Dear Sir, Langerhans cell histiocytosis (LCH) is a relatively rare disorder, accounting for less than 1% of all osseous lesions. LCH involving bone has been reported in a wide age distribution ranging from the first few months to the 8th decade of life.[1] Pain and swelling of the affected area occur most commonly. Other findings are related to the bone involved. Clinically and imaging studies can point out malignancy or infection.[2] Herein we report a case of Langerhans cell histiocystosis of femeur which simulated as osteomyelitis.A 9-month-old male infant was referred to our hospital with right hip pain for 10 days. Clinical examination showed a soft mobile palpable mass on greater trochanter of right femur. Radiograph showed radiolusent lytic lesion that was localized on the upper metaphysis of right femur with periosteal reaction and little expansion. Adjacent soft tissues showed increased density and thickness . Magnetic resonance imaging (MRI) revealed a heterogeneous contrasting lesion on upper right femur that was hypointense on T1 and hyperintense on T2 weighted images. Also there was a 5 mm x 2 mm area on his right distal femur which was hyperintense on T2, and hypointense on T2 weighted images. ESR was 69 mm first hour and hemoglobin 11.1 g/dl. Other laboratory tests were within normal limits. Patient was shifted to orthopedics department. Clinically the case was considered as osteomyelitis and after optimisation, operation was performed. Simple curettage of inflammed area of the bone was done. Removed tissue was sent for histopathology.Grossly, there were 3 pieces of material (0.7cm to 2 cm in size) with a yellowish-pinky colour. Microscopically there were numerous bean shaped atypical histiocytes, eosinophils and neutrophils . Immunohistochemically, these atypical histiocytes were CD1A, S100,CD3 positive . The case was diagnosed as Langerhans cell histiocytosis. The patient is doing fine at biannuall follow-ups. Clinically and radiographically there are no signs of recurrence.The case was diagnosed as Langerhans cell histiocytosis. The patient is doing fine at biannuall follow-ups. Clinically and radiographically there are no signs of recurrence.LCH of bone can be divided into three major categories on the basis of type and extent of the organ involvement: solitary bone involvement, multiple bone involvement (with or without skin involvement), multiple organ involvement .[3] The most common sites are the cranial vault, jaw, humerus, rib, and femur. In our case femur was involved. The classical cytological features include high cellularity composed of sheets and isolated LCH seen admixed with polymorphous population of numerous eosinophils, neutrophils, lymphocytes, plasma cells, multinucleated giant cells, and macrophages. They show variable degree of pleomorphism and mitotic activity. The key to the diagnosis is to identify the LCH through its characteristic features, namely, nuclear grooves and nuclear pseudoinclusions. Presence of dendrite-like cytoplasmic processes in LCH is a rare but is a characteristic feature. We observed many atypical histiocytes but could not identify any demonstrative cytoplasmic process. Degree of eosinophil infiltration varies in different areas of LCH lesion and different organs, thus their number can vary from scant to abundant in cytology smears. Their presence can help in diagnosis.[4]Histopathologically, there are dense intradermal infiltrations of histiocytes showing abundant eosinophilic cytoplasm and kidney-shaped or indented nuclei associated with lymphocytes and eosinophils. These histiocytes show strong expression of S-100 protein and CD1a, markers of LCs. Differential diagnosis includes hemangioma, juvenile xanthogranuloma, Spitz nevi, mastocytoma, osteoid osteoma, osteoblastoma , broad and includes infection, malignancies such as Ewing sarcoma, lymphoma, or metastasis.[5-7] LCH has a lytic appearence with an ill defined border in the imaging studies similar to osteomyelitis so osteomyelitis must be kept in differantial diagnosis.[8] In literature there are different options for managing LCH. First option is not to do anything, because it can spontaneously regress. Operation can be performed for either to make a diagnosis or to relieve symptoms by partial resection. Topical or intralesionel steroids can be used. Radiotherapy is another option. Chemotherapy can be used in systemic cases. Recurrences usually appear in 2 years but duration can be very long so it is important to follow these patients for a long period of time with imaging studies.[9] In our case simple curettage was performed. After the operation patient's mobility improved. His serial imaging studies showed no sign of recurrence, and to date he is healthy. Source of Support: NilConflict of Interest: None declared


In this article, we present our experience with a case of myxolipoma of the tongue.Lipoma is a mesenchymal benign tumor occurring with relatively high frequency. However, myxolipoma, one of the histological variant of lipoma characterized by mature adipose tissue and abundant mucoid substances, in the oral cavity is quite rare.The patient was a 52-year-old man who noticed a painless mass on the left border of tongue about 2 years ago. The lesion was noted at a complete medical checkup, and the patient was admitted to our institution for detailed examination. The mass was a palpable, soft and elastic nodule, 15 mm in diameter, covered with normal mucosa in the left inferior aspect of the tongue. The border of the tumor was well-defined, and computed tomography (CT) revealed a fat density within the mass. On the basis of these finding, the tumor was clinically diagnosed as lipoma and was excised under general anesthesia. Histopathologically, the tumor was a well-defined lobulated mass surrounded by a thin fibrous capsule within the muscle of the tongue. The tumor was diagnosed as myxolipoma because it was consisted of solid proliferation of mature adipocytes replaced by abundant mucoid substances. The post operative course was uneventful, and there was no evidence of recurrence 4 years after surgery. Lipoma is the most common neoplasms arising from fat tissue. They are usually having the character of slow-growing, soft and silent masses. Angiolipoma, spindle cell lipoma, myelolipoma, chondorolipoma and myolipoma are some histologic variants of lipomas. Myxolipoma is a lipoma admixed with abundant mucoid substances and is considered to be a lipoma with a high degree of myxoid change ,2.However mixolipoma is infrequently observed as neoplasm in the trunk, it rarely occurs in oral region. Only fourteen cases of involving the oral regions, including tougue, buccal mucosa, and lowerlip have been reported in the English literature -4. The iA 52-year-old man was referred for a painless mass of the left lateral tongue. He had been aware of the mass for about 2 years previously, but had not sought medical attention. Three months before his first visit, he was found to have this mass at a general checkup. There is nothing special in his previous medical or familial history.Intraoral examination revealed a solitary, 15 × 15 mm-sized mass on the left border of the tongue  and was excised under general anesthesia. The mass was well demarcated and was easily separated from the surrounding tissue.The excised mass was elastic-soft and yellowish-white and showed lobulation on its cut surface Figure , 5. HistPostoperatively, there has been no lingual dysfunction or any particular problems. The post operative course was uneventful, and there was no evidence of recurrence 4 years after surgery.Although lipoma represents the most common mesenchymal tumor, its occurrence in the oral and maxillofacial regions is not frequent. As lipomas are occasionally altered by an admixture of other mesenchymal elements, the microscopic variants have been described, including fibrolipoma, sclerotic lipoma, chondrolipoma, osteolipoma, myolipoma and angiomyxolipoma. Myxolipoma is a lipoma admixed with abundant mucoid substances and is considered to be a lipoma with a high degree of myxoid change,2. The mMyxolipoma has to be distinguished from benign and malingnant lipomatous tumors with abundant mucoid substances, such as chondroidlipoma, spindle cell lipoma with myxoid changes and myxoid liposarcoma. Chondroidlipoma shows nest-like or cord-like growth of lipoblasts, and its mucoid matrix is resistant to hyaluronidase. SpindleAs for treatment, surgical excision becomes first choice -4. ThereThe authors declare that they have no competing interests.SO, MR, MT, IO, GO, YM, NCG and NK conceived of the study and participated in its design and coordination. SO and MR drafted the manuscript and contributed equally to this work. MR, NCG and NK were involved in revising the manuscript. All authors read and approved the final manuscript.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.The article processing charges are funded by the Deutsche Forschungsgemeinschaft (DFG), "Open Access Publizieren".


A malignant fibrous histiocytoma occurring on the scalp near a primary operation site is extremely rare.A 74-year-old Chinese man presented with a one-month history of recurrent headaches, vomiting and left limb atony. He had undergone a successful clipping operation through the pterional approach for an anterior communicating aneurysm five years previously. One month before presentation, he developed a headache without apparent cause that was especially severe in the right frontal region. He also had a small tumor on the right side of his forehead at the original incision site. The tumor had gradually increased from soybean size to egg size in one month; this growth was accompanied by nausea, projectile vomiting of gastric contents and left limb atony. The subcutaneous tumor was totally resected along with some affected cranial tissues. Our patient’s postoperative recovery was good, and he was safely discharged 20 days after the surgery. He was free of recurrence over two years of follow-up.in situ, early diagnosis and radical surgical resection are key to a successful outcome.Dissection of the temporal muscles and deep fascia using electric resection and electrocoagulation through the pterional approach may cause tissue degeneration, which may in turn lead to cancer development. In our patient’s case, the reason for the development of the tumor five years after his surgical aneurysm repair was unclear; it may have represented a primary malignant fibrous histiocytoma of the scalp that had no relationship to the operation. We followed up our patient for two years and he had no tumor recurrence. Because malignant fibrous histiocytoma of the scalp has a high degree of malignancy and readily recurs  First reported by O’Brien and Stout in 1964  stated tA 74-year-old Chinese man presented with a one-month history of recurrent headaches, vomiting and left limb atony. He had undergone a successful clipping operation through the pterional approach for an anterior communicating artery aneurysm five years previously. One month before presentation, he developed a headache without apparent cause that was especially severe in the right frontal region. He also had a small tumor on the right side of his forehead at the original incision site; the tumor had gradually increased from soybean size to egg size in one month. This was accompanied by nausea, projectile vomiting of gastric contents and left limb atony. Computed tomography demonstrated a subcutaneous tumor on the right side of his forehead, a scalp lesion complicated with right frontal lobe hemorrhage, and a right ventricular hematocele. We gave our patient oxiracetam ) for treatment of neurotrophy. On the third day after admission, he suddenly lost consciousness. Urgent head computed tomography showed an intracranial hemorrhage, the volume of which had increased from 40mL to 90mL as well as persistent bleeding and a midline shift of over 1.5cm (Figure Our patient then underwent surgery to resect the subcutaneous tumor and evacuate the intracranial hematoma under general anesthesia. An incision was made along the one from the original surgery. During the operation, it became apparent that the main part of the tumor was located in the subcutaneous tissue of his right frontal region. The tumor was approximately 7cm×6cm×4cm Figure , fully eA pathological microscopic examination of the resected tumor Figure  showed ain situ, early diagnosis and radical surgical resection are key to a successful outcome [MFH is one of the most common soft tissue sarcomas in adults ,4. The m outcome ,12.in situ, thus early diagnosis and radical surgical resection are key to a successful outcome.The cancer in our patient may have been induced by dissection of his temporal muscle tissue and deep fascia using electric resection and electrocoagulation through the pterional approach. MFH of the scalp has a high degree of malignancy and recurrence 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.The authors declare that they have no competing interests.WJ analyzed and interpreted the patient data and searched the existing literature on malignant fibrous histiocytoma. XYH and FL obtained the patient’s photographs and designed the figures. DB wrote the case report and revised the manuscript. LY and ZWM assisted with the writing and editing of the manuscript. All authors read and approved the final manuscript.


This paper reports a case of testicular synovial sarcoma with molecular genetic analysis. A 24-year-old male presented with painless scrotal mass. Ultrasonography showed a heterogeneous mass of 66 mm × 34 mm in size involving the inguinal region. Histological examination of a surgical biopsy showed a grade III monophasic growth pattern of spindle cell proliferation. Immunohistochemical analyses indicated positive staining for pancytokeratine and epithelial membrane antigen. Cytogenetic analysis showed the presence of CYT-SSX1 mutation, and CT scan showed non-specific pleural micro-nodules with a size of 7.5 mm. The patient had an extended left orchidectomy but was lost to follow-up for 1 year. A local recurrent scrotal mass of 32 mm × 25 mm, multiple inguinal lymph nodes, and increased pleural nodules, which were confirmed by histological examination, were treated with three cycles of adriamycine and ifosfamide chemotherapy, surgical resection, and radiotherapy with complete response. After 3 months, the patient developed local recurrence and pulmonary metastases that did not respond to second-line chemotherapy based on gemcitabine and paclitaxel. The patient had dyspnea at the time of this writing and chest pain, and is under third-line chemotherapy based on Deticene after 30 months of following up. This patient died on November 16, 2012 after a resperatory failure and malignant pelural effusion. Synovial sarcoma should be considered in the differential diagnosis of soft tissue tumor and it should be aggressively treated to improve prognosis. Although our patient has shown numerous factors of bad prognosis, he has had a relatively long survival time. Synovial sarcoma represents 5% to 10% of all histological types of STS. It is a mesenchymal neoplasm that appears in children and young adults and most frequently affects the extremities. Generally, it is regarded as a high-grade tumor and patients develop metastases particularly in the lungs. Optimal therapy is unknown, and the prognosis remains guarded. Unusual locations have been reported, including the mediastinum, lung, and peritoneum. We report a new case of an unusual location: the testis.Soft tissue sarcoma (STS) accounts for 1% of all adult malignanciesIn April 2010, a 24-year-old man presented with painless left testicular swelling without infection or trauma history. Clinical examination showed a firm mobile mass with a size of 62 mm × 40 mm and no palpable inguinal lymph nodes were found. He had no relevant past medical history. Scrotal ultrasonography revealed a large heterogeneous testicular mass of 66 mm × 34 mm in size in the inguinal region. Abdominal ultrasonography showed no other lesions. B human chronic gonadotrophin and α-fetoprotein levels were normal. The patient underwent surgical biopsy. Histological examination indicated a monophasic growth pattern of spindle cells (Computed tomography (CT) scan showed non-specific pleural micro nodules of 7.5 mm in length. Surgery with extended left orchidectomy was performed, but the patient was lost to follow-up for one year.In May 2011, he presented with local recurrent scrotal mass of 32 mm × 25 mm in size and multiple inguinal lymph nodes. Chest X-ray and CT scan showed an increase in pleural nodule size and number , monophasic epithelial type, monophasic fibrous type, and undifferentiated type. Poorly differentiated synovial sarcoma shows a high-grade appearance with high cellularity, high mitotic activity, and high atypia.The differential diagnosis includes fibrous tumors of the pleura (CD34), and malignant peripheral nerve sheath tumor (PS100). Massive tumor calcification and numerous intratumoral mast cells are associated with poorer prognosis. Tumor size (>5 cm), mitotic activity, age, and microscopic margins are the most relevant independent prognostic factors.Karyotyping with fluorescent in situ hybridization and genetic molecular analysis constitute the only methods to confirm the diagnosis, especially in poorly differentiated forms. Translocation  resulting in CYT-SSX gene fusion is the cytogenetic characteristic. The two mutations, CYT-SSX1 and CYT-SSX2, are correlated with the biphasic and monophasic forms, respectively. CYT-SSX1 seems to be associated with a more aggressive form of synovial sarcomaSynovial sarcoma is generally treated and considered a high-grade sarcoma with frequent metastases. At an age older than 20 years at diagnosis, the size of the tumor is more than 5 cm, and the undifferentiated subtype are the main prognostic factors. Histological features such as intratumoral mast cells, tumor necrosis, high mitotic rate, and vascular invasion suggests aggressive tumor behavior.. Therapy mainly includes surgical resection with adequate safety margins. Radiotherapy showed a risk reduction of local recurrence from 7% to 50%. It is also recommended in case of positive resection margins. Chemotherapy is administered in metastatic cases, or if surgery was incomplete, it can be given in recurrence cases. A combination of ifosfamid and doxorubicin was used in the first line therapy. A combination of gemcitabine and docetaxel is another therapeutic option. Prognosis used to be poor with a survival period of only two months, but with aggressive chemotherapy, a survival rate of 14 years has been reported.Five-year and 10-year overall survival rates are between 24%-68% and 11%-56%, respectivelyAlthough our patient presented many factors of poor prognosis, such as age, tumor size, and high mitotic activity, he has had a relatively long survival. The patient was probably already metastatic at diagnosis, but the disease seemed to have a low progressive course. Surgery, radiotherapy, and chemotherapy in case of recurrence or metastases are probably optimal treatments. This case leads us to think whether synovial sarcoma in the testis has a better prognosis.. It is coded by a gene in the Xq28 region and is detected by RT-PCR of mRNA or by immunohistochemical staining, which can have a diagnostic value in ambiguous cases. Recent clinical trials using genetically modified T-cells that express a T-cell receptor targeting NY-ESO-1 showed the first successful case of immunotherapy in synovial sarcoma with an objective response of 45% to 67% and low toxicity because no normal tissue expresses this antigen. Furthermore, testis tissue is not targeted because it does not express a class I major histocompatibility complex. Patients should be encouraged to take part in these clinical trials.New targeted immunotherapies are emerging. NY-ESO-1 is a cancer testis antigen expressed in normal testis tissue and in some tumors, such as breast cancer, myxoid/round cell liposarcoma, melanoma (25%), synovial sarcoma (80%)In summary, although synovial sarcoma should be considered in the differential diagnosis of soft tissue tumor in unusual locations, it should be aggressively treated with complete resection or multimodal therapy to improve the prognosis of patients.


Colorectal cancers have potential for lymphatic and hematogenous metastases. Surgery is the definitive treatment, but the prognosis can be improved with the addition of chemotherapy, radiotherapy or both. However, the incidence of recurrence, both local and distant, remains significant. Distant metastases occur most often in the liver and lung; however, metastases to bone, adrenals, lymph nodes, brain, skin and the oral region have been reported. Metastases to the oral region are uncommon and may occur in the oral soft tissues or jaw bones. The prognosis in such patients is usually very poor. We report a case of colorectal carcinoma with metastasis to the floor of the mouth. This is probably the first reported case of metastasis to the floor of the mouth in a patient with colorectal cancer. Metastatic spread from colorectal carcinoma is quite predictable, and is initially by the lymphatic route, followed by the hematogenous route. The commonest sites for distant metastases are liver and lung. Metastatic tumors in the oral region are uncommon and account for approximately 1% of all malignant oral tumors.Figure 1). There was nodal involvement (two lymph nodes), and it was staged T3N1M0. Postoperatively, she received a course of radiotherapy (RT) consisting of 50 Gy in 25 fractions to the whole pelvis. Following RT, she received 6 cycles of 5-fluorouracil/leucovorin. She remained symptom free until January 2007. She was readmitted with persistent lower abdominal pain of 1-month duration. Clinical examination was noncontributory. CT scan of the abdomen revealed 3.8×3.0×3.2-cm hypodense lesion in the mid-presacral region. CT-guided fine-needle aspiration cytology of the lesion was suggestive of recurrence of adenocarcinoma. She was treated with 6 cycles of FOLFOX4 , and a CT scan of the abdomen subsequently revealed complete disappearance of the lesion. Within 2 months, she was readmitted with a growth in the floor of the mouth . A punch biopsy was performed. Histopathological examination revealed a well-differentiated adenocarcinoma , and the original primary and floor-of-mouth metastases were morphologically similar. She was treated with chemotherapy regimen consisting of FOLFOX4 with bevacizumab. Two cycles of chemotherapy elicited no response, and the growth was progressively increasing in size. Chemotherapy was stopped and she was treated with radiotherapy consisting of 64 Gy/32 Fr. She did not respond to radiotherapy either and succumbed to the disease after 20 days.A 42-year-old woman was admitted in December 2003 with a 10-day history of blood and mucus in the stool. Rectal examination and endoscopic evaluation showed a circumferential lesion that began 4 cm above the anal verge and caused an obstruction. A CT scan of the abdomen demonstrated a mass of 4.5 cm in length with filling defect. A transanal incisional biopsy was performed. Histologic examination revealed an adenocarcinoma. The patient underwent anteroposterior resection with total mesorectal excision. Histological examination revealed a mucinous adenocarcinoma of the rectum extending through the whole muscular layer with invasion of the serosa (Table 1). Few cases of metastasis to the oral cavity from colorectal carcinoma have been reported in literature (Table 2). In most cases, the site of metastasis is gingival. Our case is unique in that probably this is the first case report of metastasis to the floor of mouth from colorectal carcinoma.From available evidence, it is known that patients with disseminated colorectal cancer fare poorly, which was observed in the present case. Oral metastatic tumors are uncommon and comprise approximately 1% of all malignant oral neoplasms.Whereas early detection and surgery remain the main therapeutic options for adenocarcinoma of the colon, a good response to chemotherapy in an advanced case is also reported.


The presentation of scalp metastases from leiomyosarcoma of the vena cava is an extremely infrequent event. There are no other publications that describe such finding and very few of leiomyosarcoma in vessels. About this event we have reviewed the English literature describing studies on scalp metastases and skin metastases in general: their incidence, origin, clinical appearance, meaning, and diagnosis. The case we describe would be the second one presented worldwide because, as far as we know, it has been only one more published in 2005. Leiomyosarcoma of the inferior vena cava is a rare neoplasm with poor prognosis. It accounts for only about 0.5% of adult soft tissue sarcomas, although within the group of sarcomas arising from the venous system, it is the most common location, nearly 50% of cases .Leiomyosarcoma is known for its resistance to chemotherapy, radiation therapy, and propensity for haematological dissemination. The diagnosis used to be delayed due to the absence of symptoms during months. Most of them are metastatic disease at the moment of the histological confirmation. The presentation of scalp metastases from leiomyosarcoma of the vena cava is an extremely infrequent event. A 29-year-old woman was referred with a subcutaneous nodule in the scalp of three-month duration as first symptom. The nodule was 1 cm sized, not ulcerated, and without swelling signs. It did not show hair loss and had grown asymptomatically.The first biopsy revealed an epithelioid sarcoma . Tumoral cells expressed vimentin immunoreactivity and epithelial membrane antigen (EMA). They did not express any specific mesenchymal marker. High Ki67 expression level was observed (>25%). As long as margins were involved, the second biopsy—complete resection—revealed a sarcoma with muscle differentiation, a leiomyosarcoma grade II. The immunohistochemical results expressed vimentin, desmin, caldesmon, and EMA . Other mComputed tomography (CT) revealed a mass (diameter 3.6 cm) near from the right adrenal gland that included inferior vena cava with heterogeneous capture of contrast and fat density. Magnetic resonance image demonstrated that the heterogeneous mass was in this vessel and had low-density centrum and probable necrosis. The mass displaced the right adrenal gland. It was showed one nodular lesion in liver in segment VIII that seemed to be a metastasis as first possibility. Tomography with emission of positrons (PET) confirmed the mass near from the right adrenal gland with malignance signs  and the focal deposit in liver. Percutaneous needle biopsy of the mass confirmed the diagnosis.2 day 8) and gemcitabine (900 mg/m2 days 1 and 8) each 21 days. After 3 cycles, it was found a partial response of the retroperitoneal mass without changes in the liver. Three months after diagnosis, surgery was planned. The mass and the vena cava involved were resected en bloc and an endoluminal prosthesis was emplaced. The tumor sized 4.5 cm and invaded the 50% of the vessel lumen. It compressed the adrenal gland without infiltration. The hepatic deposit was also resected (1.6 cm). After this procedure, she received three cycles more with the same scheme. She received neoadjuvant chemotherapy with Taxotere (100 mg/m2 days 1–4) and adriamycin (30 mg/m2 days 1–3), 6 cycles. After 6 months enlargement of the pulmonary nodes were observed and a new hepatic metastasis was found. She received a new scheme with trabectedin (1.3 mg/m2), and after 12 cycles, it was stopped because of hepatic progression. She died 59 months from diagnosis.After 6 months, TC showed liver and lungs relapse. She was given a second-line chemotherapy with ifosfamide , abdomen (20.2%), and scalp (12.6%) . Other s0.3%, abdPositron-emission tomography (PET) examinations with 8–53% detection rates play an important role for diagnosis, but they reveal a high number of incorrect positive findings (sensitivity of 91.9% and specificity of 81.9%) . CurrentThe clinical presentations of the skin metastases vary over a wide morphologic spectrum, although most frequently are multiple nodules –4. They In most cases, the metastatic deposit shows histological features that resemble the underlying primary malignance. However, metastases may be more anaplastic and exhibit less differentiation. Immune markers and ultrastructural studies are needed , 4, 5, 7There is a paucity of literature in regards to leiomyosarcoma of the vena cava with metastases to the scalp. Vandergriff et al.  have repVandergriff concludes that the scalp is the most common cutaneous site of involvement of metastatic leiomyosarcoma occurring in half of all patients with skin metastasis. It would be necessary another 50 years more in order to report at least 15 new additional cases.


A 75-year-old long-term male smoker and poorly controlled hypertensive presented with a 3-month history of intermittent epistaxis refractory to cauterisation with silver nitrate. Nasopharyngeal examination revealed a mass in the post nasal space. An urgent endoscopic biopsy confirmed an extraosseous plasmacytoma. Post operative radiotherapy was scheduled. No evidence of recurrence of disease following completion of treatment has been detected during clinical surveillance.Epistaxis; Nasopharynx; Biopsy; Plasmacytoma; Radiotherapy A 75-year-old long-term male smoker and poorly controlled hypertensive presented with a 3-month history of intermittent epistaxis refractory to cauterisation with silver nitrate. Nasopharyngeal examination revealed a smooth mass extending inferiorly from the right fossa of Rossenmuller and effacing the posterior pharyngeal wall . An urgeSerum and urine assays for Bence Jones protein were negative and there was no evidence of gammopathy. A bone marrow biopsy and Computed Tomography and Magnetic Resonance imaging did not demonstrate any metastasis or skeletal involvement. The Excisional biopsy had been complete and the patient underwent radical radiotherapy with a 45Gy dose in 20 fractions of the nasopharyngeal field.No evidence of recurrence of disease following completion of treatment has been detected during clinical surveillance.A plasmacytoma is a very rare discrete solitary mass of neoplastic monoclonal plasma cells, first described by Schridde in 1905 . They reTissue biopsy, serum electrophoresis (to exclude myeloma) and radiological skeletal survey with bone marrow biopsy to determine skeletal involvement is necessary for diagnosis. Treatment includes a combination of surgical excision and radiotherapy. Follow-up radiological and electrophoresis assessment is required after treatment to detect recurrences and progression to myeloma (10-30% frequency). The overall 10 year survival is 70% .


Splenic cysts are rare entities and are classified as true cysts or pseudocysts based on the presence of an epithelial lining. Congenital nonparasitic true cysts can be epidermoid, dermoid, or endodermoid, present at a young age, and are commonly located in the upper pole of the spleen. Surgical treatment is recommended for symptomatic, large (more than 5 cm), or complicated cysts. Depending on cyst number, location, relation to hilus, and the major splenic vessels, the surgical options include aspiration, marsupialization, cystectomy, partial cystectomy (decapsulation), and partial or complete splenectomy. Laparoscopic techniques have now become the standard approach for many conditions, including the splenic cysts, with emphasis on the spleen-preserving minimally invasive operations. We present the successful extended partial laparoscopic decapsulation of a giant epidermoid splenic cyst in a young female patient that, although asymptomatic, was unfortunately followed by complete splenectomy five days later due to a misinterpreted abdominal CT suggesting splenic postoperative ischemia. Echinococcus and nonparasitic [Splenic cysts are rare entities and are classified as true cysts (primary) or pseudocysts (secondary) based on the presence of an epithelial lining. True cysts can be further subdivided into parasitic caused by arasitic , 2.Nonparasitic, true cysts are congenital or neoplastic. Congenital cysts can be epidermoid (90%), dermoid, or endodermoid, present at a young age (children and young adults) and are commonly located in the upper pole of the spleen. CA 19-9 and CEA levels are elevated in the epidermoid cyst's contents and in the patient's serum. Pseudocysts are believed to develop after posttraumatic intraparenchymal or subcapsular splenic hematomas and occasionally after splenic infarcts or infections. Secondary cysts account for 75% of all nonparasitic splenic cysts .The frequent use of abdominal imaging and the increasingly successful nonoperative management of splenic injuries contribute to a rise in the incidence of nonparasitic splenic cysts, which otherwise are considered to be rare.Most of the patients with a splenic cyst have symptoms, and most asymptomatic cysts can be detected during physical examination of the abdomen . Cysts lLaparoscopic techniques have now become the standard approach for many conditions, including the splenic cysts, with emphasis on the spleen-preserving minimally invasive operations, since complete splenectomy carries the risk of overwhelming postoperative infection and thrombocytosis . Apart fWe report the successful extended partial laparoscopic decapsulation of a giant epidermoid splenic cyst in a young female patient that, although asymptomatic, was unfortunately followed by complete splenectomy five days later due to a misinterpreted abdominal CT suggesting splenic postoperative ischemia.A 19-year-old female patient was admitted at the emergency department in our hospital, complaining about abdominal pain in left upper quadrant. The patient's medical history was unremarkable, including previous abdominal trauma. During physical examination, a palpable mass was found in the left hypochondrium. The routine biochemical and hematological investigations were normal. Chest and abdominal X-ray showed the elevation of the left hemidiaphragm  and the The abdominal computed tomography scan (CT scan) demonstrated a well-circumscribed, large nonenhancing unilocular , hypoattenuated cystic (approximately 8 Hounsfield Units) lesion in the upper pole of the spleen lacking internal septa and therefore not favouring a parasitic origin . The lesSerum levels of CA 19-9 were elevated , while serum levels of CEA were normal and Echinococcus serum antibodies were negative.The negative history for antecedent abdominal trauma together with the imaging and laboratory findings were suggestive of a giant symptomatic primary nonparasitic congenital splenic cyst for which surgical treatment was indicated.Surgical treatment was carried out by laparoscopic approach. Under general endotracheal anesthesia, the patient was placed in a modified lithotomy 20° antitrendelenburg position with right lateral position at 10°. A 10-mm trocar was inserted in a standard umbilical position using the Hasson's open technique, pneumoperitoneum was created by carbon dioxide insufflation, and a 30° camera was inserted subsequently. Due to the large size of the cyst that caused displacement of adjacent organs and structures, additional trocars were placed as shown in The omentum attached over the spleen was dissected and pulled down revealing an anterior cyst at the upper pole of the spleen . The splHistologic examination of the resected specimen showed that it was a 9 cm in diameter part of a splenic cyst. The inner surface had septations and multiple trabeculations, which were gray yellow and firm. The cyst wall contained hyalinizated dense fibrous tissue, and the cortex was covered with keratinized squamous epithelium. The lining varied from flattened single-layer cuboidal to fully stratified keratinized squamous epithelium. There was no evidence of epithelial atypia or malignancy, the stratified squamous epithelium was positive in the immunohistological stains for CA19-9, ceratines 8, 18, and 19. The histopathological findings confirmed the diagnosis of a congenital splenic epidermoid cyst. The patient's recovery was prompt and clinically uneventful. A follow-up abdominal CT scan at the 3rd postoperative day showed perisplenic fluid accumulation and an air-filled cavity contiguous with the splenic parenchyma, while the parenchyma of the splenic remnant was falsely misinterpreted as having perfusion defects, especially in the margins of the cystic wall left in situ Figures . SubsequThe histologic examination of the resected specimen  revealed a macroscopically intact spleen together with its portal vessels and a cystic wall remnant. The splenic parenchyma presented mild congestion and edema without evidence of ischemia or infarction. The cystic wall attached to the splenic remnant had the same histologic features with the previously resected cyst. 3 at 10 days, but returned to within the normal range by 3 weeks after surgery. Her serum levels of CA19-9 were normal 4 months after the operation. There have been no abdominal symptoms during 12 months of follow-up.The postoperative course was uneventful, and the patient was vaccinated against postsplenectomy infections and discharged 7 days later with a normal abdominal US and Doppler exanimation. The platelet count increased to 850000/mmNonparasitic cysts larger than 5 cm are susceptible to hemorrhage, rupture, and infection. Therefore, surgical treatment is recommended for symptomatic, large (more than 5 cm), or complicated cysts . The conLaparoscopic techniques have now become the standard approach for many conditions, including the splenic cysts, with emphasis on the spleen-preserving minimally invasive operations . LaparosA high proportion of cyst recurrence has been reported after simple aspiration or marsupialization of the cyst, and, therefore, these techniques are not recommended , 2. The When partial splenic resection is not feasible due to technical difficulties, some authors have proposed an alternative technique called “cystic decapsulation”. Decapsulation involves a near total resection of the cyst leaving the portion of cyst wall contiguous with the splenic parenchyma in situ. Decapsulation is a simpler and more rapidly performed procedure, with less blood loss than partial splenectomy –13. The Finally, although perioperative complications of the laparoscopic treatment for splenic diseases have been reported , open prIn our case, the female patient presented with a large symptomatic splenic cyst. Laboratory and imaging studies were suggestive of a primary nonparasitic congenital splenic cyst, and surgical treatment was decided as recommended , 2. We dIatrogenic splenic injury is a rare complication and may be caused during abdominal surgery, cardiac surgery, and colonoscopy , 19. SplSplenic infarction may develop insidiously during surgery and may be diffuse or focal. Peripheral splenic arterial branches are end arteries with poor collateral circulation. Perfusion defects in these vessels after occlusion, injury, or severe hemodynamic derangement during operation leads to infarction. Causes of splenic artery occlusion include embolic disease , atherosclerosis, arteritis, splenic artery aneurysm, sickle cell disease (thrombosis), splenic torsion, and mass lesions such as pancreatic carcinoma , 22. Splenic infarcts may be silent or the patients may present left upper quadrant pain of acute onset with or without fever. The CT appearance of splenic infarction is time depended after the triggering event . The infIn the present case, an iatrogenic intraoperative injury during recent laparoscopic decapsulation or postoperative torsion of the vessels at the splenic hilum due to extended mobilization was considered as the possible causes of splenic ischemia due to the CT findings in an otherwise asymptomatic patient. Total splenectomy was consequently performed, while the histologic examination of the resected spleen showed an intact splenic parenchyma with mild congestion and edema without evidence of ischemia or infarction. A thorough search of the literature in PubMed did not reveal any report of a similar complication after laparoscopic cyst decapsulation of the spleen. In a single case, as reported by Chin et al., splenic ischemia was recognized during laparoscopic partial splenectomy and was treated with conversion to total splenectomy . In this case we showed the CT imaging appearance of a normal spleen in the 3rd postoperative days after successful and uneventful laparoscopic partial cyst decapsulation. We did not find a similar image in the literature, except in one case of a spontaneously ruptured congenital splenic cyst, which had a CT appearance resembling to our case . RetrospThe laparoscopic partial cyst decapsulation is a safe and feasible surgical approach for the treatment of large congenital splenic cysts offering the advantages of a simple, not time-consuming, spleen-preserving technique with minimal blood loss, no major complications and short postoperative recovery. Contrast-enhanced CT is the diagnostic imaging modality of choice for the detection of possible splenic injury in patients with complicated postoperative course. The critical consideration of clinical signs together with imaging finding must be taken to account for the optimal treatment of a splenic injury iatrogenic or not, avoiding thus unnecessary splenectomies.


Glioblastoma (GBM) rarely presents as an infratentorial tumor in adults. The authors present a case of concomitant supratentorial and infratentorial GBM in an adult. A 72-year-old man presented with headache, nausea, vomiting, and lightheadedness. Initial MR images revealed enhancing masses in the right cerebellum and right posterior periventricular region. The patient underwent a suboccipital craniotomy and resection of the cerebellar lesion. Final histopathology was consistent with glioblastoma. The patient went on to receive standard radiation treatment for GBM with concurrent and adjuvant temozolomide. However, the patient experienced clinical deterioration within a few days after starting radiotherapy. He and his family decided to forego treatment and pursue palliative care. The patient expired three months after the initial diagnosis. Autopsy findings supported the diagnosis of GBM with leptomeningeal gliomatosis and involvement of the cerebrum, cerebellum, and spinal cord. The authors review the literature and propose that the pathogenesis of multiple and multicentric GBM may involve neural stem cells within the subventricular zone or could result from tumor dissemination along established CNS routes, such as white matter tracts and CSF pathways. Glioblastoma (GBM) is the most common intraparenchymal primary brain tumor, representing approximately 30% of all brain tumors and 50% of astrocytomas , 2. GBM Multiple gliomas were first observed by Virchow in 1864 and Bradley in 1880 –9. In thHere, we report a case of a multifocal and multicentric GBM involving the supratentorial and infratentorial regions in an adult and review the literature on all previously documented cases. We also discuss the pathogenesis of this unique presentation of GBM, thereby providing greater insight into the origin and growth of GBM. This insight may lead to changes to the treatment approach of this rare presentation of GBM.A 72-year-old right-handed male presented to the Emergency Department with headache, nausea, vomiting, and lightheadedness of approximately one week duration. On neurological examination, the patient was intact without any focal deficits. Computed tomography (CT) of the head showed multifocal areas of cerebral edema with areas of abnormal increased attenuation, predominantly involving the right cerebellar hemisphere and adjacent to the right lateral ventricle posteriorly. The cerebellar edema was associated with mass effect on the fourth ventricle and obstructive hydrocephalus. These findings were most suspicious of brain metastases. Subsequent magnetic resonance (MR) imaging of the brain with and without contrast revealed enhancing masses in the right cerebellum measuring 2.9 cm × 2.4 cm and in the right posterior periventricular region measuring 1.0 cm × 1.4 cm as well as subtle areas of abnormal enhancement adjacent to the temporal horn of the right lateral ventricle . The patThe patient underwent a suboccipital craniotomy and resection of the cerebellar lesion. Final histopathology was consistent with glioblastoma . Specifi2). The patient experienced clinical deterioration with headaches and mental status changes within a few days of starting radiotherapy. He and his family decided to forego treatment and pursue palliative care. The patient expired at home two months later.Postoperative MR imaging showed leptomeningeal enhancement within the Sylvian fissures and diffuse FLAIR signal abnormality within bilateral cerebral hemispheres . The patp53 and were all focally positive.Autopsy findings supported the diagnosis of GBM with leptomeningeal gliomatosis and involvement of the cerebrum, cerebellum, spinal cord, neurohypophysis, and choroid plexus. In particular, two grossly separate lesions were present within the right hemisphere of the cerebrum, adjacent to the occipital horn of the right lateral ventricle, which were microscopically connected by infiltrating tumor cells and were consistent with GBM . Tumor cIn this paper, we report a case of multifocal and multicentric supratentorial and infratentorial GBM in an adult. Our review of the pertinent literature revealed that only 11 other cases of concomitant supratentorial and infratentorial GBM have been documented. The clinical findings in all published adult cases of supratentorial and infratentorial GBM are summarized in P = 0.014) but not between patients with multicentric GBM and solitary GBM  [Previously, multifocal and multicentric GBM have been associated with a worse prognosis than solitary GBM, with median patient survival estimates of 6–8 months after different treatment modalities –15. A re, resp.) . PatientThe radiographic appearance of multifocal and multicentric GBM is indistinct from that of metastases, with MR imaging displaying multiple contrast-enhancing masses , 12, 17.The diagnostic workup for multifocal and multicentric GBM is generally the same as that for solitary GBM. Dissemination of GBM, however, can occur intracranially or throughout the spinal axis . In suchMultifocal and multicentric GBM do not exhibit any histopathologic characteristics that differentiate them from typical, solitary supratentorial GBM , 20. Patp53 mutations and multifocal GBM that correlates the pattern of p53 mutation to tumor migration and augmented growth [P = 0.036) [Although there is still no unified theory regarding the pathogenesis of multifocal and multicentric GBM, several hypotheses have been developed. According to earlier theories, multicentricity arises from two events , 10. Thed growth , 24. In = 0.036) . This moIn addition to the molecular pathways involved in multifocality and multicentricity, studies have correlated the tumor pattern at diagnosis and recurrence with the spatial relationship to the subventricular zone (SVZ) and cortex as seen on MR imaging . More spCurrently, there are no clear guidelines regarding the optimal management of multifocal and multicentric GBM . While tp53 was positive in both the cerebellar and cerebral lesions. Expression of c-Met was not determined.In our case, we describe an adult with concomitant supratentorial and infratentorial GBM. The patient expired three months after the initial diagnosis. Autopsy findings supported the diagnosis of GBM with leptomeningeal gliomatosis. Only 11 such cases have been previously published in the literature. Tumor dissemination could have occurred through seeding of the CSF by the periventricular mass. Alternatively, the supratentorial tumor location at the SVZ could have served as a nidus for dissemination along CNS pathways. Molecular characterization of the tumor with immunohistochemical staining for Currently, there is no specific treatment protocol for multiple or multicentric GBM. Unfortunately, these patients are often excluded from clinical trials. Our patient had a rapid clinical decline despite standard treatment and elected to forego additional treatment. Whether he may have benefitted from an alternative treatment approach with targeted molecular agents is uncertain.Our compilation and assessment of the case reports on supratentorial and infratentorial GBM in adults documented to date yield several conclusions. This presentation of GBM is rare. MR imaging characteristics are similar to metastatic tumors and therefore histopathological confirmation is necessary. Concomitant supratentorial and infratentorial GBM has the same histological appearance as that of solitary GBM. Finally, molecular and genetic analysis of these unique tumors may provide insight into their pathogenesis as well as the origin and growth of GBM in general.


A 57-year-old man who presented with urinary retention was found to have a sarcomatoid carcinoma of the urethra. Evaluation with CT scan of the abdomen and pelvis revealed multiple pulmonary nodules and osteolytic lesions of left posterior ribs. After external beam radiation therapy and six cycles of systemic chemotherapy, patient underwent a surgical resection of the urethral cancer. After his surgery, patient was also found to have multiple brain metastases and underwent whole brain radiation therapy, nine months after his initial diagnosis. Sarcomatoid carcinomas of the genitourinary tract are extremely rare tumors that require a very aggressive, multimodal treatment approach. Sarcomatoid carcinoma  is an aggressive tumor that is very rarely seen in urinary tract. We present a case of urethral cancer that initially presented as a urethral stricture failing endoscopic management. To our knowledge, this is the first case of sarcomatoid carcinoma of male urethra without a history of prior urinary tract cancer. We also discuss the pathological study and management of urethral cancer with a literature review. A 57-year-old man was referred with a two-year history of dysuria, weak stream, hematuria, and urinary retention. He had previously underwent two direct visual internal urethrotomy (DVIU) procedures with self-catheterization using a 14Fr catheter as well as dilation in the office. Patient was also complaining of chronic left shoulder and low back pain. On physical examination, he was noted to have urethral induration and a mass in the deep penoscrotal region and bilateral inguinal lymphadenopathy. His flow rate was 8 mL/sec with a 465 mL postvoid residual.CT of the abdomen and pelvis showed 5 cm heterogenous enhancing hypodense mass arising from the right side of penis that was worrisome for primary neoplasm as well as a T12 vertebral fracture. CT of the chest showed multiple pulmonary nodules and multiple osteolytic lesions in left posterior 6th and 7th ribs. He was taken to the OR for cystoscopy, urethral biopsy of this mass, and placement of suprapubic tube. Biopsies of the urethral lesions were consistent with sarcomatoid carcinoma with no squamous features. Immunohistochemical stains were positive for pancytokeratin, CAM 5.2, and AE1/AE3, focally positive for smooth muscle actin, p63, and p53, and negative for ALK-1, CK 5/6, and CD31.He was treated with a course of external beam radiation therapy to his left shoulder and T12 vertebra that helped his chronic pain. He underwent two cycles of cisplatin and gemcitabine based systemic chemotherapy; however, his disease continued to progress. He was switched to MVAC chemotherapy . After 4 cycles of MVAC chemotherapy, he underwent resection of urethral cancer mass with partial penectomy, partial scrotectomy, and total urethrectomy involving the membranous junction. There was noted to be invasion of the corpus spongiosum, corpus cavernosum, base of the penis, and perineal soft tissue. On postoperative day 2, he developed new-onset seizure-like activity involving his right arm. CT of the head demonstrated multiple hemorrhagic brain metastases. He underwent a course of whole brain radiation therapy. The urethral mass measured 8.5 × 7.2 × 6.4 cm with high grade sarcomatoid carcinoma histology. The tumor invaded into the periurethral corpus spongiosum, the corpus cavernosum, base of penis, and the perineal soft tissue with negative margins. Final pathological staging was Stage IV. TNM staging was pT3 Nx cM1. Sarcomatoid carcinoma is an aggressive tumor that can occur in any organ. These tumors are composed of both malignant mesenchymal and epithelial elements. It is very rarely seen in the urinary tract, and only a very limited number of cases in the renal pelvis and ureter –5, bladdThis patient initially presented with recurrent urethral stricture that failed multiple endoscopic treatments. Since urethral neoplasms are rarely seen and urethral strictures occur quite frequently, diagnosis of urethral cancer is usually delayed . The posThese tumors are usually very aggressive possibly because they are diagnosed at an advanced stage . A complAlthough sarcomatoid carcinoma in the urethra is a rare event, the prognosis in these cases appears to be very poor. Since this rare but aggressive cancer to male urethra mimics urethral stricture, every effort should be made to establish an early diagnosis and aggressively treat them using a multimodal approach.


We present the case of a 38-year-old woman that presented with a very slowly enlarging mass of the tongue. MRI revealed a large mass originating in the tongue base and extending to the supraglottic space. Biopsy of this tumor confirmed the diagnosis of schwannoma. An endaural approach allowed complete resection of the tumor. Definitive histopathological examination showed a plexiform schwannoma. Schwannoma or neurilemoma represents a benign tumor potentially affecting any nerve. The head and neck region is a relatively common location for schwannomas, but tongue schwannomas are considered to be rare and pose the problem of both clinical and histological differential diagnosis of tongue masses. Schwannoma or neurilemoma is a benign tumor originating from the Schwann cell of the nerve sheath. It usually presents as an individual and encapsulated tumor and can virtually affect any peripheral, autonomic, or cranial nerve. Plexiform schwannoma is an anatomical variant of schwannoma characterised by intraneural and multinodular growth, most often involving the skin and subcutaneous tissues of the head and neck region . Twenty-We report the case of a patient presenting a giant schwannoma of the tongue base, presenting as a slowly enlarging mass. Publication of this case was approved by the Ethical Committee of our institution.A 38-year-old Algerian woman in previous good health, presented with a twenty-year history of a slowly enlarging mass of the tongue. She sought for an ENT consultation at our hospital because of progressive dyspnoea, dysphonia, and severe dysphagia. Further interrogation revealed a very important asthenia (to the point that she had to stop working), involuntary loss of 10 kg in one year , and a family history of hypothyroidism. Oral cavity examination revealed a large, mobile, and hard mass of the tongue base, obstructing almost completely the oropharynx and part of the supraglottic area .We suspected an ectopic thyroid in the first place and performed a neck ultrasound that showed a thyroid gland in normal position. Doppler analysis suggested thyroiditis. Laboratory workup revealed hypothyroidism  and a high title of antithryoid peroxidase antibodies (anti-TPO), confirming Hashimoto disease.Magnetic resonance imaging (MRI) showed an 8.5 × 5 × 6 cm pedicled bilobed basilingual mass, well delimited and encapsulated .A highly cellular tissue organised in palisading fascicles, S100-positive cells, and rare atypical cells was seen at immunohistochemical examination of a biopsy of the mass, suggesting the diagnosis of schwannoma.Substitution therapy for Hashimoto disease was started immediately.Surgical removal of the tumor was decided. After induction, nasotracheal intubation was guided with a fiberoptic bronchoscope passed through the tube. We then performed an endoral excision of the mass, which presented a well-delineated plan separating it from the surrounding tongue muscles . Cold-stNo primary nerve originating this lesion was macroscopically identified during surgery. The tongue was closed by primam intentionem and the patient was immediately extubated, with an uneventful postoperative course. Definitive histopathological analysis confirmed the diagnosis of plexiform schwannoma of the tongue .The tongue is the most common location for intraoral schwannoma, followed by the palate and the oral mucosa. Tongue schwannoma shows no gender predilection and may present at any age . Often appearing as a painless and slowly enlarging mass of the tongue, when schwannomas reach a certain size they may cause dysphagia, voice changes, and breathing difficulties .Our patient had a family history of hypothyroidism, which lead us to suspect lingual thyroid. This was ruled out by ultrasonographic examination showing a morphologically normal thyroid gland in its usual basicervical position. The diagnosis of Hashimoto disease would at least partially explain the asthenia, but since there is not an established relationship between tongue schwannoma and autoimmune thyroiditis, the association of these entities in our patient is probably coincidental.Entities such as lipoma, hemangioma, eosinophilic granuloma, epidermoid and dermoid cysts, epithelial hyperplasia, granular cell tumor, benign salivary gland tumors, rhabdomyoma, leiomyoma, lingual thyroid, and lymphangioma, are all benign soft-tissue lesions that should be considered, in addition to schwannoma, in the differential diagnosis of a slowly growing and macroscopically circumscribed mass of the tongue. In some cases, glandular malignant processes, squamous cell carcinoma and sarcomas may share similar clinical features , 5.Peripheral nerve sheath tumors can be benign or malignant. Benign peripheral nerve sheath tumors include basically schwannoma and neurofibroma, the latter usually found in individuals with neurofibromatosis type 1. Malignant peripheral nerve sheath tumors are a rare variety of soft-tissue sarcoma, presenting very rarely in the head and neck region and carrying poor prognosis .Immunohistochemical examination should allow distinguishing schwannomas from other nerve-originating tumors. Two different histopathological schwannoma patterns are recognised. Antoni A, with high cellularity and little stromal matrix is the commonest form, and Antoni B, with lower cellular growth and a myxoid stroma. Plexiform schwannomas share the basic features of other schwannomas, especially its benign nature with no metastatic potential, but the distinction between plexiform schwannoma and plexiform neurofibroma or even malignant peripheral nerve sheath tumours can be more difficult , 7. SeveRadiological exams permit to assess the extension of the lesion, and they are useful as preoperative workup. Magnetic resonance imaging is the best exam to show deep lesions of oral cavity soft tissues. Even if a certain number of particular features are found in the imaging of schwannomas, none is specific. An overlap exists in the imaging characteristics of schwannoma and other soft-tissue tumors, especially neurofibroma .Treatment of schwannomas of the tongue is surgical. A transoral approach allows us to completely remove even the largest lesions, with a very low rate of morbidity and recurrency .Our patient's tumor measured 85 mm in greatest size, becoming the largest schwannoma of the tongue reported so far in the literature to our knowledge.


Aqueductal stenosis may be caused by a number of etiologies including congenital stenosis, tumor, inflammation, and, very rarely, vascular malformation. However, aqueductal stenosis caused by a developmental venous anomaly presenting as congenital hydrocephalus is even more rare, and, to the best of our knowledge, has not yet been reported in the literature. In this study, we review the literature and report the first case of congenital hydrocephalus associated with aqueductal stenosis from a developmental venous anomaly.The patient is a three-day-old, African-American baby girl with a prenatal diagnosis of hydrocephalus. She presented with a full fontanelle, splayed sutures, and macrocephaly. Postnatal magnetic resonance imaging showed triventricular hydrocephalus, suggesting aqueductal stenosis. Examination of the T1-weighted sagittal magnetic resonance imaging enhanced with gadolinium revealed a developmental venous anomaly passing through the orifice of the aqueduct. We treated the patient with a ventriculoperitoneal shunt.Ten cases of aqueductal stenosis due to venous lesions have been reported and, although these venous angiomas and developmental venous anomalies are usually considered congenital lesions, all 10 cases became symptomatic as older children and adults. Our case is the first in which aqueductal stenosis caused by a developmental venous anomaly presents as congenital hydrocephalus. We hope adding to the literature will improve understanding of this very uncommon cause of hydrocephalus and, therefore, will aid in treatment. It is common for intraventricular cerebrospinal fluid (CSF) flow to become obstructed at the aqueduct of Sylvius . The obsAlthough DVAs are thought of as congenital findings, all 10 previously reported cases became symptomatic as older children or adults. To the best of our knowledge, we present the first case of symptomatic congenital hydrocephalus from aqueductal obstruction due to a DVA.The patient transferred to our institution was a three-day-old, African-American baby girl with a diagnosis of congenital hydrocephalus. Prenatal ultrasonographic screening at 20 weeks was unremarkable. At 37 weeks, the maternal abdominal girth exceeded the expected range, and a second ultrasound revealed interval development of congenital hydrocephalus Figure . A CaesaThe baby was born healthy with no obviously anomalous anatomy aside from macrocephaly, splaying of her cranial sutures, and a full anterior fontanelle. She opened her eyes spontaneously and cried to stimulation. Her pupils were equal and reactive to light; she had normal muscle tone and was moving all extremities symmetrically. Her head circumference was 35.5 cm, which correlated to the 50th percentile.An MRI of the brain corroborated hydrocephalus with the presence of a DVA within the third ventricle Figure . Even afThe baby's head circumference gradually increased over several days, and serial ultrasound examination showed she had progressively worsening ventriculomegaly, so she underwent placement of a ventriculoperitoneal shunt on her fifth day of life.She was positioned supine with the head turned left, exposing the right occipital scalp. A curvilinear skin incision over the occipital scalp centered on the lambdoid suture was made for ventricular access. A linear skin incision in the midline periumbilical area was made to access the peritoneum for the distal end of the shunt catheter. A PS Medical low-pressure valve and distal catheter was then tunneled and passed in a typical fashion.Ultrasound was used to define the trajectory of the ventricular catheter . XanthocShe recovered well from the surgery and did not experience any post-operative complications. Routine post-operative imaging identified proper positioning of the shunt components and interval improvement of the hydrocephalus. Clinically, the patient recovered from the procedure and was subsequently discharged home in good condition.A number of congenital and acquired factors are known to cause aqueductal stenosis and subsequent obstructive hydrocephalus -3. VascuAqueductal stenosis caused by a vein or DVA is extremely rare ,10,15-17In our case and a recent report of two other cases, an MRI based on a gadolinium-enhanced T1-weighted sequence revealed the aqueductal obstruction and its causes . AqueducDVAs are usually benign lesions and considered normal variants that drain healthy brain tissue and do not need treatment because there is no risk of rupture and bleeding. Attempts to remove or occlude them may cause venous infarction or edema of normal parenchyma ,5,15. ThA discussion of the pros and cons of endoscopic third ventriculostomy (ETV) versus ventriculoperitoneal shunt (VPS) to address hydrocephalus is beyond the scope of this report. In brief, we chose to treat our patient with a VPS because of ETV's high rate of failure in the infant age group .Aqueductal stenosis and consequent obstructive hydrocephalus by a DVA is extremely rare. Congenital hydrocephalus can present in this way. Enhanced T1-weighted sagittal MRI sequences may be informative for examination of the aqueductal region. No treatment is recommended for the DVA; however, CSF diversion may be necessary for the treatment of hydrocephalus. ETV should be considered in older children with this pathology or later shunt malfunction in this population.Written informed consent was obtained from the patient's next of kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.DP and SH were major contributors in writing and editing the manuscript. WEW, DJC, and TGL provided critical revisions. AJ was a major contributor in the design, writing, and editing of the manuscript. All authors read and approved the final manuscript.


Inflammatory myofibroblastic tumor (IMT) is a rare lesion found mostly in children and young adults and originates from the lung, abdominopelvic region, and retroperitoneum. Clinical manifestations of IMT or imaging are nonspecific and diagnosis is based on histopathological and immunohistochemical findings. Minority of all IMTs will metastasize. IMT in the oral cavity is an extreme rarity and this is a first case report of IMT in maxilla causing delayed tooth eruption and multiple cervical root resorption with an 11-year-old child. The IMT reported here was positive for smooth muscle actin, vimentin, and anaplastic lymphoma kinase (ALK1) with immunohistochemistry. Only three IMTs of the jaws have been reported so far and none of them had delayed root eruption and tooth resorption. This unusual case of IMT in a child was also ALK1- positive supporting neoplastic origin of her tumor. The case presented here underscores the importance of histopathological examination of the tissue found in any root resorption especially in the case of multiple resorptions. Inflammatory myofibroblastic tumor  is composed of myofibroblastic cells with inflammatory infiltrate , 2. It iAn 11-year-old previously healthy girl with no medications or allergies had complicated eruption of the left maxillary canine tooth 23 and first premolar 24. A panoramic tomography showed a large resorption of the mesial side of the root of 24 . There wAt TUH, the bone defect was confirmed. No swelling, deep gingival pockets, or mobility of the teeth in this region were detected on the clinical examination. The adjacent teeth were vital, but there was a slight decrease in vitality related to the second premolar and first molar. The gingiva in the region showed bluish colouring. Panoramic and intraoral X-rays revealed external resorption in the roots of left maxillary lateral incisor and canine teeth. An osteolytic area in the same region (teeth 22-23) was detected consistently with an image obtained three months earlier . FurtherThe clinical and radiographic pictures were nonspecific. Resorption of multiple teeth and the perforation of palatal cortex suggested locally aggressive lesions. The differential diagnoses included aggressively behaving odontogenic tumors, nodular fasciitis, and malignant lesion. Ameloblastoma is the most common aggressive benign odontogenic tumor but it is rare in children. The resorption of adjacent teeth is common with ameloblastoma but it often presents as a swelling of jaw. Furthermore, the image in panoramic tomography was not lobulated as often found with ameloblastomas. Nodular fasciitis is a benign, reactive process with rapid growth that mimics malignant soft tissue sarcomas. However, also nodular fasciitis is usually diagnosed in adults. Nodular fasciitis is often superficially located at soft tissue mass and is associated with pain. The likely possible malignancies under consideration were malignant soft tissue sarcomas, malignant bone tumors, and Burkitt's lymphoma. Of the malignant soft tissue sarcomas, rhabdomyosarcoma  is a relatively common malignant soft tissue tumor in children. Fibrosarcoma is a malignant mesenchymal tumor of fibroblasts that may arise in the jaws, especially the mandible. It usually affects young adults but has been reported also in children. Malignant bone tumors constitute the 6th most common group of malignant neoplasms in children, with osteosarcoma being the most common pediatric bone tumor and Ewing's sarcoma family of tumors being the second. Both may result in an osteolytic image of the jaws in radiographs. Burkitt's lymphoma is related to EBV. It typically affects children and maxilla and may cause tooth displacement or loss, gingival expansion, and pain. The lesion was treated by enucleation and curettage and the beyond-repair damaged teeth with resorption were extracted and sent for histopathologic examination to the Department of Oral Pathology and Oral Radiology, Institute of Dentistry, University of Turku, Turku, Finland.Histopathological investigation revealed a compact cellular spindle cell proliferation with collagenous stroma with storiform architecture . The pro99mTcHDP) did not reveal signs of occult disease. After three years of followup, there was no recurrence.At the department of pediatric oncology, the patient was clinically examined and no symptoms or clinical signs of disease were detected locally or distantly. Furthermore, the complete blood count with C-reactive protein (CRP) and erythrocyte sedimentation rate were within normal limits. Whole body magnetic resonance imaging (MRI) or bone scintigraphy  classification of soft tissue tumors . HistomoIMT is classified as a benign lesion but local aggressiveness may be associated with chromosomal aberrations . AggressFor oral IMTs which are most likely to display local aggressiveness but not metastasize, management with complete surgical resection and 10-year postoperative reassessment has been suggested . With noThe cause of external cervical root resorption is poorly understood. Etiological factors from dental treatment (or trauma) to bruxism and developmental defects have been suggested. It is well established that certain neoplasms or cysts can cause tooth resorption. Multiple idiopathic cervical root resorptions are most frequently seen in young females. In the present case, we suggest that the resorptions were most likely caused by the tumor. Interestingly, a recent publication reported an IMT of a maxillary sinus which caused pulp necrosis of maxillary teeth . Maxillary IMTs are extremely rare. Furthermore, oral IMTs with ALK positivity present the minority of the reported cases. The case presented here underscores the importance of histopathological examination of the tissue found in any root resorption cavities especially in the cases with multiple resorptions.


Colocutaneous fistula caused by diverticulitis is relatively uncommon with colo-umbilical fistulas being even rarer. We herein report a rare case of a spontaneous colo-umbilical fistula due to diverticulitis of the sigmoid colon. The fistula developed from a diverticulum of the sigmoid colon that discharged through the umbilicus after two episodes of acute diverticulitis. The condition was successfully treated by resectional surgery. Diverticular disease is an increasingly common condition in Western countries especially among elderly people. The disease affects mostly the left colon. It is usually asymptomatic and becomes manifest only when complicated with bleeding or inflammation. Acute diverticulitis may complicate with free perforation resulting in fecal or purulent peritonitis or with pericolic abscess formation, involvement of adjacent organs, and fistulation. Fistulas occur in approximately 5% of cases either spontaneously or after surgical or drainage procedures , 2. The A 54-year-old man presented with a 15-day history of low-grade fever and continuous mild pain around the umbilicus. Three days before admission he noticed foul smelling discharge emanating from the umbilical pit. His medical history included irritable bowel syndrome, diverticulosis of the sigmoid colon, and two episodes of acute diverticulitis over the last two years that were treated conservatively.3) and C-reactive protein (2.8 mg/dL). A plain abdominal radiography was unremarkable.On physical examination the abdomen was slightly distended with mild tenderness in the left lower quadrant without palpable masses or any evidence of acute abdomen. Guarding, rigidity, and rebound tenderness were absent and bowel peristalsis was normal. The umbilicus and the periumbilical skin were inflamed and macerated, with spontaneous fecal discharge from the umbilical pit . The labAbdominal computed tomography (CT) revealed diffuse diverticulosis in the sigmoid colon with inflammatory changes and mesenteric stranding and a cavity beneath the abdominal wall at the level of the umbilicus communicating with the sigmoid colon . There wLaparotomy demonstrated a fibrotic fistulous tract between the umbilicus and the sigmoid colon lined with granulation tissue attributable to the previous attacks of diverticulitis. The affected part of the sigmoid colon along with the fistulous tract and the umbilicus were resected en bloc , and a HColonic fistulas to internal viscera such as urinary bladder, vagina, uterus, fallopian tube, ureter, jejunum, and colon or to the skin of the abdominal wall and the perineum (colocutaneous fistulas) arise either spontaneously due to perforation of a diverticular abscess into an adjacent organ or to the skin or as a complication after resection surgery for diverticulitis. Colovesical fistula is the most common internal fistula 48%) while colocutaneous fistula is relatively rare (5%) with the colo-umbilical type being even rarer. A colocutaneous fistula occurs usually after surgical intervention or percutaneous drainage of a diverticular abscess whilst spontaneous fistulation is uncommon –8. In ou% while cThe diagnosis of colocutaneous fistula is usually apparent from the history and the presence of a fistulous opening in the skin. Drainage of feces from the cutaneous opening is a constant and characteristic feature. Barium enema examination will often confirm the diagnosis. However, computed tomography is the preferred study for colocutaneous fistulas providing information on both the intra- and extraluminal features of the fistulous tract, the part of bowel involved, and the presence of abscesses, obstruction, or perforation . Water sConservative management of colocutaneous fistulas consisting of withdrawal of oral intake, fluid and electrolyte replacement and nutritional support with total parental nutrition, control of both local and systemic sepses, and local skin care results in low closure rates and has the possibility of fistula recurrence. However, it can be used as an initial step to control local or systemic sepsis, to reduce the inflammation, and to lessen the operation. In our patient, the short and wide fistulous tact made spontaneous closure of the fistula very unlikely and thus surgical management was undertaken. Currently, resection of the fistulous tract and the affected colon with primary colonic anastomosis in a single stage is the treatment of choice for diverticulitis-associated fistulas. The success rate of the single-stage procedure is approximately 90% without increased postoperative morbidity and mortality rates , 2, 5. TIn conclusion, we here presented a rare case of spontaneous colo-umbilical fistula complicating diverticulitis of the sigmoid colon which was successfully treated by resectional surgery.


Peritoneal loose bodies, or peritoneal mice, are rare asymptomatic lesions that are usually found as an incidental finding during abdominal surgery or autopsy. Giant loose bodies, measuring more than 5 cm, are rare and only a few cases are reported in the literature. These bodies are usually infarcted appendices epiploicae, which become detached and appear as a peritoneal loose body in the abdominal cavity. They may re-attach themselves to a surface, such as the lower aspect of the spleen or omentum, in which case they can be called a "parasitized peritoneal body", as in our case.We report a case of a giant loose peritoneal body measuring 7 × 5 cm found incidentally in a 64-year-old Indian man who presented with acute intestinal obstruction. We present the current hypothesis and our opinion on the genesis of such large bodies and discuss the problems in diagnosis.Peritoneal loose bodies are common but giant peritoneal loose bodies are very rare. These giant bodies usually do not require any treatment until they become complicated. Present diagnosis modalities have limitations in the diagnosis of mobile lesions in the abdominal cavity, so care must be taken to avoid unnecessary laparotomies in uncomplicated cases. Peritoneal loose bodies are rare and found incidentally at laparotomy. In most cases they are small in size . Giant loose bodies (more than 5 cm) are very rare and only a few cases have been reported in the literature . Its exaThese loose bodies are usually incidental findings that do not require any specific treatment until they become complicated -6. GenerA 64-year-old Indian man was referred with complaints of abdominal pain, vomiting and not passing flatus or feces for four days. Our patient's general condition was poor; he was febrile, with a pulse rate of 124/minute and blood pressure 90 mm/Hg. X-rays of his abdomen showed multiple air fluid levels suggestive of acute intestinal obstruction. With the provisional diagnosis of acute abdomen  our patient was resuscitated and sent for an urgent laparotomy. On exploration, our patient had severely dilated small gut loops with the terminal ileal loop twisted around the omental band and adherent to his left pelvic wall. On releasing the omental band, the ileal loop was dissected free from his left pelvic wall. Once the loops had been released, we found a large, white, oval shaped, extra-luminal body in the region of his sigmoid colon  are very rare and only a few cases have been reported in literature -10. The We believe that once an appendix epiploica gets saponified and calcified the exudative serum fluid (rich in protein) accumulates around it and, because of increased temperature in the peritoneal cavity, it gives the appearance of a boiled egg. With time, the size of the peritoneal body increases because of a gradual deposition of body serum at the periphery. Sometimes the free peritoneal body attaches to the omentum and receives a blood supply from it , as in our case.Our histological findings suggest that saponified and calcified appendices epiploicae form the yellow central part and gradual deposition of peritoneal serum around it form the outer white layer, hence giving the appearance of a boiled hen's egg.Pre-operative diagnosis of these lesions is difficult, because most of the time these lesions are asymptomatic and found during routine exploration of the abdomen for some other pathology.The most common form of presentation in symptomatic patients is causing intestinal obstruction, as in this case. If a patient presents with features of intestinal obstruction and X-ray films shows a calcified lesion in the abdomen, which moves with a change in position of the patient, there should be a high index of suspicion for diagnosis of a giant loose peritoneal body. Additional tests which can be done to diagnose peritoneal mice are CT and MRI scans, which can be useful for differentiating these from other lesions. However, it is very difficult to differentiate between these loose bodies and other abdominal benign lesions with calcification, like granuloma or tuberculosis.In our case, because the patient presented with acute intestinal obstruction and an X-ray of his abdomen only showed multiple air-fluid levels and no calcified lesions, our patient was directly taken up for an urgent laparotomy without waiting for CT or MRI scans.Peritoneal loose bodies are rare and, in most of the cases, small in size. However, giant loose bodies are very rare and only a few cases have been reported in the literature. The current hypothesis on their development is uncertain.Pre-operative diagnosis of these lesions is difficult and a high index of suspicion should be kept in any symptomatic patient with a mobile lesion in the abdomen or a calcified lesion in the pelvis on X-ray.No specific treatment is required in asymptomatic patients, however if these entities become associated with complications like intestinal obstruction, or if there is an abdominal mass of obscure origin, or when diagnosis is in doubt, then exploration is required.Written informed consent for publication could not be obtained despite all reasonable attempts. Every effort has been made to protect the identity of our patient and there is no reason to believe that our patient would object to publication.The authors declare that they have no competing interests.AS assisted in the operation, contributed to manuscript conception, research, acquisition of data, drafting and writing of the manuscript as well as pre-operative and post-operative management of the patient. AJ carried out the histopathological evaluation and critically review of the manuscript. KKM carried out the critical review and revision of the manuscript. SV carried out the operation and contributed to the critical review of the manuscript. All authors read and approved the final manuscript.


Ectopic brain anaplastic ependymomas with no connection to the ventricles are rare. We present a rare case of a 25-year-old male who presented with generalized convulsions. Computed tomography (CT), Magnetic Resonance Imaging (MRI), and magnetic resonance spectroscopy (MRS) showed characters of an intra- and extra-axial lesion. Intraoperatively, the lesion was a cortical solid mass that had no connections to the dura or to the ventricle. The histological diagnosis showed an anaplastic ependymoma with WHO grade III with distinctive desmoplasia. A literature review of ectopic anaplastic ependymomas regarding their clinical presentations, management, and prognostic factors was performed. There is a need to establish a clinically based histopathological grading system for anaplastic ependymomas. Ectopic anaplastic ependymomas should be included in the preoperative differential diagnosis. Ependymomas are a subtype of glioma that arise from the ependymal cells within the ventricles and the central canal of the spinal cord. These types of tumors account for 1.9% of all primary CNS tumors .Ependymomas arising outside of the ventricles and that do not have any connections to the ventricles specially cortical ependymomas are very rare .Anaplastic ependymomas account for 8.6% to 11.5% of all ependymomas . The optWe present a rare case of a cortical anaplastic ependymoma that preoperatively was not typical for intra-axial or extra-axial lesion. We reviewed all cases of ectopic anaplastic ependymomas without connections to the ventricles in the literature to gather information regarding their sites, clinical presentations, pathological features, management, and outcomes.A right-handed 25-year-old male presented with a generalized convulsion. He reported having focal-like seizures for years without treatment. He reported slight numbness in his left fourth and fifth fingers. No other symptoms or neurological deficits were present.The initial brain CT revealed a mass in the right central area that appeared to be a meningioma. An MRI 3-Tesla scan of the brain demonstrated a solid, well-demarcated homogeneous mass which may have a dural attachment . An MRS A frontoparietal craniotomy was performed with MRI-guided navigational assistance. Grossly, the lesion was a cortical firm, gray, and solid mass with no connection to the dura matter. The gross lesion appearance and separation from the dural matter allowed for clear planes of dissection between the tumor and normal brain tissue. Intraoperatively, the tumor was not found to be connected to the ventricle. A gross total resection of the mass was achieved. Postoperatively, the patient had no deficits. The preoperative light numbness in his left hand improved during the hospital stay. After diagnosis of an anaplastic ependymoma, an MRI of the total neural axis was performed, without evidence of droop metastasis.On histological examination, the tumor was primarily of high cellularity and had sharp borders with the surrounding CNS tissue, in which piloid gliosis with rosenthal fibers was seen. The tumor cells were diffusely distributed with little fibrillary intercellular substance. The tumor cell nuclei had finely dispersed chromatin with moderate anisonucleosis and some giant nuclei. Mitotic activity was increased, with up to four mitotic figures seen per high-power field, 11/10 hpf. In fields of lower cellular density, perivascular pseudorosettes were seen. Gemistocytic cells and calcifications were distributed throughout the tumor tissue. Focally, the tumor tissue contained many collagenous fibers.Immunohistochemically, the tumor cells expressed GFAP, S100, and vimentin. An anti-EMA reaction showed dot-like staining of the microlumina. CD34 was only expressed in the endothelial cells. Thirty percent (30%) of the tumor cell nuclei showed a positive anti-Ki67 reaction.On electron microscopy, some microlumina and remnants of cilia were detected Figures , and 6.The final diagnosis was an anaplastic ependymoma with WHO grade III and distinctive desmoplasia Figures , and 6.Follow-up at 6 months revealed that the patient had well-controlled epileptic seizure activity, and an MRI of the brain showed no evidence of residual tumor or tumor recurrence. Adjuvant local radiotherapy was performed .We identified 24 cases of ectopic anaplastic ependymomas with no connections to the ventricle. There were 13 males (54.2%) and 11 females (45.8%), and the ages ranged between 0.3 and 70 years (mean 28.8 years). Of the 24 ectopic anaplastic ependymoma cases, 22 were located supratentorially (91.7%) and 2 were located infratentorially (8.3%). Nineteen cases (79.2%) were intra-axial and 5 (20.8%) were extra-axial ependymomas. The location, clinical presentation, radiological finding, and outcomes are summarized in The frontal location was dominant, being reported in 11 cases (45.9%), including 2 frontoparietal, one frontotemporal, and one central. The most often reported clinical presentation was seizure and medically intractable epilepsy. The most often reported radiological appearance was solid with cystic formation. Preoperatively, 2 cases mimicked a meningioma, and one case mimicked a glioblastoma. Of the 24 anaplastic ependymomas, there was one case in which the exact grading is debatable . SurgicaOf the 24 cases, 12 (50%) patients had no postoperative deficits, 2 (4.2%) had mild deficits, one (4.2%) patient was handicapped, and one (4.2%) patient was bedridden. Six patients (27.3%) died during the follow-up period.Ectopic brain ependymomas that do not have any connection to the ventricular system have been reported in all regions of the brain –13.Our unique case had isointense signals on T1- and T2-weighted images and homogeneous contrast enhancement with no dural tail sign. The MRS showed characteristics of an extra-axial lesion. Our case reflects the difficulty in differentiating ectopic anaplastic ependymomas from other dural-based extra-axial lesions and other gliomas on the basis of signal characteristics alone. Due to the rarity of ectopic ependymomas, these tumors are generally not included in the differential diagnosis. Several authors reported anaplastic ependymomas that mimicked meningiomas , 14.Despite the malignant designation of anaplastic ependymomas, they tend to be solid and well demarcated with limited infiltration to the edges of the lesion. The diagnosis of an ectopic anaplastic ependymoma is not easy. The diagnosis varies widely depending on the pathologist's experience with ependymomas –17. It wSurgical excision with adjuvant radiotherapy is the primary management of anaplastic ependymomas , 19, 20.The 5- and 10-year survival rates reported in the literature were 65% und 37%, respectively, with a great disparity among the studies , 29–31. Site-related outcomes were reported with the worst outcomes for intraparenchymal anaplastic ependymomas . SuccessAn increased risk of recurrence was reported with a high histological grade, incomplete resection, and a Karnofsky performance status that is less than or equal to 80 .Tumor grade as a prognostic factor was contradictory; certain authors reported that tumor grade was an independent prognostic factor that influences outcome , and othThere is a need to establish a clinically based histopathological grading system for anaplastic ependymomas and a need to increase the awareness of these lesions during preoperative studies.


Tumor lysis syndrome (TLS) is an oncologic emergency that is caused by massive tumor cell lysis. It is commonly associated with hematological cancers like leukemia and lymphoma and uncommonly with solid nonhematologic tumors as well. However, spontaneous tumor lysis syndrome (STLS) without any cytotoxic chemotherapy rarely occurs in solid tumors. We describe a case of STLS in a metastatic adenocarcinoma of unknown primary and review the literature of STLS in solid non-hematologic tumors to identify various risk factors for pathogenesis of this entity. A 59-year-old Caucasian female with past medical history of hypertension, obesity, mild osteopenia, and >20 pack years smoking history presented to the primary care physician with a more than four-month history of generalized weakness, anorexia, weight loss of more than 30 pounds, a growing subcutaneous mass in the right lower back and back pain in the lumbar region. MRI of the spine revealed confluent bulky soft tissue mass measuring approximately 14 cm anteroposterior × 13 cm transverse in size in the retroperitoneum region and pathological compression fracture of L1 vertebrae. Further, CT scan of chest/abdomen/pelvis confirmed bulky retroperitoneal mass/adenopathy with extensive liver metastases and multiple tiny pulmonary nodules .The mass at lower back was excised and immunohistochemical staining of subcutaneous mass was positive for cytokeratin 7, cytokeratin 20, mucicarmine and villin but negative for ER, mammaglobin, TTF-1, napsin A, CDX2, p63, calretinin, and hepatocyte antigen. The villin positivity in conjunction with the cytokeratin 7 positive expression is suggestive of a noncolorectal gastrointestinal origin, including pancreaticobiliary/gallbladder source. The tissue of origin assay was unable to locate the primary source and subsequently, diagnosis of poorly differentiated adenocarcinoma of unknown primary was rendered. Ten days after the biopsy, while waiting for chemotherapy to begin, patient presented to her primary care physician with severe nausea and vomiting, altered mental status and decreased urine output. Basic metabolic panel (Chem-7) was consistent with dehydration with blood urea nitrogen (BUN) and creatinine of 31 mg/dL and 1.6 mg/dL, respectively, elevated from her baseline normal values about 10 days later. Patient was given a liter of intravenous fluid and was sent back home with antiemetics.Four days later, after nausea and vomiting persisted with lethargy, weakness and very poor appetite, patient presented to emergency room. Chem-7 revealed BUN of 117 mg/dL with a creatinine of 7.5 mg/dL. Other pertinent lab values with normal values in parentheses showed calcium 6.5 (8.9–10.3) mg/dL, phosphorus 8.8 (2.6–4.6) mg/dL, uric acid 26.5 (2.8–6.6) mg/dL, and lactate dehydrogenase 1265 IU/L with normal transaminases. Obstructive uropathy was excluded by retroperitoneal ultrasound. Patient went into oliguric acute renal failure (ARF) and was treated with intravenous fluids. However, renal function did not improve over the next two days, and hemodialysis was started along with rasburicase. Lab abnormalities were consistent with TLS. Due to poor prognosis, patient was not started on chemotherapy and discharged to hospice without chemotherapy, and she died three weeks later.Tumor lysis syndrome (TLS) is an oncologic emergency that is caused by massive tumor cell lysis with the release of large amounts of potassium, phosphate, and nucleic acids into the systemic circulation. The Cairo-Bishop definition of TLS , proposelaboratory TLS is defined as either a 25% change or level above or below normal for any two or more serum values of uric acid, potassium, phosphate, and calcium within 3 days before or 7 days after the initiation of chemotherapy in the setting of adequate hydration and without use of any uricosuric agent;clinical TLS is defined as laboratory TLS plus one or more of the following that was not directly attributed to a therapeutic agent: increased serum creatinine ;TLS most often occurs after the initiation of cytotoxic therapy in patients with high-grade lymphomas (particularly the Burkitt subtype) and acute lymphoblastic leukemia. TLS has been rarely described after treatment of nonhematologic solid tumors including breast cancer, germ cell tumors , small-cWe believe that our case had STLS because of evidence of necrosis in the tumor and was probably precipitated by the dehydration aggravated due to nausea and vomiting. We excluded other causes for ARF, such as postrenal obstruction (by ultrasound and CT scan), urinary tract infection , parenchymal kidney disease . Elevated serum uric acid is present in acute renal failure of any cause, especially in prerenal azotemia, but values are generally below <15 mg/dL except in TLS. In our patient, serum uric acid >25 mg/dL was again consistent with TLS. The typical laboratory findings in outpatient such as hyperuricemia, hyperkalemia, hypocalcemia, hyperphosphatemia, and ARF met the laboratory and clinical criteria of TLS.According to TLS expert panel guidelines , most soThe risk factors for STLS in solid tumors, based on the review of literature and these case reports, include chemotherapy sensitive or high proliferative index tumors, tumor burden, presence of liver and bone metastasis, abnormal lab values including elevated serum uric acid, serum LDH, and concomitant presence of risk factors for renal failure. The most important risk factor was the tumor burden with every report documenting stage 4 cancers having extensive liver and bony metastases and presence of big-sized masses (>10 cm), which bestow them high predilection to undergo autonecrosis releasing intracellular contents. The biopsy in most of the cases revealed necrotic tissue in the center of the masses as well as in liver and bone metastases. The metastases in liver and bone compared to an abdominal mass are at higher risk to undergo ischemic central auto-necrosis due to pressure induced by surrounding normal parenchyma on a fast growing tumor, whereas, an intra-abdominal mass like in retroperitoneal region can grow to a significant size without lysis.Another important risk factor would be preexistent impaired renal function that can be further compromised with the progression of the syndrome. The renal function deterioration can lead to deficient clearance of elevated uric acid produced by tumor lysis and deposition of the crystals in renal tubules (uric acid nephropathy). The use of nephrotoxic agents like intravenous contrasts agents, nonsteroidal anti-inflammatory drugs, nephrotoxic antibiotics and drugs that inhibit uric acid secretion  should be avoided.STLS is not as common in solid tumors compared to hematologic malignancies, but we believe that it might be an underdiagnosed entity, as many cases of mild grade TLS with less deranged abnormalities in the laboratory parameters can be missed easily. The patients with above risk factors should be treated prophylactically with aggressive hydration and uricolytic agents before the initiation of chemotherapy. Our case highlights the criticality of identifying STLS in solid tumors with big tumor burden and treating prophylactically to prevent this cataclysmic syndrome.