarcoma tumor tissue Figure .Figure 3PJS patients have a higher risk for malignancies at a young age, especially colorectal, breast, gynecologic, small bowel, gastric, and pancreatic cancer. The relative cancer risk by site is estimated to vary between 4.8 to 18 times that of the general population, with a lifetime cumulative cancer risk up to 93% . The risClinical suspicion for PJS needs to remain high in order to make the diagnosis. The hyperpigmented melanotic lesions may be missed since they may be few in number, fade with age, and present most prominently on the fingers and in the oral cavity without being noticeable on the lips as in our patient Figure A-B. The The most common ovarian neoplasm is the sex cords tumor with annular tubules (SCTAT), which is typically multifocal, calcified, and bilateral. Approximately 10% of patients with PJS will develop SCTATs that require surgery. These tumors are usually of a low malignant potential and carry a good prognosis ,9]..9].Adenoma malignum (ADM) is a rare variant type of highly differentiated adenocarcinoma of the endocervical glands that comprises 1-3% of all cervical adenocarcinomas; it can be aggressive and have a poor prognosis despite its benign histologic appearance. The percentage of patients with PJS who develop ADM is 5% or less, and about 10% of patients with ADM have PJS ,[10].Sarcomas are not usually associated with PJS. There is only one case report of an epithelioid leiomyosarcoma originating in a small bowel hamartomatous polyp in a patient with PJS. The tumor showed an aggressive behavior and by the time of resection, it had metastasized to the liver. This is very unusual since malignant transformation of the hamartomatous polyps is rare . There aSTK11 mutation. The lack of a "second hit" does not disprove that the patient's tumor was related to her PJS diagnosis. "Second hit" somatic mutations are only found in about 1 in 5 samples from PJS intestinal polyps [We attempted to identify a "second hit" somatic tumor mutation to conclusively link the tumor to the patient's PJS diagnosis and germline l polyps . ExplanaSTK11 gene mutations in PJS have allowed for the development of targeted molecular therapy. STK11 mutations are associated with activation of the mTOR pathway. mTOR inhibitors have been developed for clinical use in organ transplantation and treatment of renal cancers. The mTOR inhibitors have been reported in the treatment of a PJS patient with pancreatic cancer and could possibly serve as a second line treatment for patients who have failed to respond to other therapies [Identification of herapies . The proWritten conformed 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.


Solitary plasmacytomas (SPs) represent ≤5% of all plasma cell neoplasms and mostly occur in the spine, pelvis, ribs and pectoral girdle, while rarely occurring in the sternum. The tumors typically appear as osteolytic lesions. In rare cases, SPs can manifest as bony spicules on the surface of the bone. The present study reports the case of a 74 year-old female with an osteolytic tumor localized in the sternum. The tumor displayed extensive bony destruction, with a large quantity of thick straight spicules on the surface of the bone, resembling a sunray in appearance. The imaging, laboratory and pathological examinations of the patient met the diagnostic criteria of SP. The patient was initially treated with radiotherapy at a dose of 45 Gy. Six months later, chemotherapy consisting of vindesine, Adriamycin and dexamethasone was administered. Vindesine and Adriamycin were administered at a dose of 2 and 15 mg/day, respectively on days 1–4 in a 20-day cycle. Dexamethasone was administered at a dose of 20 mg/day on days 1–4, 9–12 and 17–20 in the 20-day cycle. In total, the patient underwent 6 cycles of chemotherapy, with a total duration of 7 months. The patient was followed-up for two years after beginning therapy. At present, the patient is well, without any evidence of progressive disease or multiple myeloma. To the best of our knowledge, this is the first case in the English literature of SP in the sternum, with an unusual sunray periosteal reaction on radiological imaging. The sites of bony spiculation in the lesions that have previously been described in the literature are the mandible, orbit, vertebral body and skull vault. To the best of our knowledge, the current study presents the first case of a SP of the sternum with a unusual spiculated periosteal reaction on radiological imaging to be reported in the English literature. Solitary plasmacytomas (SPs) are rare and are characterized by a localized accumulation of neoplastic monoclonal plasma cells, without proof of systemic myelomatosis. The tumors represent ≤5% of all plasma cell neoplasms  and mostThe current study presents a case of a solitary plasmacytoma of the sternum with a spiculated periosteal reaction on radiological imaging. Written informed consent was obtained from the patient.A 74-year-old female presented with neck and anterior chest wall pain, along with swelling in the chest area that had persisted for one month. The past medical history consisted of type II diabetes mellitus and hypertension. There was no history of cancer. A physical examination revealed an ill-defined swelling on the anterior chest wall. The area was tender and no pulsation was noted. Routine urinalysis and hematological and biochemical tests were within the normal limits. A urine test for Bencae Jones protein was negative.99mTc-labelled whole-body bone scintigraphy disclosed normal results, with the exception of increased uptake in the sternum.Conventional radiography of the chest was normal. On the axial and sagittal computed tomography (CT) scans, the manubrium and body of the sternum had extensive bony destruction, with a large quantity of thick straight spicules on the surface of the bone resembling a sunray in appearance. The lesion had slight expansion and disruption of the cortex in certain places and had spread to the adjacent soft tissues. There was no calcification in the lesion . The tumIn order to obtain the correct diagnosis, an open biopsy was performed at the sternum. The histopathological examination demonstrated that the destroyed bone marrow had been substituted with abundant neoplastic plasma cells with eccentric ‘clock-face’ nuclei and copious cytoplasm. Immunohistochemical staining was positive for immunoglobulin (Ig) λ- and κ-light chain antibodies, and cluster of differentiation (CD)38 and -138 . The hisThe patient was initially treated with radiotherapy at a dose of 45 Gy delivered in 15 fractions over 21 days. Half a year later, chemotherapy consisting of vindesine , Adriamycin  and dexamethasone  was administered. In total, the patient underwent 6 cycles of chemotherapy, with a total duration of 7 months. The patient was followed for two years after beginning therapy, without any signs of progressive disease or conversion to multiple myeloma.The association between SP and multiple myeloma is currently unclear. Certain individuals regard SP as a distinct entity from multiple myeloma, while others regard SP as an early presentation of multiple myeloma . The diaSternal plasmacytoma usually presents as an osteolytic expansile lesion or a typical ‘punched-out’ lytic lesion on radiological examination . The tumet al reported a case of solitary mandibular plasmacytoma, which displayed a lytic lesion with a sunray periosteal reaction on the conventional radiograph (et al reported a solitary osteosclerotic mandibular plasmacytoma with prominent sunray spiculation, which radiologically mimicked an osteosarcoma (In the present case, the tumor appeared as an extensive, mildly expansile lytic lesion, with destruction of the cortex and adjacent soft tissue mass. Around the periphery of the lesion, there were straight bony spicules extending outward from the cortex that resembled sunrays in appearance. To the best of our knowledge, there have only been a few previous case studies with bony spicules forming the appearance of sunrays –12. The diograph . Lipper osarcoma . This unosarcoma . Consideosarcoma . In the osarcoma . A mixedosarcoma ; this coosarcoma , howeverIn conclusion, malignant tumors should be considered if an osteolytic lesion with destruction of the cortex and soft tissue mass is detected in the sternum. Although unusual, a diagnosis of plasmacytoma must be considered in the presence of a lytic lesion with peripheral bony spicules of sunray appearance.


Dear Editor, A male, three-year-old child with morphostructural alteration developed over the last yearin the region of the mandible at left, presenting with recent onset of pain, with no otherassociated complaints. Laboratory tests did not demonstrate any alteration and magneticresonance imaging (MRI)  showed a-5. The mandible is the most affected site, particularly in itsposterior portion, corresponding to 22% of cases,2,4, followed by the metaphyseal region oflong bones. Desmoplastic fibromas may occur at any age range, although its higher incidenceis observed at the first three decades of life-3,6. Despite conflicting data, it seems thereis no predilection for sex,6. Localrecurrence is frequently observed in cases where complete resection is not. Clinically, thepatients are either asymptomatic or may present with pain, edema, joint effusion andpathological fracture-6. The differential diagnosis shouldconsider rhabdomyosarcoma, fibrosarcoma, giant cell tumor, among others. Despite theimaging methods usefulness in the lesion delimitation, the diagnosis is histopathological.Desmoplastic fibroma is an extremely rare, benign bone tumor with aggressive and usuallyinsidious behavior, representing 0.1% of all primary bone tumors,3-6, but there are reports of lesions withhypersignal on T2weighted images-3,6. The enhancement may be variable, andaccording to some authors, such variation may be a result of the cellular content of thelesion,4. In the present case, there washomogeneous iso/hyposignal on T1-weighted images and subtle hypersignal on T2-weightedimages, with foci of low signal intensity. After gadolinium injection, marked contrastenhancement, with noticeable perineural dissemination through the third division of thetrigeminal nerve were observed. Such aspects on T2weighted sequences, and the presence ofperineural dissemination are not commonly observed as compared with the typical imagingpattern described at MRI.At MRI, most lesions present with iso/hyposignal on T1weighted images and low signalintensity on T2-weighted images–3 mm2/s aresuggestive of malignancy.In the present case, the value for apparent diffusion coefficient was 1.45 ×10–3 mm2/s, corroborating the previously described findings.Reports on diffusion in desmoplastic fibromas were not found in the literature. In thepresent case, areas of diffusion restriction were not observed. Recent studies highlightthe use of diffusionweighted imaging in the evaluation of head and neck lesions, showingthat apparent diffusion coefficient < 1.22 × 10The authors conclude that the diagnosis of desmoplastic fibromas should be considered inpatients under the age of 30 presenting with tumor particularly located in the mandible,and that such a hypothesis cannot be ruled out in case of less noticeable foci ofhyposignal on T2-weighted images.


Location of ectopic tooth in a nondentate area like the maxillary antrum is rare. A 17-year-old boy, with one year history of recurrent right facial swelling and radiographic finding of a maxillary third molar tooth located at the posterior wall of the maxillary antrum, is presented. Under endotracheal intubation, the tooth was extracted through a Caldwell-Luc antrostomy approach and patient had an uneventful recovery and has been symptom free for eight months. Ectopic tooth in the maxillary antrum is rare and is commonest with maxillary third molar. It may be symptomless but is more commonly associated with inflammatory symptoms. The treatment of choice is surgical excision which is mostly carried out with Caldwell-Luc approach, even though endoscopic approach is being reported. Maxillary antrum is an uncommon location of an ectopic tooth. Only thirty cases were reported in a medline search between 1980 and 2010 .Odontogenesis (tooth development) begins in the sixth week in utero at the time of maxillary and mandibular dental lamina formation . It undeTooth eruption process is, in most cases, a passive one but an abnormal tissue interaction during odontogenesis may result in ectopic tooth development and eruption . The disEctopic tooth has been rarely reported and this report is being added to the literature to contribute to the body of knowledge on the topic.A 17-year-old student was referred to the Oral and Maxillofacial Surgery unit, on account of an accidental radiologic (Orthopantomograph-OPG) discovery of an ectopic tooth in the right maxillary antrum . There wExamination revealed a right facial swelling, mild pain, and a discharging sinus from the right upper buccal sulcus in relation to tooth number 16. There was full complement of teeth on that arch except tooth number 18. All the teeth were firm and noncarious. The patient had experienced 3 episodes of infection in the past one year. The patient was not HIV positive and there was no history of diabetic mellitus or sickle cell disease. Further systemic examination revealed no abnormality.Further radiological investigation included CT scan as well as Occipitomental and True Lateral of the Skull. Investigation revealed that a molar tooth was located at the posterior wall of the antrum surrounded by radiopacity and no sign of a cyst .Through Caldwell-Luc antrostomy approach under endotracheal intubation, the tooth was extracted, pus was evacuated, antrum was properly irrigated, and wound was closed with a resorbable suture with an antral pack in place, which was removed 3 days later . The heaEctopic eruption of a tooth within the dentate region is often seen in clinical practice, which is more common in mandible but such a condition in a nondentate area like maxillary sinus is very rare , 10. EctMaxillary teeth in maxillary sinus may be discovered accidentally, precipitate sinusitis, or sometimes result in ophthalmic symptoms . SomayajWater's view, OPG, and lateral cephalogram are simple and inexpensive projections for radiographic evaluation of an ectopic tooth in the maxillary sinus. Though expensive, CT and MRI certainly have an edge over conventional radiographs . In addiThe treatment of an ectopic tooth in the maxillary sinus is removal, as it may lead to cyst formation if left untreated . The traIn conclusion, ectopic tooth in the maxillary antrum is rare and is commonest with maxillary third molar. It may be symptomless but is more commonly associated with inflammatory symptoms. The treatment of choice is surgical excision which is mostly carried out with Caldwell-Luc approach, even though endoscopic approach is being reported.