Introduction. We present the extremely rare case of a patient with three metachronous osteosarcomas within 22 years without evident pulmonary manifestation of disease 30 years after first diagnosis. Case Presentation. In 1983, a high-grade osteosarcoma of the left distal femur was diagnosed in an 18-year-old Caucasian male. He received rotationplasty accompanied by pre- and postoperative chemotherapy. Ten years later, an osteoblastic osteosarcoma occurred in TH12. En bloc resection and pre- and postoperative chemotherapy followed. In 2005, the patient developed another high-grade osteosarcoma in his right distal femur. Treatment included a wide resection and reconstruction with a tumour endoprosthesis as well as (neo)adjuvant chemotherapy. After the third tumour occurrence, cytogenetic and molecular genetic examinations  were performed, showing a normal genetic pattern. Screening for metastases never showed clinical evidence of extraskeletal tumour manifestation. Discussion. In patients presenting metachronous osteosarcoma, identification of their lesions clonality (second primary tumour or metastases) could lead to a better understanding of tumour development and help to filter patients who need extended long-term followup due to a higher risk of late occurring sarcoma recurrence. Osteosarcoma occurs with an incidence of four to five cases per million inhabitants per year, representing the most common nonhaematopoietic primary malignant bone tumour . It is dNevertheless, some patients still experience recurrences, and of those, less than one quarter become long-term survivors . The higThe pathogeneses of metachronous osteosarcoma are still unclear. They are discussed either as metastases of the primary tumour to the bone or second primary lesions. Histopathology does not offer sufficient methods of differentiation yet . TheorieHerein, we present the extremely rare case of a patient with three osteosarcomas within 22 years in different sites of the skeletal system without any other manifestation of disease 30 years after first diagnosis.In 1983, a high-grade osteosarcoma of the left distal femur was diagnosed by biopsy in an 18-year-old Caucasian male at the University Hospital Vienna. The patient did not show evidence of pulmonary metastases. He received preoperative chemotherapy according to the Rosen T10 protocol, based on high-dose Methotrexate monotherapy, showing a regression grade of five  on a six-part scale system according to Salzer Kuntschik et al.  . RotatioTen years later, in 1993, an osteoblastic osteosarcoma occurred in the twelfth thoracic vertebral body, again without any other sign of systemic manifestation of disease. After preoperative chemotherapy using Carboplatin/Etoposide, the patient received en bloc resection and dorsal stabilisation from TH12 to L3 at the same institution. Histologic workup revealed a regression grade of three according to the Salzer Kuntschik scale and postoperative adjuvant chemotherapy with Adriamycin for one cycle and Carboplatin/Etoposide for 4 cycles was administered.In 2005, 12 years after vertebral resection, the same patient showed swelling and pain in his right distal femur. The following biopsy at the University Hospital Graz revealed again a high-grade osteosarcoma , and oncMobilisation was difficult due to the rotationplasty of 1983 on the left leg and the recently implanted tumour endoprosthesis on the right one. Two months after the last operation during the ongoing adjuvant chemotherapy, our patient fell and suffered from an open luxation of the recently operated knee. Revision surgery with change of prosthesis was necessary. After the revision procedure, no further complications occurred and the patient is now able to walk without braces or canes representing a good functional outcome . At the After the second tumour relapse, we performed cytogenetic and molecular genetic examination after obtaining our patient's written informed consent. Based on the major genetic alterations mentioned in the literature as predisposing to osteosarcoma development, we were focusing on Li-Fraumeni-like syndrome (p53 gene mutation) and retinoblastoma syndrome (RB 1 gene mutation), but no mutations in the target genes could be found.Development of interdisciplinary osteosarcoma treatment has increased patients survival and the event free interval concerning tumour relapse. Nevertheless, patients still experience tumour recurrence. In this context, pulmonary metastases, with or without secondary bone lesions, are seen more frequently than cases of solitary osseous recurrences . ReportsGenerally, the prognosis of patients who relapse with bone lesions is worse than that of patients with pulmonary metastases. In a study cohort of 52 patients at the Rizzoli Institute, only patients with single, late appearing, and resectable bone malignancies presented a similar outcome compared to patients with secondary lesions to the lungs . Aung etA possible interpretation concerning the poorer outcome in patients with early recurrence <24 months), compared to those with late occurring tumours (>24 months), was given by Brandal et al. [4 months,Besides worse life expectancy, Bacci et al.  found a Rodriguez et al.  stated tConditions predisposing to osteosarcoma development, including tumour suppressor pathway alterations of the p53 gene, finding clinical presentation in the Li-Fraumeni-like syndrome, and retinoblastoma gene 1 germline mutations, are well described in literature in the context of primary osteosarcoma development . NeverthAnother hypothesis concerning the differentiation of tumour metastases from second primary lesions was given by Franke et al. . They asOn the other hand, the two tumours in the lower limbs were both responding to chemotherapy with a regression grade of five (>50% viable tumour cells) according to Salzer Kuntschik , whilst The treatment of metachronous osteosarcoma remains difficult. San-Julian et al.  suggesteThe precise etiology of metachronous osteosarcoma still remains unclear. In our case, a genetic component is obvious, although we could not find an association with the so far known osteosarcoma related main gene mutations. Identification of those lesions' clonality (second primary tumours versus metastases) could lead to a better understanding of tumour development and help to filter patients who need extended long-term followup due to a higher risk of late occurring sarcoma recurrence.


The current study reports a case of multiple microvenular hemangioma (MH). A 35-year-old male presented with dark red maculopapules on the trunk and limbs that had been apparent for 5 years. The number of lesions exceeded 100 in total. A histological examination demonstrated multiple, irregular, branching venules in the dermis, without any endothelial atypia. On immunohistochemical analysis of the vascular structures, the endothelial cells stained positive for CD31, CD34 and factor VIII, and the perivascular cells stained positive for SMA and HHF-35. These observations were consistent with a diagnosis of MH, and should be differentiated from the most common differential diagnosis of patch-stage Kaposi’s sarcoma. There was no clear effect following topical application of recombinant human interferon α-2b gel. MH usually presents as a solitary, purple-to-red papule/plaque measuring 5–20 mm in diameter. MH was first described by Bantel A 35-year-old male presented to Cangzhou Central Hospital  in September 2009 with multiple dark red maculopapules on the trunk and limbs . The patUpon examination, numerous dark red, circular, non-scaly maculopapules measuring 5 mm in diameter were noted on the chest, back, abdomen, buttocks and upper and lower extremities. The lesions had a diffuse distribution with clear borders and were painless. An attempt to count the number of lesions found >100. The lesions were found in a greater concentration on the chest and back. The head, face, palms and soles were unaffected.Nothing abnormal was found on chest X-ray or abdominal B-mode ultrasound scan. The patient’s blood profile, urine and stool routine, liver function, renal function and electrolyte levels were also normal.Two lesions of the chest were removed completely for diagnosis. The tumors were of the same histopathology and demonstrated proliferation of irregularly branched, thin-walled venules infiltrating the sclerotic dermal collagen and the presence of few blood vessels with inconspicuous lumina . A mixtuImmunohistochemically, the endothelial cells of the proliferating vessels expressed CD31 , CD34 F and factet al in 1989. At that time, the study reported microcapillary angiomas in three female patients  . HoweverAdditional differential diagnoses include multinucleate cell angiohistiocytoma (MCA) and reactive angioendotheliomatosis (RAE). In MCA, the characteristic cells are large, with irregular, unusually-shaped, scalloped or angular margins . The RAEThe distinctive feature of the present case that makes it an important academic observation is that, besides the multiple skin lesions, there are also epidermal changes on the histopathology, which include spreading and interdigitating epidermal processes and an increase in the basal lamina pigmentation. Furthermore, the course of this case was longer than usually observed in MH.For cases of this disease with minor solitary lesions, excision has been associated with cure and usually no recurrence is observed. In cases with major or multiple skin eruptions, regular follow-ups should be performed, although the disease is self-limiting in nature. In the present case, some primary lesions disappeared without further treatment; however, a few new eruptions appeared on the patient’s back and chest.


Traditionally, there are several approaches to manage left-sided colonic carcinoma obstruction, such as tumor resection with primary anastomosis, tumor resection with end-colostomy and loop-colostomy. Recently, colonic stent insertion was introduced as a bridge prior to definite surgery. We demonstrated a hybrid approach for obstructed sigmoid carcinoma using colonic stent, followed by single incision laparoscopic colectomy (SILC). A 58 year-old man presented with complete left-sided colonic obstruction. He underwent emergency colonoscopy with metallic stent placement. One week later, he was performed SILC. He recovered well after the operation without any postoperative complications. The pathological result showed adequacy of oncologic resection. This hybrid approach of colonic stent insertion and SILC can be safely performed. Eight to twenty-nine percent of colorectal cancer patients presented with colonic obstruction -4. The oLaparoscopic colectomy for colon cancer treatment became more popular in the past decade. The data from several studies -13 showeThe approach with placement of colonic stent before laparoscopic colectomy seems to be an ideal approach for left-sided colonic obstruction -18. Howe2) with underlying of seizure who presented with abdominal distention, constipation and vomiting 4 days. On physical examination revealed dehydration, abdominal distention and hyperactive bowel sound. Plain abdominal films revealed dilation of small bowel and large bowel till sigmoid colon.  was placed over the wire  and divided. The dissection was continued proximally to splenic flexure and upper rectum distally. Sheath was incised continuously. Wound retractor  was applied. Left sided colon was bringing to abdominal wall. Resected specimen was removed.  and presented of perineural invasion. Two of 14 lymph nodes showed metastatic carcinoma (pN1a). Proximal and distal margins were uninvolved. At present he is doing well.Malignant left-sided colonic obstruction is the most common etiology for emergency condition in colon cancer . TraditiDohmoto  who is tP = 0.002). Oral intake was resumed earlier in SLAP . Similarly of positive results, Cheung et al [P = 0.04). None of the patient in endo-laparoscopic group had a permanent stoma compared with 6 patients in the open surgery group (P = 0.03). However there is a recent RCT from France [There are several studies showed the good results when use colonic stent as a bridge before laparoscopic surgery -27. Parkng et al  reportedm France  which coSingle incision laparoscopic surgery is a new emerging laparoscopic technique, which performed colonic resection through only one incision. Some papers proposed of safety and feasibility of this technique -17. In aThis is the initial report for management of left-sided colonic obstruction with colonic stent insertion followed by SILC that showed the good results of recovery period. Moreover, oncologic resection is still adequate. However, we need more patients to enroll for the further study.In Summary, a new hybrid approach using colonic stent as a bridging therapy combine with SILC can be performed with promising results. This approach might be a good alternative way to manage left-sided colonic obstruction. Nevertheless, to determine its benefits, larger prospective comparative studies to standard open or laparoscopic colectomy with cost analysis, oncologic outcomes, and long-term follow-up will be necessary.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.AT and TA designed study and performed the operation. AT and TA wrote the paper. All authors read and proved the final manuscript.


Introduction. Intestinal ischaemia is a devastating disease process that could lead to bowel gangrene and death if either not diagnosed early or left untreated; death is usually caused by irreversible shock, intestinal necrosis, or septicaemia. It is usually seen in elderly patients with atherosclerotic disease. The course of bowel ischaemia may affect variable lengths of the intestine and it is not unusual for the condition to be followed by uneventful recovery. Case presentation. We are reporting an unusually rare case where an elderly patient passed an extraordinarily long segment of bowel, including rectum, the whole of the large bowel, and part of the small bowel, through anus following an episode of nonobstructive mesenteric ischaemia (NOMI) complicating myocardial infarction. To our knowledge, there are only eight cases reported in the literature where the condition was diagnosed upon the passage of short segments of the large bowel particularly of the rectosigmoid segment through the anus. Conclusion. Physician should keep a high level of suspicion in order to prevent it or at least recognise it early on and offer adequate management and hence reducing morbidity and mortality. An elderly female patient was treated for a fractured right neck of femur. Her Auston-Moore right hip replacement was uneventful. One week later, she became unwell with increasing shortness of breath and abdominal distension. A diagnosis of posterior myocardial infarction and pseudo-obstruction of the bowel was made and treated accordingly. Her general condition was fluctuating with recurrent diarrhoea, swinging temperature, and persistent confusion. Three weeks following her admission and while on the rehabilitation ward, a foul vaginal discharge was noted and a cordlike piece of tissue was seen protruding through the vagina. Later on, a piece of bowel was passed out through the vagina and placed in formalin pot following resection. The patient had had a vaginal shelf pessary fitted sometime ago. A diagnosis of vaginoenteric fistula was strongly suspected. The patient was too unwell for imaging studies such as MRI or CT scan. During this period and despite intensive treatment of her septicaemia, she continued to deteriorate and passed away six weeks following the hip replacement. A postmortem examination was refused by the relatives.The specimen was a long cordlike greenish yellow tissue measuring 1350 × 20 mm maximum without any identifiable viable tissue . Microscopic examination of sections of the tissue revealed predominantly large and partly small bowel tissue with different and variable thickness ranging from mucosa and submucosa up to and including the inner and outer layers of the muscularis propria Figures .3 of descending colon down to the upper rectum was passed per anum [Spontaneous passage of a large bowel cast caused by acute ischaemic injury is an extraordinary complication of mesenteric ischaemia. A literature search through PubMed using the keywords colon, rectum, ischaemia, infarction, sloughed, passed, per vaginam, and per rectum revealed 8 cases in which a short segment ranging between 25 cm of recto-sigmoid and 96 cmper anum –6. HowevOf the eight reported cases, ischaemic complication occurred following abdominal aortic aneurysm repair in five cases; all patients survived this complication except for one case of a 67-year-old male who passed a full thickness cast of sigmoid colon 25 cm long following abdominal aortic aneurysm repair; the latter case was reported by Sado et al. in J Jpn Soc Colorectal Dis (in Japanese) . Whilst  The mortality following bowel ischaemia remains high although it has dropped from 85% to 60% over the last 30 years due to advances in early diagnosis and management. However, the latter, as expected, has led to increase in the incidence of this entity . The aetiology of bowel ischaemia is obviously secondary to a compromise in blood flow to the bowel. This may be attributed to a predisposing obstructive factor causing segmental ischaemia or has a non-obstructive aetiology. The latter is usually secondary to cardiovascular events and could affect the major supply of the celiac axis and superior and inferior mesenteric arteries. The likely causes of non-obstructive intestinal ischaemia includedissecting aortic aneurysm with secondary compression of both main mesenteric arteries, vasospasm of both mesenteric arteries secondary to medications for example, digoxin, ergot, and cocain intoxication , low-flow phenomenon precipitated by hypovolaemia, severe hypotension, or shock ,haematological clotting causes predisposing to excessive clotting mechanism.In our case report, there was no history of clotting disorder, vasoactive medication, or aortic dissection. Other than the unlikely event of two simultaneous and separate thrombi of the superior and inferior mesenteric arteries, it seems that low-flow phenomenon complicating myocardial infarction and progressing to extensive intestinal tissue necrosis and pronounced sloughing of this tremendous length of bowel is the most likely pathological explanation to what had happened to our patient. Mortality is usually very high if this unexpected extensive and severe intestinal infarction presented together with the recent myocardial infarction.NOMI accounts for 15–20% of acute cases of intestinal ischaemia and is mostly seen in patients who are already critically ill . SystemiThis lady had had a vaginal ring pessary fitted for uterine prolapse. The clinical history is clear that there was a significant foul vaginal discharge and a cordlike pieces of tissue protruding from the vagina. It is clinically recognised that neglected vaginal pessary in certain circumstances might erode through vaginal wall predisposing to a fistula .This is a very unusual case of extensive non-obstructive intestinal ischaemia affecting the area supplied by the superior and inferior mesenteric arteries, that is, including rectum, the whole of the large bowel, and part of the small bowel. Ischaemic change was most likely secondary to a low flow phenomenon precipitated by prior myocardial infarction. Physician should keep a high level of suspicion in order to prevent it or at least recognise it early on and offer adequate management and hence reducing morbidity and mortality.


Mucosa associated lymphoid tissue lymphomas are the most common lymphomas of the salivary glands. The benign lymphoepithelial lesion is also a lymphoproliferative disease that develops in the parotid gland. In the present case report, we describe one case of benign lymphoepithelial lesion with a subsequent low transformation to grade mucosa associated lymphoid tissue lymphoma appearing as a cystic mass in the parotid gland.A 78-year-old Caucasian female smoker was referred to our clinic with a non-tender left facial swelling that had been present for approximately three years. The patient underwent resection of the left parotid gland with preservation of the left facial nerve through a preauricular incision. The pathology report was consistent with a low-grade marginal-zone B-cell non-Hodgkin lymphoma (mucosa associated lymphoid tissue lymphoma) following benign lymphoepithelial lesion of the gland.Salivary gland mucosa associated lymphoid tissue lymphoma should be considered in the differential diagnosis of cystic or bilateral salivary gland lesions. Parotidectomy is recommended in order to treat the tumor and to ensure histological diagnosis for further follow-up planning. Radiotherapy and chemotherapy should be considered in association with surgery in disseminated forms or after removal. Mucosa associated lymphoid tissue (MALT) lymphomas are non-encapsulated clusters of lymphocytes found throughout the mucosal tissues of the aero-digestive tract. The non-Hodgkin type lymphomas that arise from these lymphocyte aggregates (MALT lymphoma) are of B-cell lineage, the commonest involving the salivary glands . A MALT The lymphoma arising from MALT was first described by Isaacson and Wright in 1983 . MALT lyIn the present case report, we describe one case of BLL with a subsequent low transformation to grade MALT lymphoma appearing as a cystic mass in the parotid gland.A 78-year-old Caucasian female smoker was referred to our clinic with a non-tender left facial swelling that had been present for approximately three years. The patient was otherwise asymptomatic. She had no history of malignancy or autoimmune diseases. A firm mobile mass was present in the left parotid gland.Examination revealed a 5 cm firm mobile mass in the superficial lobe of the left parotid. The left facial nerve was intact . No other abnormalities were found in the nasopharynx, oral cavity, larynx or ears. There was no pathological enlargement of the cervical lymph nodes. Laboratory tests were within normal limits. Hepatitis B virus (HBV) and hepatitis C virus (HCV) serologies were negative.The patient had undergone ultrasonography-guided fine needle aspiration of the left parotid gland several months before. The cytological examination revealed mononuclear and inflammatory cells and a diagnosis of a chronic parotiditis was made. The patient was given antibiotics but with little effect. Unfortunately, the mass on the left parotid continued to enlarge. After admission to our clinic, a helical CT scan was performed which revealed a solid mass with an irregular surface in the left parotid gland Figure .The patient underwent resection of the left parotid gland with preservation of the left facial nerve through a preauricular incision Figure . IdentifThe pathology report was consistent with a low-grade marginal-zone B-cell non-Hodgkin lymphoma (MALT lymphoma) following BLL of the gland. According to the report, there was infiltration of the normal salivary tissue by a heterogenous mixture of lymphocytes and isolated blastic cells.Postoperative recovery was uneventful and facial nerve function was intact. CT scan of the head, neck, chest and abdomen at two and six months after the surgery revealed no evidence of lymphoma infiltration. Our patient had isolated surgical treatment without chemotherapy. By the time this report was completed, the patient had been followed for 13 months without evidence of recurrence.Primary lymphomas of the salivary glands are rare and account for 4.7% of lymphomas at all sites -11. TherThe native absence of MALT within the salivary glands necessitates the development of acquired MALT from underlying lymphoid stimulation and infiltration before MALT lymphoma can develop . Low-graAssociation between MALT lymphoma and autoimmune diseases such as systemic lupus erythematous ,18 or inAccording to Anacak et al  salivaryRadiological representation of MALT lymphomas of the parotid gland is scarce . AccordiCurrently, there is controversy in the reported literature regarding the accuracy of PET-CT scan in MALT lymphomas. Elstrom et al  evaluateMost non-gastric MALT lymphomas have been noted to be indolent. Disseminated disease is relatively slow to develop in affected patients. Up to 50% of the patients with non-gastric MALT lymphoma have multiple involved sites . WhetherAn association between hepatitis C virus (HCV) infection and B-cell lymphomas has previously been reported, especially in countries in which the prevalence of HCV is relatively high ,32. OtheIn our practice, clinical examination, preoperative FNA of the suspicious lesion and radiological investigation in certain cases, is part of the preoperative assessment of a suspicious parotid gland lesion. However, Ando et al  suggesteThieblemont et al  suggesteOnce MALT lymphoma is diagnosed, an in-depth evaluation for synchronous multi-sited involvement and disseminated disease should be undertaken before initiation of local therapy. Radiotherapy and chemotherapy should be considered in association with surgery in disseminated forms or after removal. Sarris et al suggested  irradiatRegional and distant relapses are not common in gastric MALT lymphomas, but extragastric MALT lymphomas tend to be more aggressive and may recur in the regional or distant lymph nodes and in other organs ,41. AccoSalivary gland MALT lymphoma should be considered in the differential diagnosis of cystic or bilateral salivary gland lesions. After histopathological confirmation of a suspicious parotid gland lesion as a parotid gland MALT lymphoma, careful follow-up is needed with attention either to the remaining parotid gland or to other major salivary glands and organs in the head and neck region.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.PKo prepared the manuscript and reviewed it for publication. ES performed the review of the literature. PKa collected the patients' data. GK supervised the general management and follow-up of the patient and the writing of the manuscript. All authors read and approved the final manuscript.MALT: mucosa associated lymphoid tissue; BLL: benign lymphoepithelial lesion


A 30-month-old male infant presented with sacrococcygeal and pre-sacral mass. Ultrasound (US) abdomen revealed a huge pre-sacral mass with irregular margins extending into the pelvis, pushing the rectum antero-laterally. CT scan and MRI confirmed the US findings. Serum alpha fetoprotein level was abnormally elevated. Histopathological examination of surgical-specimen suggested sacrococcygeal yolk sac tumor (YST).  The majority of sacrococcygeal tumors are benign, teratomas [1]. These tumors however have the potential for malignant degeneration. Malignancy is usually limited to a single element, a yolk sac tumor also known as endodermal sinus tumor (EST) [2]. This tumor may less commonly be present in "pure" form. Malignant germ cell tumors account for 3% of childhood neoplasms and yolk sac tumor is the most common histopathological subtype [3]. Although, most germ cell tumors in children originate in the gonads, the most common primary site for YST is the sacrococcygeal region [4]. Due to the rarity of sacrococcygeal YST only few case reports and small series have been reported. Here we present a case of primary YST of the sacrococcygeal region.A 30-month-old infant presented for a right buttock soft tissue mass and constipation. The mass was a 6 cm x 4 cm nodular area of induration. The child had a normal birth history with no significant past medical history. Physical examination was otherwise normal for a healthy, appropriately developing baby. Serum alpha fetoprotein level was 706 ng/ml . An ultrasound of the right buttock demonstrated a solid soft tissue hypoechoic mass, sized 6 cm x 4 cm. Abdomino-pelvic computerized tomography (CT) scan showed an irregular 6 x 7.5 x 5.5 cm solid mass of low-density invading the right greatest gluteal muscle. The mass was depressing the sigmoid and the rectum. No invasion of the spine was seen. Abdominal and pelvic magnetic resonance imaging (MRI) of the pelvis demonstrated a large heterogeneous pelvic mass extending into the right gluteal region with significant mass-effect on adjacent organs . It showed infiltration of lower sacral spinal canal. No lesions were seen on a radiograph of the chest. Surgical-biopsy consisted of whitish gray, friable and hemorrhagic fragments. Histopathological examination showed a tumor with papillary structures and several glomeruloid aggregates resembling Schiller-Duval bodies . Neoplastic cells were relatively large, with pale eosinophilic to water-clear cytoplasm including some hyaline droplets with round to oval vesicular nuclei. Periodic acid-Schiff stain showed intracytoplasmic and extracellular eosinophilic globules, which were diastase resistant. Immunohisto-chemical stains for cytokeratin and AFP were strongly positive.The patient completed five cycles of bleomycin, etoposide, and cisplatin neoadjuvant therapy. The AFP levels returned to normal by the start of the fourth course of chemotherapy. An abdomino-pelvic MRI performed at the completion of chemotherapy showed no residual tumor. The patient underwent a coccygectomy. The postoperative period was uneventful. After 3 years of follow-up, the patient remained without evidence of recurrent disease and serum AFP remained normal.According to Brown, germ cells in the developing embryo arise in the yolk sac, migrate around the hinder end of the primitive gut to the genital ridge on the posterior abdominal wall, and are finally absorbed into the developing gonads. It is suggested that during this migration, some germ cells may get left behind on the journey or may stray too far and come to rest at various sites along the dorsal wall of the embryo near the midline. The primordial germ cells give rise to an undifferentiated germ cell line. The undifferentiated germ cells undergo differentiation into embryonic (somatic cells) or extra –embryonic cells of yolk sac, chorion and allantoin cells. Patients with sacrococcygeal yolk sac tumor present most often with complaints of constipation or buttock swelling [4]. Sacrococcygeal YST develops exclusively in children less than 3 years of age .The imaging of sacral tumors in children has great values in identifying the position, contents and invasion. YST, consisting of fat-free soft tissue, is often complicated with hemorrhage, necrosis and cystic degeneration. Thus on CT and MRI the signal density of EST is heterogeneous. Honeycomb-like change is an imaging characteristic of YST [7]. Obscure boundary between tumor and surrounding tissue, sacral invasion and metastases are signs of malignancy. Each child with a sacrococcygeal germ cell tumor should be studied for alpha fetoprotein (AFP). AFP determination is useful in the diagnosis, to monitor the results of therapy and detects metastases and recurrence after therapy. The production and release of AFP is not limited to YSTs. It is commonly found, both in serum and in tissues, in embryonal carcinomas and teratocarcinomas containing only yolk sac elements and even in those without morphologically recognizable elements of this type [8]. However, AFP is also normally found in foetal serum and in that of the newborn infant [9]. Levels decrease and then reach adult levels about 8 months after birth [10].Gross examination of YSTs typically reveals a mass that is predominantly solid and is soft, white, gray, or pale yellow. Cystic degeneration as well as necrosis and hemorrhage are often present.Microscopic patterns of YST are numerous. They are described and illustrated by Teilum in his classical writings on this tumor [11]. Several different patterns are usually admixed. They are characterized by the intermingling of epithelial and mesenchymal elements in a specific organoid fashion. Micocytic, glandular-alveolar and papillary formations are common. Many of the cystic spaces are lined by flattened, endothelium-like layer of cells. The stroma can be quite cellular, spindle shaped, and reminiscent of smooth muscle. Perivascular Schiller-Duval bodies, which are almost always present in sizable samples but may be absent in limited material, as in biopsies, are the most distinctive features of yolk sac tumor. Periodic acid Schiff –positive hyaline intracytoplasmic and extracytoplasmic droplets are consistently seen in yolk sac tumors. Immunohistochemically, these tumors characteristically stain for alpha-fetoprotein. However, unlike serum levels which are rises in all cases of YST, immunohistochemical assay may be negative for AFP in some cases. Most of the immunoreactivity for AFP is not in the hotline globules but it is rather seen diffusely or in granular fashion throughout the cytoplasm of the tumor cells [8]. The treatment of malignant sacrococcygeal GCTs, such as primary yolk sac tumor is dependent on the extent of disease. Local disease is best managed surgically, while advanced tumor stages benefit best from adjuvant platinum based chemotherapy. Survival rates using these strategies are higher than 80% [12].Source of Support: NilConflict of Interest: None declared


Xanthogranulomatous osteomyelitis is a very rare form of chronic osteomyelitis which presents like a bone tumor. Three cases of xanthogranulomatous osteomyelitis have been described previously in the literature. We present a case of xanthogranulomatous osteomyelitis occurring in the distal part of tibia. This case presented as swelling in a 13-year-old boy. Simple curettage proved to be curative in the present case. Xanthogranulomatous reaction is a rare form of chronic inflammation characterized histologically by presence of high number of foamy histiocytes admixed with lymphocytes and plasma cells . Cases oA 13-year-old boy presented to Department of Orthopedics with pain and swelling in the right ankle. Swelling had appeared about 1 year back and was gradually increasing in size. Examination revealed bony thickening over the lateral malleolus. Ankle and subtalar joints had full range of mobility. Parameters of complete blood count were within normal range. ESR was 68 mm at 1st hour. Plain radiography revealed a submetaphyseal lytic lesion in the distal tibia with a sclerotic margin around it . With th On gross examination, specimen consisted of multiple, soft, gray brown to gray white tissue bits, with largest measuring 0.5 × 0.5 × 0.3 cm. Histopathology showed small irregular necrotic bony fragments and degenerated cartilage, surrounded by mixed inflammatory cell infiltration consisting of plenty of foamy histiocytes along with lymphocytes and plasma cells . Few neuXanthogranulomatous osteomyelitis is a close mimicker of neoplastic lesions of bone. In fact, xanthogranulomatous inflammation in various organs forms a mass lesion giving an appearance of tumor . HistoloPatients of previously described 3 cases were aged 5 years, 14 years, and 50 years. Two cases were seen in boys and one was seen in a postmenopausal lady. Sites involved in these cases were first rib, tibial epiphysis, and ulna [Microscopic differential diagnoses for xanthogranulomatous osteomyelitis are Langerhan's cell histiocytosis, Erdheim-Chester disease, and lipid storage disorders. Langerhan's cell histiocytosis commonly affects mid-shaft portions of long bones and often has infiltration of eosinophils. Erdheim-Chester disease is a multifocal disorder with frequent involvement of extraskeletal tissues. Histology shows presence of cholesterol clefts along with foamy histiocytes. In case of lipid storage diseases, such as Gaucher's disease and Niemann Pick disease, foam cell transformation is usually seen within bone marrow. Moreover, a clinical background often helps in differentiating storage disorders from xanthogranulomatous osteomyelitis .This case is presented because of its rarity. There are only 3 cases of xanthogranulomatous osteomyelitis reported so far in the literature. It is very important to differentiate this lesion from bone neoplasms. A microscopic examination is essential in establishing correct diagnosis.


Background. Complete androgen insensitivity syndrome is a rare syndrome in which the uterus is absent and testes rather than ovaries are present. Patients usually visit a gynecologist due to primary amenorrhea. Case. A forty-eight-year-old woman with lower abdominal pain and anamnesis of uterus agenesis was operated on due to bilateral cystic masses. A 5 × 3 × 1.2 cm left adnexal cyst revealed the presence of a serous cyst with a hypoplastic ductus deferens. A smaller cyst of the right adnexa revealed immature testis tissue with Leydig-cell hyperplasia. After karyotype and hormonal examinations, laparoscopic gonadectomy was performed. Conclusion. Attention should be paid in all cyst-removing operations in cases of uterus agenesis, due to the high incidence of malignancy. Not of less importance is the issue of informing the patient in the most appropriate way. Complete androgen insensitivity syndrome is a type of male pseudohermaphroditism in genotypically XY and phenotypically female patients, in which there is a defect that prevents normal androgen receptor function. Due to the presence of the anti-Müllerian hormone, the internal genital female organs are absent, and testes rather than ovaries are present . PatientThe incidence of neoplastic formation is 52%, half of which are malignant, and usually occurs after puberty . In ordeWe report a case of bilateral cyst removal in a 48-year-old woman, followed by gonadectomy, both performed laparoscopically.A 48-year-old woman, presented to us complaining of lower abdominal pain. At the age of 16 she had been visited by a gynecologist due to primary amenorrhea, and uterus agenesis was diagnosed by ultrasound. No further examinations were made at the time. The patient, married for 13 years, had normal female external genitalia, mature secondary sexual characteristics, Tanner stage II breast development with pale areolae, and scanty pubic and axillary hair. In the gynaecological examination, vulva and perineum appeared normal, but no cervix was visible and the proximal part of the vagina ended blindly. During bimanual examination, a 5 cm large cystic mass was palpated at the left adnexal region and a small right adnexa was palpated in the proximity of the internal inguinal canal opening. Ultrasound confirmed the absence of the uterus and revealed a 5 cm cystic mass on the left and another smaller cystic mass of 3 cm at the right adnexa. No signs of malignancy were observed.Laparoscopy was performed to remove the cysts using a 3-port technique, and they were sent to the pathologist. Particular attention was paid to remove the cysts in the endobag to prevent eventual tumor cell dispersion. The histology revealed immature testicular tissue  and Leydig-cell hyperplasia in the 5 cm large cyst, and a serous cyst with on it part of a small ductus deferens, with no apparent testicular structures, at the right adnexal specimen.β-HCG were all found within normal limits. Serum testosterone levels were 44.77 nmol/L, elevated and similar to normal male values, gonadotropins were found within normal values , LH/FSH quotient 1.2, SHBG : 80.0 nmol/L and TSHi on serum 0.527 mE/L, also within normal values. Chromosomal analysis was made on lymphocyte cultures from peripheral blood and revealed XY karyotype.After that, tumor markers and hormonal evaluation, and also cytogenetic analysis, were performed. CA-125, CEA, a-FP, and Intravenous urography excluded renal anomalies. The definitive diagnosis of androgen insensitivity syndrome was made and the patient was programmed for laparoscopic gonadectomy. The pedicles of the 2 adnexa, which were situated at the internal inguinal opening Figures , were fiThe histopathologic report revealed the presence of testicles on both sides, marked by Leydig-cell hyperplasia, and on the left side presence of small sites of ovarial stroma. Considering the age of the patient, followup was programmed and hormonal replacement therapy was given, and particular importance was attributed to the way of informing the patient. Due to the fact that she was married and evaluating the psychological effects that would provoke knowing that she is genotypically a man, we decided to inform the patient that she had some kind of gonadal dysgenesis and that the gonads would be removed for the high degeneration risks, deliberately omitting to inform her about the karyotype results.Androgen insensibility syndrome is the most common cause of male pseudohermaphroditism, responsible for 10% of the cases of primary amenorrhea . DefectsThe major concern in these patients is the development of a neoplasia, most often a gonadoblastoma or a malignant dysgerminoma. Prophylactic gonadectomy is advised in the postpubertal period.Laparoscopic gonadectomy offers the minimum psychological trauma which is of major importance in these women, for the rapid recovery and short hospital stay. It is a safe and simple procedure and is easily performed using bipolar coagulation forceps, Roeder loops and endobags, as long as the surgeon is cautious while removing the gonads or eventual cysts, to avoid tumor cell contamination, even more in patients of a certain age, as the one we present, where the malignancy incidence is much higher.Hormonal replacement therapy was recommended, due to its protective effects . In adolWe presented a rare case of bilateral cysts in a mature patient with complete androgen receptor insensitivity. Laparoscopic gonadectomy is effective and the importance of removing the cysts and later on the gonads intact is stressed.


Large cell neuroendocrine carcinoma (LCNEC) of the ovary is a rare tumor and is now included in the World Health Organization tumor classification. Its prognosis is generally very poor even when the diagnosis is made at an early stage. We report a case of pure large cell neuroendocrine tumour of ovary, appearing 9 months following laparoscopic type I hysterectomy, bilateral pelvic lymph node dissection with ovarian preservation of anatomically normal looking ovaries performed for a cervical biopsy diagnosis of cervical intraepithelial neoplasia grade III with foci of invasion. The rarity lies in the rapid onset (9 months) of a large tumor following conservation of an anatomically normal ovaries. Surgical debulking and five cycles of chemotherapy (Etoposide and Cisplatin) were administered to the woman. She is on followup with no clinical or radiological evidence of disease recurrence for 6 months. Ovarian pure large cell neuroendocrine (LCNEC) carcinoma is a rare tumour. Only 6 cases have been reported and all of them have been unilateral tumors. We report 7th case of pure LCNEC which was bilateral. They are rapid growing tumours with generally poor prognosis. Literature is replete with different modalities of adjuvant therapy. We have discussed the clinical presentation, histopathology, surgical debulking, and chemotherapy options and have also reviewed the literature. A 40-year-old perimenopausal lady presented to us with a histopathological diagnosis of cervical intraepithelial neoplasia grade III with foci of invasion. A laparoscopic type I hysterectomy and bilateral pelvic lymphadenectomy along with conservation of normal looking ovaries was performed. She was clinically and radiologically free of disease on followup for 6 months. In the ninth month she presented with history of acute distension and pain abdomen, loss of weight and appetite, fever with chills, and itching all over the body. Her general condition was good. On abdominal examination a firm, irregular mass was felt occupying the suprapubic region and extending towards left iliac fossa, and left lumbar region, measuring 20 × 28 × 22 cms with restricted mobility and minimal ascites. On per vaginal and rectal examination, vault was healthy, and a firm, irregular abdominopelvic mass, measuring 25 × 28 × 22 cms, splaying the rectovaginal septum, with restricted mobility, rectal mucosa was free but thinned out. Bilateral parametria were supple. Both the pelvic side walls were free of tumor. Fine needle aspiration cytology of the mass revealed poorly differentiated malignant neoplasm. Complete haemogram, biochemistry, and chest X-ray were within normal limits. Ca-125 value was 280.80 IU/ml(high) and CEA was 7.66 ng/mL(high). Ultrasonography of abdomen and pelvis showed normal liver, gall bladder, and spleen except for left kidney with evidence of grade I hydroureteronephrosis secondary to compression by the mass. Uterus was not seen-post operative status. A large lobulated heterogenous mass lesion with few areas of necrosis in it, situated posterior to and indenting the base of the urinary bladder, measuring 9.5 × 12 × 20 cms extending laterally up to the left iliac fossa with minimal ascites was reported.During surgery minimal haemorrhagic ascites was seen in the peritoneal cavity. Bilateral solid ovarian tumours with breech and deposits on the capsule were seen. The right ovarian tumour measuring 6 × 7 × 6 cms, adherent to the vaginal vault and pouch of douglas, and left ovarian tumour measuring 20 × 15 × 14 cms., solid and burrowing into the pouch of douglas adherent to rectum, vault, bladder base, and left lateral pelvic wall was released from the vital structures by sharp and fine dissection after tracing the ureters bilaterally, through a retroperitoneal approach Right ovarian tumour measuring 11 × 8 × 5 cms, capsule intact, surface shows nodularity, cut section shows solid, cystic, and firm. Left ovarian mass 11 × 9 × 7 cms, external surface was irregular and nodular, capsular breach was noted. Cut section shows haemorrhagic, solid and cystic areas. Omentum measured 28 × 6 × 3 cms  Bilateral ovaries show poorly differentiated malignant tumour-carcinoma, with wide areas of necrosis. Mitotic rate 15–20/high power field, capsular breech noted in the left ovary. Omentum showed tumour deposits, and 3 paraaortic nodes were reactive. Bowel and bladder deposits showed tumour cells, CK, EMA were positive. CK 7, CK 20, Inhibin and Chromogranin were negative. Focal areas were positive for Synaptophysin, suggesting a final diagnosis of Large Cell neuroendocrine Carcinoma Ovary Postoperative adjuvant chemotherapy consisting of Etoposide 100 mg/M2 from day 1 to day 5,and Cisplatin 100 mg/M2 in divided doses on day 1 and day 2 was administered 3rd weekly for 5 cycles. Patient tolerated chemotherapy, except for in-patient admission for neutropenic fever on day 7 during the 3rd and the 4th cycle. She was given symptomatic and supportive treatment. She is on followup for 6 months and has no clinical or ultrasonographic evidence of disease recurrence. The incidence of pure large cell neuroendocrine carcinoma (LCNEC) of ovary is very rare. Primary ovarian LCNEC is synonymous with undifferentiated carcinoma of non-small cell neuroendocrine type, according to the World Health Organization classification .To date, only 40 cases of large cell neuroendocrine carcinoma (LCNEC) of the ovary are reported. 34 cases were associated with other histologic subtypes and 6 cases were purely LCNEC. They are generally associated with poor patient outcomes. The present case is the seventh case of pure form of ovarian large cell neuroendocrine carcinoma and the other rarity is, it is the first case arising bilaterally. This extremely rare clinical presentation of a huge mass arising from an anatomically normal ovary confirmed laparoscopically, 9 months back was reiterating the fact that this tumour was rapidly growing. Some authors have similar opinion –6. The age of patients was ranging between 27–81 years and a median age of 61 years. The commonest presentation was abdominal pain and distension in 50% (3/6) of cases, similarly the present case presented with abdominal distension. The size of the tumour ranged from 9 cms to 35 cms with an average of 16 cms. The tumor was bilateral in only the present case. Left side was more common in 50% of the cases (3/6). A range of therapeutic options have been used like surgery with chemotherapy,  As reported by Kim et al. , the pur2 for 5 days along with cisplatin 100 mg/M2 divided doses on day 1 and day 2 every third weekly was administered. Survival data of the available patients: 5-year survival of the 33 LCNEC cases was 34.9% as discussed by Dundr et al. [Various combinations of chemotherapy were used, including cisplatin and cyclophosphamide followed by etoposide and cisplatin; or paclitaxel and carboplatin protocols Three of the six patients have died of the disease immediately following surgery to 4 months after surgery. Present patient is on followup for last 6 months.Out of the 6 cases of pure LCNEC, 2 cases had early stage disease and survived for a minimum of 10 months. In 3 cases there is no stage of disease mentioned but have had short survival of 4 months. One case had stage 4 disease and died after 2 months. Present case is a stage III disease who has survived for 6 months without evidence of recurrence, probably the longest Due to paucity of literature and heterogeneity in presentation and response to surgery, chemotherapy, and radiation it is oblivious to predict the efficacy of surgical debulking, chemotherapy and radiation protocols, hence there is a need for prospective clinical studies for most optimal modalities of treatment. May be a combination of optimal debulking, adjuvant chemotherapy  with close followup of patients and use of radiation when appropriate may improve the progression free and survival rates of this rare tumour.


Pulmonary metastasis from leiomyosarcoma is rare and its clinical management is challenging. A single lung metastasis from a perineal leiomyosarcoma occurred in a 79-year-old woman. Five months after resection of the lung metastasis, a new metastatic tumor developed in the contralateral lung. Since the patient did not desire to receive hospitalized treatment, TS-1  therapy was started on an outpatient basis. The lung metastasis has been successfully controlled for at least 17 months with excellent tolerability. The clinical features and the treatment of this case are discussed. Leiomyosarcoma, a relatively rare malignant tumor of smooth muscle origin, can arise from the wall of the gastrointestinal tract, wall of the uterus, soft tissues  that are rich in a smooth muscle, or wall of the great vessels . In geneLeiomyosarcomas are considered to metastasize more commonly via the hematogenous route . The timA 79-year-old woman visited our hospital because of an abnormal shadow on chest X-ray performed during a routine medical checkup in October 2009. She had no particular symptom. She had a medical history of uterine myoma in 1975, which was surgically removed by hysterectomy. She also had a medical history of perineal leiomyosarcoma in 2001. The tumor size was 3 × 2 cm and there were no lymph node metastases or remote metastasis. The disease stage was stage I according to Unio Internationalis Contra Cancrum (UICC). The tumor was totally removed and she had no chemotherapy. She had no family history of significant diseases.On physical examination, her temperature was 35.8°C, blood pressure 119/78 mmHg, and pulse 68 beats per minute. The oxygen saturation was 97% with ambient air. Physical examination and laboratory tests  showed nAlthough she had been in her normal state of health after surgery, another tumor was detected in the opposite lung in July 2010. Simple CT of the chest revealed a nodule with an irregular but well-defined margin in S10 of the left lung , and theAccording to our literature search of the past decade in Japan, the lung was the first metastasis site in 53 cases of leiomyosarcoma. The ratio of females to males was approximately 3 to 1. The median age in the 53 cases was 58 years old . The primary lesion was in the uterus in 31 cases, in the urinary bladder in three cases, in the thigh in two cases, and in the inferior vena cava in two cases. Perineal leiomyosarcoma has not been reported as the primary lesion. The mean interval from diagnosis of the primary tumor to detection of the pulmonary metastasis was 31 months (0 day to 12 years); in 11 cases, the metastasis was detected over 5 years after diagnosis of the primary tumor. Some cases were diagnosed as leiomyosarcoma with metastatic lesions due to the rapidly developing metastasis. The mean survival after diagnosis of the lung metastasis was 18 months . The present case's interval to the diagnosis of lung metastasis was relatively long, that is, 8 years from resection of the primary tumor. Because of the varied prognosis of leiomyosarcoma, patients should be closely monitored for a long period even if the primary lesion was surgically removed.According to a previous report on the growth rate of metastasis from leiomyosarcomas of the stomach, the doubling time was 20 months or less . UterineTS-1 is a fluoropyrimidine derivative in which tegafur is combined with two 5-FU-modulating substances, 5-chloro-2,4-dihydroxypyridine (gimeracil), and oteracil potassium, at a molecular ratio of 1 : 0.4 : 1. TS-1 was invented by Shirasaka et al. in 1991, and is the first self-rescuing concept-based anticancer agent . AlthougA case with a lung metastasis from a perineal leiomyosarcoma was reported. A leiomyosarcoma can reoccur over 5 years from the initial diagnosis; therefore, patients should be monitored for a long period of time. Radical treatment in elder patients with recurrent leiomyosarcoma may result in diminished QOL. The present case may indicate the possibility of TS-1 for inoperable leiomyosarcomas, which permits treatment on an outpatient basis.


Leiomyomas are the second most common tumors of epididymis. Patients with leiomyomas are sometimes misdiagnosed with testicular tumors. A Case of a patient with a scrotal mass presenting with testicular torsion is reported. Concurrent occurrence of testicular torsion and epididymal leiomyoma is an extremely rare condition. Leiomyomas may arise from any tissue that contains smooth muscle. In genitourinary system, their most common locations are mentioned to be uterus and renal capsule . EpididyA 42 year-old man presented to emergency department because of sudden left scrotal pain and a scrotal mass. He stated that he had had the mass for one year but the pain had started 4 hours ago and gradually increased. He defined the pain as sharp and continuous in nature which radiated to left inguinal region. Past medical history of the patient was unremarkable. Physical examination revealed a tender, erythematous right scrotum with a nontransilluminating mass of 4 cm which was contiguous to testis. Routine laboratory tests, including total blood count, blood biochemistry, and urinalysis showed no pathologic results. Testicular tumor markers  were also evaluated owing to the palpated testicular mass. However, the results were within normal limits. Scrotal doppler ultrasonography assessment indicated findings consistent with a solid scrotal mass and concurrent testis torsion but the origin of the mass, whether testis or epididymis, could not be delineated. The patient was informed and prepared for an operation with the prediagnosis of testicular torsion and testis tumor. An inguinal approach was preferred for observation of testis and paratesticular structures. On gross examination during operation the mass was observed to be fixed to both testis and epididymis and radical orchiectomy was performed. Gross pathological examination revealed a white mass in which a whorled pattern could be easily observed on the cut surface . MicroscNot many cases exist in the literature reporting the testicular masses presenting with testicular torsion. And most of the cases reported are the malignant tumors of the testis –7.To our knowledge no cases of testicular torsion with epididymal leiomyoma has been reported. Tumors of epididymis are rare cases. Following adenomatoid tumors in incidence, leiomyomas have been reported to be the second most common tumor of epididymis . Its incMacroscopically leiomyomas are tumors which are encircled with a gray-white capsule. A whorled pattern is usually observed on the cut surfaces. Microscopically, interlacing smooth muscle fibers on a fibrous and sometimes hyalinized stroma are characteristic features of the tumor . LeiomyoPhysical exam and sonographic evaluation can usually define paratesticular lesions. However it is not dependable enough in distinguishing malign paratesticular lesions from benign ones . Most of the lesions can be dissected easily from the testis but in our case the lesion could not be dissected from the testis and the origin of the mass could not be defined precisely. Because the malignancy risk could not be eliminated, radical orchiectomy was performed. If epididymal origin is considered during surgery, frozen section can be used to confirm the benign nature of the lesion and testis-sparing surgery can be performed for the preservation of the fertility.Tendency to testis torsion due to paratesticular mass has not been established. A thorough evaluation in every patient admitted for any scrotal disease should be performed.


Neuroblastoma is the most frequent extracranial solid tumor in childhood, but it is seldom diagnosed prenatally. It usually presented with adrenal masses. Presentation of other localization is extremely rare. We report a case of cervical neuroblastoma identified at 20 weeks of gestational age. This is the third case diagnosed antenatally on neck region in the literature. Additionally, it is the first case that extended to the brain. We also discussed the literature for cervical neuroblastoma detected prenatally. Fetal cervical neuroblastoma is an extremely rare condition that should be considered in the presentation of fetal solid neck masses , 2. DiffA 32-year-old woman  was referred for evaluation of a fetal neck mass, which had been identified on routine sonography at 20 weeks' gestation. Family history revealed that the father was 35 years old and no consanguinity. The detailed ultrasonograhic evaluation revealed a fetus with a 20 weeks appearance and weighed 372 gr. Left lateral neck mass was observed with sized 49 × 38 mm that starts from preauricular region to left mandibular site. The mass was hyperechoic, mainly solid, and contained few cystic components. There were destructive lesions on left mandibula, maxilla, and sphenoid bone. It was extended to the brain. Figures .An abortion was performed at 20 weeks and 5 days of gestation with parents consent.The macroscopic appearance of fetus is showed on Aborted fetus was examined with X-ray. It showed cervical mass in Computerized tomography of the head and neck region revealed no extension of the mass into thorax but extension into the brain shown in Histopathological examination of the tumor was consistent with neuroblastoma which was shown in The mother had none of the following conditions during the course of her pregnancy: maternal exposure to hydantoin, phenobarbital, alcohol, teratogenic agents or radiation, familial neuroblastoma, and maternal diabetes mellitus. There was no family history of malformations. Blood pressure of mother was within normal limits during pregnancy; she did not experience any infection or disease during pregnancy. She did not take any medication and her tests for fetal anomalies were also within normal limits.The incidence of neonatal neuroblastoma has been reported to be 0.61 per 100.000 births . The majPrenatal diagnosis of neuroblastoma by ultrasound was first reported in 1983 . Ultrason = 112). Only two neuroblastomas were diagnosed prenatally on neck region in this report. The most common initial finding was a mass detected either by ante- or postnatal sonography or by physical examination during the neonatal period with signs and symptoms referable to the location of origin [Fetal cervical neuroblastoma is an extremely rare condition that should be considered in the presentation of fetal solid neck masses. In a recent review, a total of 271 fetuses and neonates presented with neuroblastoma and 41.3% of them were diagnosed prenatally , or fetal goiter. Masses in the anterior neck may cause hyperextension of the neck when large and are frequently associated with polyhydramnios . Our casCesarean section can be utilized if there appears to be an increased risk for dystocia or tumor rupture.Cervical neuroblastomas should be listed in the differential diagnosis of fetal neck mass that was determined by ultrasonography.


Acute gastric dilatation is a rare entity, with varying aetiologies the majority of which are benign. Delay in diagnosis and treatment could result in sequelae such as gastric emphysema (pneumatosis), emphysematous gastritis, gangrene, and perforation. Gastric emphysema as a result of a benign nongangrenous condition such as gastroparesis, adynamic ileus can be successfully managed conservatively. Here, we present an interesting case of acute gastric dilatation resulting in gastric emphysema following massive postpartum hemorrhage. Acute gastric dilatation is a rare entity, with varying aetiologies, the majority of which are benign. Prompt recognition and appropriate management are essential to prevent sequelae such as gastric emphysema (pneumatosis), emphysematous gastritis, gangrene, and perforation –4. Like A 40-year-old African gravid 3/para 3 woman was admitted to the intensive care unit (ICU) following a caesarian section for placental abruption and fetal distress. She was booked for antenatal care at approximately 34 weeks of pregnancy with antenatal care prior to that done in Africa for this pregnancy. Her initial assessment was satisfactory Tables  and 2, bShe underwent emergency C-section after she presented with antepartum haemorrhage, and intraoperative findings were a low-lying placenta with large retroplacental clot with abruption. Postoperatively, she continued to bleed per vagina in ICU and received 7 units of red cell concentrate (RCC), 4 units of plasma, 4 pools of platelets, and an oxytocin infusion. Despite treatment, she continued to bleed and was transferred back to theatre, where a uterine tamponade balloon was successfully inserted. Her total blood loss was estimated in excess of 3 litres.On the same evening, she began to complain of abdominal pain. On examination, her vital signs were within the normal range. Abdominal examination revealed a distended abdomen with tenderness in the epigastrium and left upper quadrant and was tympanic to percussion over the epigastric region. There was no guarding or rigidity noted. An abdominal X-ray showed significant gastric distension with diffusely noted gas pattern consistent with gastric emphysema . CT confHer further clinical course was uneventful, and she responded very well to conservative management. A repeat CT, one week later, showed absence of gastric wall gas or free air . The larAcute gastric dilation was first described by Powell et al. in 2003 , and it streptococci, Escherichia coli, Pseudomonas aeruginosa, Clostridium perfringens, and Staphylococcus aureus [Acute gastric dilatation can result in gastric emphysema, emphysematous gastritis, and ischemic necrosis. Ischemic necrosis, in the case of gastric dilatation, is postulated to be due to venous insufficiency , 5, 6. Ps aureus .Gastric emphysema secondary to mechanical aetiologies is far more common than primary gastric emphysema. Causes include obstruction, trauma, and rupture of pulmonary bullae, enteric tube placement, and upper endoscopic procedures –8. In thClinically, patients with infectious gastric emphysema appear septic and complain of fever, chills, and abdominal pain , 9–11. TEarly endoscopy in the stable patient may be of use, particularly if the CT scan suggests gastric or esophageal involvement. Where diagnosis is emphysematous gastritis, gastroscopy and biopsy are advocated to facilitate a diagnosis, which can show evidence of submucosal abscesses or exudative gastritis , 15. In Treatment focuses on early diagnosis, broad-spectrum antibiotic cover, nasogastric decompression of the stomach, and total parenteral nutrition, thus halting the vascular congestion and ensuing ischemia –5. When In our case, it is reasonable to presume that multiple factors have played a role in the development of acute gastric dilatation and gastric emphysema. This patient's co-morbidities, gestational diabetes with poor diabetic control leading to gastroparesis, and chronic constipation are likely to have been a factor in the development of acute gastric dilatation and subsequently gastric emphysema. It is also likely that other factors can potentially aggravate the condition in this patient such as increase in intra-abdominal pressure because of gastric dilatation and acute massive haemorrhage, leading to transient poor perfusion of the gastrointestinal tract. Also the use of opiates for pain relief can further potentiate depression of gut motility. From the above case report and discussion, it is clear that the diagnosis and treatment of underlying cause of gastric emphysema are most important for a favourable outcome then simple gastric decompression.


Desmoid tumors are benign neoplasms that most often arise from muscle aponeurosis and have been associated with both trauma and pregnancy. The etiology of desmoids has not been determined. We report the case of anterior abdominal wall desmoid tumor in a female patient with previous history of cesarean section. Preoperative ultrasound and computed tomography demonstrated a large mass mimicking a large hematoma or an intraabdominal mass. The tumor was removed by wide excision with safe margins. The abdominal wall defect was reconstructed with polypropylene mesh. Subsequent histology revealed a desmoid tumor. Desmoid tumors in females are often associated with pregnancy or occur post-partum. The reasons behind this association are unclear. The most common sites are in the abdominal muscles. Desmoid tumors are rare neoplasms, accounting for 0.3% of all neoplasms and less than 3% of all soft tissue tumors with an estimated incidence in the general population of 2-4 per million of population per year –3. They A 28 years old woman consulted for a firm mass in the left upper abdomen. The interview found no history of family or personal neoplasms, familial adenomatous polyposis (FAP), colorectal disease, no concept of hormonal contraception, alcoholism or smoking. The patient was secondparous delivering by cesarean section in her first pregnancy five years earlier, through a Pfannestiel incision and by vaginal delivery in her second pregnancy 16 months ago.The patient noticed a small tumor in the anterior abdominal wall for the first time 6 months post partum. This tumor, initially neglected by the patient, was gradually increasing in size and associated with a sustained pressure in her cesarean scar inciting the woman for consulting 10 months after its discovery.On clinical examination, the general condition was maintained. We found a huge anterior abdominal wall mass measuring 20 cm × 25 cm, firm, painless, fixed to the superficial and deep levels. Blood parameters analyses were within normal range.Ultrasonography showed an oval mass of 21 centimeters with hypo and heterogeneous echogenicity and well defined margins in the left anterolateral abdominal wall. Preoperative computed tomography scan images revealed a well-circumscribed, large mass (23 × 18 × 13 cm) with heterogeneous density. The mass was close to the left rectus abdominal muscle and after intravenous administration of contrast medium it demonstrated no enhancement even in the delayed images . After tThe decision was made for laparotomy, which was performed via a midline incision. A large mass attached firmly to the inner aspect of the abdominal wall was found intraoperatively with no infiltration of abdominal cavity organs. Surgeons took over and the mass was removed by wide excision with a safe margins. The defect was reconstructed with polypropylene mesh. Subsequent histopathology revealed features of a desmoid tumor .The postoperative course was uneventful and the patient was discharged at the 9th postoperative day. Coloscopy, performed 3 months later, was normal.Desmoid tumors can occur sporadically or as part of an inherited syndrome, familial adenomatous polyposis . There iFurthermore authors reports that some desmoids, both in pregnant and non-pregnant patients, respond to treatment with the anti-estrogen tamoxifen . What isThe etiologic diagnosis for an abdominal mass is varied especially in females. In fact, these masses may arise from the reproductive organs, gastrointestinal system, abdominal wall, urinary system, adjacent soft tissues, and metastases. Previous case reports showed that desmoids tumors may mimic other intra abdominal tumors . This caIn many times, the diagnosis is suspected intraoperatively, showing a tumor growing from the abdominal wall. Most often they are confined locally, but may show infiltration of adjacent structures. Histology is the only evidentiary method which demonstrates long fascicles of spindle cells of variable cell-density with few mitoses and absence of atypical nucleus-separations. Characteristically, there is a diffuse cell infiltration of adjacent tissue structures. Immunohistochemical response for actin can be partially positive and immunohistochemical muscle cell markers delimit desmoid tumors from fibro sarcomas . SurgeryDesmoid tumors in females are often associated with pregnancy or occur post-partum. The reasons behind this association are unclear. The most common sites are in the abdominal muscles, but they have been reported in multiple other areas of the body. Surgical resection is the treatment of choice and is usually curative even when surgical margins are involved.


Cardiac metastasis from known cervical cancer is rare. Even through a routine check-up, this type of metastasis can present as pulmonary emboli. Suspicion of this diagnosis in an oncology patient with complicating pulmonary emboli but no evidence of deep vein thrombosis is important, especially in cervical cancer patients with extensive pelvic lymph node metastasis and vascular invasion of a primary tumor. Early recognition may aid in improving the prognosis. We present a case of intracardiac metastasis arising from a squamous carcinoma of the cervix in a patient with pulmonary tumor emboli and review other cases from the literature. Metastatic disease of the heart is rare. The incidence quoted in the current literature, based on autopsy, is approximately 1.23% [Classically, there are four pathways of cardiac involvement: (1) retrograde lymphatic spread, (2) direct extension from the adjacent viscera, (3) hematogenous spread, and (4) transvenous extension through the vena cava into the right side chambers.Myocardial involvement is much less common. It is usually the result of hematogenous spread, and is associated with widespread disseminated disease. Intracavitary, endocardial, or valvular metastatic deposits, such as the one described in our case, occur in less than 6% of cases . Of partWe report a rare case of human papillomavirus (HPV)-detected metastatic intracardiac mass from known cervical cancer. The clinical, radiological and histological features are described. Simultaneously, the literature for all reports of these rare intracardiac metastases is reviewed.A 32-year-old woman diagnosed in 2009 with International Federation of Gynecology and Obstetrics (FIGO) stage IIA2 cervical cancer(squamous cell carcinoma), presented to our hospital emergency room with exertional dyspnea lasting four days. The patient had previously received multimodality treatment from a multidisciplinary oncology group, which, because the patient was young and wanted to preserve ovarian function, included the following treatment: radical hysterectomy along with ovarian transposition and pelvic and para-aortic lymph node dissection and concurrent chemoradiation with six cycles of weekly cisplatin, including tomotherapy in pelvic and para-aortic lymph node area.Thirteen months after completion of adjuvant treatment, 18-fludeoxyglucose positron emission tomography-computed tomography (18-FDG PET-CT) revealed no evidence of recurrence. However, just two months later, she was examined on admission with chest CT  angiography. The examinations revealed cardiac masses occupying the right ventricle and right atrium and no evidence of deep vein thrombus Figure B.In view of the threatened outflow obstruction caused by the right ventricular mass, the patient underwent an open excision of the intracardiac mass Figure A. At theThe histopathology of the surgical specimen revealed this to be a cardiac metastasis from known cervical squamous cell carcinoma Figure A. AdditiAfter the patient’s condition was stable, she was transferred to a medical oncologist for recurrence management. She underwent carboplatin with paclitaxel chemotherapy for palliative treatment at the medical oncology department. On a follow-up chest CT and echocardiogram after three cycles of the chemotherapy, cardiac mass and pulmonary thromboemboli were no longer seen. The patient was supposed to undergo six more cycles of the chemotherapy. She tolerated the chemotherapy well, but during the last month of her life, the recurrent cancer mass surrounding the celiac axis caused extensive hepatobiliary obstruction. The cancer mass in her heart was still regressed. She died of cachexia one year and four weeks from diagnosis of cardiac metastasis.The clinical presentation may include nonspecific symptoms, such as chest pain, weight loss, malaise, or more characteristic symptoms, such as congestive cardiac failure secondary to intracardiac obstructions, valvular involvement, or pericardial effusions, arrhythmias due to involvement of the conduction system, or, as in our case, embolic events. Thus, metastases should be suspected in oncology patients if they develop inexplicable heart failure, neurological deficits, or recurrent pulmonary emboli, particularly when no peripheral source for the emboli can be identified.Vascular invasion of primary cervical cancer is associated with hematogenous spread of cervical cancer Figure B.In our study, pathologic findings of primary cervical cancer showed vein and lymphatic invasion of squamous cell carcinoma with moderate differentiation. The authors suggest that the carcinoma of the cervix metastasized along the inferior vena cava to the heart, where it settled as an intracardiac obstructive mass. Pulmonary tumor emboli might decrease the right cardiac blood flow, resulting in a more hospitable environment for tumor cell adherence to the endocardial wall .et al. suggested HPV status in the sentinel lymph node might be a prognostic factor in cervical cancer [et al. suggested that tumor-free, HPV DNA-positive metastatic sites should be monitored as being at high risk of relapse [We searched PubMed between 1997 and 2011 and found nine articles, including the current case, which presented a clinical course and treatment of cardiac metastasis from known cervical cancer (Table l cancer . Yutaka  relapse .The role of HPV infection with a distant metastatic lesion is controversial. In a recent study, molecular analysis for HPV detection was performed in both uterine cervical cancer and right atrial cancer tissues. Both squamous cell carcinomas showed positivity for HPV, especially type 16. Based on this result, the tumor in the right atrium favors a metastatic tumor from the uterine cervix.To our knowledge and based on our literature search, an HPV-detected intracardiac mass has never been reported to be associated with recurrence of known cervical cancer.Cardiac metastasis from cervical cancer is very rare and difficult to diagnosis. It causes acute severe heart failure,a life-threatening condition for which there is currently no standard treatment. After our review of the clinical courses of cardiac metastasis from cervical cancer, we suggest that patients with a high risk of recurrence must be carefully evaluated using echocardiography for cardiac metastasis, even if there are no symptoms.Further studies may reveal the risk and aggravating factors for cardiac metastasis from cervical cancer, and may provide additional evidence to support our suggestion.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SWB contributed mainly to the design, literature review and the writing of the work. JSP, the corresponding author who provided the case, planned and approved the written work. STP and EYK helped correct the manuscript. HS performed the cardiac surgery and described the operative finding. SHH performed the palliative chemotherapy on the patient and edited the discussion. All authors read and approved the final manuscript.


Rhabdomyosarcoma is the most frequent malignant intraorbital tumour in paediatric patients. Differentiation of tumour recurrence or metastases from post-therapeutic signal alteration can be challenging, using standard MR imaging techniques. Diffusion-weighted MRI (DWI) is increasingly considered a helpful supplementary imaging tool for differentiation of orbital masses.We report on a 15-year-old female adolescent of Caucasian ethnicity who developed isolated bilateral thickening of extraocular eye muscles about two years after successful multimodal treatment of orbital alveolar rhabdomyosarcoma. Intramuscular restricted diffusion was the first diagnostic indicator suggestive of metastatic disease to the eye muscles. DWI subsequently showed signal changes consistent with tumour progression, complete remission under chemoradiotherapy and tumour recurrence.Restricted diffusivity is a strong early indicator of malignancy in orbital tumours. DWI can be the key to correct diagnosis in unusual tumour manifestations and can provide additional diagnostic information beyond standard MRI and PET/CT. Diffusion-weighted MRI is useful for monitoring therapy response and for detecting tumour recurrence. Rhabdomyosarcoma is the most frequent malignant intraorbital soft tissue tumour in paediatric patients . PrimaryIn April 2010, an otherwise healthy 15-year-old girl first presented with acute left-sided protrusio bulbi. MRI revealed an extraconal mass in the left superonasal orbital quadrant with infiltration of the paranasal sinuses and the skull base . Restricted diffusion was indicated by progressively high intralesional signal on DWI at b = 1000 s/mm2 and a corresponding drop in ADC values  from 1.25 (long-standing baseline) to 0.6 × 10−3 mm2/s in the left medial rectus muscle within three weeks. A similar time course of ADC in correlation to progressive muscular thickening was observed in all affected eye muscles  (Figure −3 mm2/s.In early 2012, three months post-therapy, the patient complained about newly occurred double vision. In comparison to numerous preceding MRI studies, a new, though initially very discrete, thickening of the left medial rectus and inferior rectus muscle was observed in the absence of other intraorbital signal alterations (Figure Rhabdomyosarcoma metastases confined to the extraocular eye muscles constitute a rare clinical entity . In our Some limitations of our study deserve consideration. Firstly, MR imaging at the time of primary diagnosis did not include DWI, so information on diffusivity in the primary tumour is not available. Secondly, some rhabdomyosarcoma may exhibit higher ADC ,6,7. WhiIn summary, diffusion-weighted MRI is increasingly regarded as a helpful tool in the diagnostic work-up of orbital tumours. Promising current data from the head-neck-region show a diagnostic benefit with improved differentiation of malignant and benign neoplasm ,8. In a Written informed consent was obtained from the legal guardians of the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.ADC: Apparent diffusion coefficient; DWI: Diffusion-weighted magnetic resonance imaging; FDG: Fluordeoxyglucose; MRI: Magnetic resonance imaging; PET/CT: Positron emission tomography/computed tomography; SUV: Standardized uptake value.All authors declare that they have no competing interests.In detail: MB and HN conceived and outlined the case presentation. NH, MWM, MB and HN collected and analysed the MRI data. MCK collected and analysed the PET/CT data. BW was responsible for compiling clinical background information. HN and MCK performed image analysis and prepared imaging data and figures. KE contributed histopathological data and microscopy images. NH and HN drafted the manuscript. All authors revised the draft and agreed on the final version.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2431/14/57/prepub


Fetal inguinal scrotal hernia is a rare condition resulting in an abnormal embryonic process of the tunica vaginalis. We report a case of ultrasound prenatal diagnosis of inguinal scrotal hernia associated with contralateral hydrocele in a woman at 37 weeks of gestation, referred to our clinic for a scrotal mass. Differential diagnosis includes hydrocele, teratoma, hemangiomas, solid tumours of testis, bowel herniation, and testicular torsion. Bowel peristalsis is an important ultrasound sign and it allowed us to make diagnosis of inguinal scrotal hernia. Diagnosis was confirmed at birth and a laparoscopic hernia repair was performed without complications on day 10. During surgery, a bilateral defect of canal inguinal was seen and considered as the cause of scrotal inguinal hernia and contralateral hydrocele observed in utero. Inguinal hernia is found in 1–4% of neonates and children. It is more common in male and is usually an isolated finding.Pediatric inguinal hernias and hydroceles are due to incomplete or abnormal obliteration of the processus vaginalis peritonei –3. This If the canal does not close, at birth, the increasing intra-abdominal pressure can force bowel loops into the scrotum determining inguinal hernia, which is a common pediatric disease , 2.Prenatal inguinal hernia is a rare condition as intra-abdominal pressure in the fetus is similar to the pressure in the amniotic cavity. Therefore very few cases are described in the literature.In this paper we report a case of prenatal inguinal scrotal hernia, associated with contralateral hydrocele, diagnosed by ultrasound examination at 37 weeks of gestation.A 30-year old woman, primigravida, was referred to the Fetal Medicine Clinic of our department at 37 weeks of gestation for evaluation of a fetal scrotal mass visualized on ultrasound. Previous obstetric ultrasound examinations at 12, 21, and 30 weeks had not shown any evident fetal abnormalities. Fetal karyotyping had not been performed.The ultrasound examination was performed using a 4.0–8.0 MHz multifrequency 3D transducer . Fetal biometry was within the normal ranges for the gestational age with normal biparietal diameter and head circumference and femur length and abdomen circumference. The amount of amniotic fluid was in the normal range.The study of the scrotum revealed an echogenic mass (50 × 46 mm) with mixed echostructure and regular walls, containing few small echo-free cystic areas in the right side  and hydrA pediatric surgeon counselling was requested after our examination. The consultant advised for clinical evaluation at birth, and emergency surgery in case of complications.The following ultrasound examination, performed a week later, found no modification in size of the mass and an increasing number of echo-free cystic areas, without signs of complications . In addiA caesarean section was performed with no complications at 38w + 6d, for podalic presentation.The neonate weight was 3700 g with an Apgar score of 8 and 9 at 1 and 5 minutes, respectively. The pediatric examination confirmed the presence of right inguinal scrotal hernia .The neonate was dismissed on the fourth day after birth and was admitted in the Pediatric Surgery Department of our hospital, where, on day 10, a laparoscopic hernia repair was performed without complications. During surgery, a similar contralateral defect of the inguinal canal was observed and repaired. This was the cause of the left hydrocele, seen in utero .No bowel symptoms on follow-up examination at 3 months were found.The prenatal diagnosis of inguinal scrotal hernia is a rare occurrence, and very few cases are described in the literature –14.Differential diagnosis of scrotal mass includes hydrocele, teratoma, hemangiomas, solid tumours of testis, bowel herniation, and testicular torsion.hydrocele; it is characterized by a fluid-filled space within the scrotum next to the testes; sonographic features are given by a typical “half moon” fluid image surrounding the testis [The most common cause of scrotal mass is e testis , 7.teratoma testis; it appears as a mixed echostructure mass, usually larger and highly vascularised on color Doppler examination (evidence of vascularity and flow) [Less common is nd flow) –17.Testicular torsion in a male fetus is characterized by an enlarged testis and epididymis and accumulation of hemorrhagic fluid between the visceral and parietal layers of the tunica vaginalis and outside the tunica vaginalis, resulting in “double-ring hemorrhage” image; in addition it is associated with the absence of testicular flow on color Doppler examination [mination , 19.fetal inguinal scrotal hernia include the following: maternal gestational age not less than 26 weeks; scrotal mass with smooth contour and predominant solid content with internal echo-free cystic components, consistent with bowel loops; intestinal peristalsis within the scrotum at real-time sonography, with movement and deformation of the echo-free cystic components [Clinical and sonographic characteristics of mponents –11. Furtmponents .In our case, at first examination, we found a smooth contour mass with small echo-free cystic components and no bowel peristalses; in the contralateral scrotal region we observed hydrocele. At this time, other possible diagnoses were considered and an ultrasound control was prescribed after one week when we found a more clear visualization of the echo-free/cystic areas and the presence of bowel peristalsis, allowing us to diagnose inguinal scrotal hernia .In conclusion, when a scrotal mass is identified in utero, it is important to evaluate all ultrasonographic signs of the lesion to discriminate inguinal scrotal hernia from teratoma, hydrocele, or testicular torsion so as to have a correct prognosis and avoid pre- and postnatal complication.