Primary pulmonary synovial sarcoma is an extremely rare tumor with only few case reports in the literature.A healthy 67-year-old woman was admitted for investigation of a pulmonary mass found on a routine X-ray. She had a history of breast cancer diagnosed and treated 13 years previously with left mastectomy followed by adjuvant endocrine therapy. No progression of the disease was reported. Thoracic computer tomography disclosed a soft-tissue mass in the lower lobe of the left lung arising in the vicinity of the pleura. No abnormal lymph nodes were noted. Further work-up for metastases was negative. Subsequently, the lower lobe of the left lung was removed and the diagnosis was a monophasic synovial sarcoma.The diagnosis of monophasic primary pulmonary synovial sarcoma requires clinical, imaging and immunohistochemical investigation to exclude alternative primary sources. The treatment of choice is excision (lobectomy or pneumonectomy), which in most of cases is helpful for diagnosis. The prognosis is usually poor. Primary synovial sarcoma of the lung is an extremely rare tumor ,2 and seWe describe a case of an asymptomatic, 67-year-old, non smoking woman with a primary monophasic synovial sarcoma presenting as a left lower lobe pulmonary mass.A 67-year-old woman was referred for investigation of a peripheral opacity in the left lung lower lobe, which was discovered incidentally on a chest radiograph. Thirteen years earlier she had undergone a radical left-sided mastectomy for breast cancer, followed by a three-year course of adjuvant endocrine therapy with tamoxifen. No adjuvant radiation therapy or chemotherapy was given after the mastectomy. Until the time of presentation, no evidence of disease recurrence had been found.The patient was a housewife, did not smoke or abuse alcohol, had no known toxic exposures and denied any family history of malignancies. She was in good general health and well nourished. Physical examination was negative apart from a post-surgical chest scar. The results of blood tests, standard biochemical tests, urine analysis, c-ANCA, p-ANCA, tumor markers and arterial blood gas analysis were normal.The chest radiograph Figure  revealedFinally, a left thoracotomy was performed, revealing a tumor in the outer part of the left lower lobe, which did not infiltrate the visceral pleura. Subsequently, a left lower lobectomy with typical excision of the satellite lymph nodes was completed. Pathological examination revealed a well-circumscribed, whitish, soft tumor measuring 2.5 × 3 × 2 cm. Microscopic examination showed a spindle cell neoplasm, with slit-like spaces and normal adjacent lung parenchyma Figure . The lymSynovial sarcoma (SS) is a rare and well-established mesenchymal tumor, accounting for approximately 10% of all soft tissue tumors ,7. SS tyThe generally accepted histological subtypes of synovial sarcoma are biphasic, monophasic spindle cell or fibrous, monophasic epithelial and poorly differentiated subtypes . Among tImmunohistochemistry plays a crucial role in the diagnosis of SS, especially in monophasic type cases. Most synovial sarcomas show immunoreactivity for cytokeratins and epithelial membrane antigen (EMA). Furthermore, 30% of them are protein S-100 positive, 60–70% CD 99 positive and 75–100% Bcl-2 positive [SYT gene from chromosome 18 to either of two homologous genes at Xp11, SSX1 or SSX2 [Cytogenetics also play an important role since both the monophasic and the biphasic form are characterised by a reciprocal chromosomal translocation  p 11.2;q11.2). The translocation fuses the .2;q11.2. or SSX2 . Despite or SSX2 ,13.Pulmonary sarcomas are rare accounting for less than 0.5% of lung cancers and most malignant mesenchymal tumors of the lung are metastases of a primary tumor from elsewhere ,6. LeiomTwo thirds of the reported cases primary pulmonary SS were centrally located and associated with complaints of postobstructive pneumonia  and hemoptysis. Peripheral tumors are less common and usually asymptomatic, but may infiltrate adjacent tissues  or give rise to distant metastases  ,2. It isSputum examination is rarely helpful and bronchoscopy is diagnostic in 40–60% of cases. Differential diagnosis includes bronchogenic carcinoma, different metastatic lesions, mesothelioma, lymphoma, Wegener's granulomatosis, pyogenic abscess, intrapulmonary hematoma, rheumatoid nodules, histoplasmosis and coccidiomycosis. Lung SS frequently metastasizes to regional lymph nodes  as well as to distant sites . Owing to its rarity and the paucity of data regarding its natural history, there are no guidelines for optimal treatment. Therefore, current treatment includes surgical resection (lobectomy or pneumonectomy) followed by adjuvant radiotherapy or chemotherapy ,6,14. PrPrimary pulmonary synovial sarcoma is an extremely rare neoplasm. Clinical and imaging investigation is necessary to exclude alternative primary sources, while a definitive diagnosis requires detailed immunohistochemical staining . A balanced chromosomal translocation, t , is found in the majority of synovial sarcomas resulting in a chimeric transcript, SYT-SSX, the role of which is so far unclear. Surgical excision with clear margins and possibly adjuvant chemo-radiotherapy is the currently accepted treatment.The author(s) declare that they have no competing interests.CHM participated in the design of the study and performed the statistical analysis. AK participated in the design of the study and performed the statistical analysis. GP carried out the molecular genetic studies, participated in the sequence alignment and drafted the manuscript. VP conceived of the study, and participated in its design and coordination and helped to draft the manuscript. MP conceived of the study, and participated in its design and coordination and helped to draft the manuscript.The authors confirm that writteninformed patient consent was sought and granted for details of this case report to be published.


Pilomatrixoma was first described erroneously as being of sebaceous gland origin but was later discovered to be derived from hair matrix cells. They are rare, slow growing tumours of the skin found in the lower dermis and subcutaneous fat and are predominantly found in the neck and the scalp. While known to be locally aggressive, no malignant form was thought to exist until it was described relatively recently. Since then, approximately ninety cases of pilomatrix carcinoma have been reported.Pilomatrix carcinoma is the rare malignant counterpart of pilomatrixoma, a skin adnexal tumour originating from hair matrix cells. Pilomatrix carcinoma can arise as a solitary lesion We report the case of a 41 year old mentally retarded male who had a longstanding lesion in the left neck for approximately fifteen years previously diagnosed as a pilomatrixoma. He presented with severe headache, falls and visual disturbance and a biopsy showed pilomatrix carcinoma of the occipital region which, on computed tomography ( CT ) invaded the occipital bone, the cerebellum and the left temporal lobe. At his initial presentation he had a craniotomy and subtotal excision of the lesion but received no adjuvant therapy. After an early intracranial recurrence he had further debulking and adjuvant external beam radiotherapy. He has had no further intracranial recurrence after three and a half years of follow-up. Here we present the pathological features of this uncommon tumour. Pilomatrix carcinomas are the aggressive variant of pilomatrixomas, a type of hair matrix tumour. Although initially thought to be benign when first described by Malherbe and Chenantais , as earlA 41 year old male presented with a cutaneous tumour in the left posterior triangle of the neck which had been present for at least fifteen years. He had a history of mild mental retardation, bilateral undescended testes and had undergone appendicectomy as a child. He was otherwise well and in fulltime employment. He had multiple prior resections of the neck lesion which revealed benign pilomatrixoma. The recurrent mass measured 4.5 centimetres and was resected with clear margins. Pathologic analysis revealed a pilomatrix carcinoma. The patient had no further treatment at that time.He represented 7 months later with a two week history of falls associated with severe occipital headache, visual disturbance and a left sided neck mass. Prior to neurosurgical evaluation a Magnetic Resonance (MR) scan was performed Figure . This shOn postoperative MR imaging of the area an early recurrence was identified at three months, necessitating further subtotal debulking. He was then referred for adjuvant radiation therapy. The patient received fractionated three dimensional external beam radiation therapy to the occiput and left neck to a total dose of 50 Gy in 25 fractions. Serial follow-up MR scans have shown no progression in size of the intracranial component and the patient remains asymptomatic after 42 months follow-up.In our case, haematoxylin and eosin stained sections Figure  of the rSince Lopansri and Mihm first noted the existence of pilomatrix carcinomas in 1980 there have been fewer than ninety cases descibed and there has been only one previously reported case of invasion of the cranial vault . In the Fayyazi and colleagues have assessed expression of the cell proliferation associated antigen Ki 67 (MIB1) in 15 pilomatrixomas . HistoloPilomatrix carcinomas are locally aggressive tumours which have a propensity for recurrence, especially when incompletely excised. Wide excision is the preferred treatment and radiation therapy should be considered in the presence of adverse features such as recurrent disease or residual macroscopic disease, as in our case. In the cases where adjuvant chemotherapy and radiation have been used there are instances of apparent cure with these as well as cases with progression and death, despite treatment. In recurrent pilomatrix carcinoma, no chemotherapy regimen has been demonstrated to provide local control or to halt metastatic spread. The diagnosis should always be considered in the differential of any recurrent skin lesion with locally invasive behaviour.Written informed consent was obtained from the guardians of the patient for publication of this Case Report and the 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.NA drafted and revised the manuscript, as well as completing the literature search. DF supplied the clinical information on the patient, and critically evaluated the manuscript. DG prepared all the pathological material and revised the manuscript. JA devised the concept for the manuscript. All authors have read and approved the final manuscript.


Sarcomas are a type of malignant tumors that arise from connective tissue. They are most of the time found in extremitiesWe are presenting a case of adult male patient, who was found to have huge abdominal mass and multiple gastric and duodenal polyps. Pathological diagnosis for all lesions was Alveolar soft part sarcoma. Although he complained from metastasis to both lungs and right atrium, all these deposits regressed spontaneously. Patient stated that he used some herbs  prescribed for him. No chemotherapy or radiotherapy was given. The duration of regression was about 5 months before other lesions appeared. Later on, he died secondary to brain metastasis.ASPS is a rare type of sarcomas that affect primarily the lower limbs. This tumor does rarely metastasize to the gastrointestinal tract. Sarcomas are a type of malignant tumors that arise from connective tissue. They are most of the time found in extremities. The term -Soft Part- is used to distinguish these sarcomas from bone sarcomas. Although soft tissue sarcomas are linked to the tissue of origin; alveolar description is based on the histological pattern of the tumor rather than the origin.A 38 year old male married patient, medically free, referred to our center from a another hospital complaining from lower abdominal pain of few months duration, intermittent, vague, gripping in nature, associated with abdominal distention.He didn't undergo any type of surgery previously. Past history of the patient was negative for any fever, sweating, vomiting, constipation, or weight changes. He also didn't suffer from obstructive urinary symptoms. Family history was negative too.On examination, he actually was found to have large left, hypochondrial mass. It was painless & hard in consistency.On investigation, his hemoglobin level and leukocytic count were within normal range. Markers for colon, liver and prostate cancers were negative. On computerized tomographic (CT) scan; there was a large heterogeneous mass measuring about 16 × 12 × 10 cm occupying the left hypochondrial region with multiple small metastatic right lung lesions figures  extendinASPS is a rare type of sarcomas, accounting for less than 1% of soft tissue sarcomas . The usuIt has been reported that certain organs can be very unusually targeted by ASPS malignant cells. Part of the list includes female genital tracts, breasts and mediastinum. -7Gastrointestinal tract is also rarely involved by these malignancies. Only 3 cases were reported. In 2001, Michael S.et al reported a case of ASPS metastatic to small bowel mucosa causing polyposis and intussuseption in a 42 year old male with long history of ASPS metastasis to lungs and brain. . PrimaryIn our case, screening for primary lesions in extremities was negative. Pathological wise, depending on the cellularity of the tumor, the primary source is more likely to be from mesenchymal cells of the mesentery than from gastric or duodenal polyps,. This means that it can be considered the first case of this origin type to be reported in the literature.Spontaneous regression is a term that is used interchangeably with spontaneous remission. Tilden Everson and Warren Cole have defined spontaneous regression of cancer as the partial or complete disappearance of a malignant tumor in the absence of all treatment or in the presence of therapy which is considered inadequate to exert a significant influence on neoplastic disease. They stated that it is not implied that spontaneous regression need progress to complete disappearance of tumor nor that spontaneous regression is synonymous with cure, all cases in which a tumor underwent apparent spontaneous regression in one area but flourished unchecked in other areas or reappeared at a later date are considered as valid examples of this phenomenon in their opinion. Until now, this definition of spontaneous regression, with few additions, has remained the one in most common usage today . In factThe highest incidence of this phenomenon was in tumors of genitourinary organs. According to Caryle Hirshberg , betweenTo the best of our knowledge, only a single case of ASPS with spontaneous regression (of pulmonary metastases) has been published. In 2003, A 14 year old male patient was described to have ASPS in femoral region. A combined chemotherapy  was performed before operation. Tumor wide resection was carried out, however, a metastatic lesion revealed in the lung continued to increase in number and size during the progress after operation, however, it decreased in size and number after several years .In 1988, Pang and colleagues have report a case of female patient with ASPS who survived for over 9 years after detection of pulmonary metastases regressed when she was treated with oral and topical Chinese herbs. She had excision of a right buttock mass in 1975. She was given cytotoxic chemotherapy, but there was no radiological response. Thereafter; she took herbal mixture from March 1979 to July 1982. Chest X-rays in 1983 showed complete disappearance of some shadows and shrinkage of others. In a study conducted by Kebudi R. et al between 1989 and 2002, the incidence of brain metastases in extracranial solid tumors was studied in pediatric age group. Only 16 out of 1100 children with extracranial solid tumors developed brain mets. One of the 16 was found to be alveolar soft part sarcoma. The aim of publishing our case, the second of its type in the literature, was to encourage the study of biological behavior of ASPS as well as common molecular characteristics that could be present in other tumors that tend to regress alone.ASPS is a rare type of sarcomas that affect primarily the lower limbs. This tumor does rarely metastasize to the gastrointestinal tract. We can only speculate on the unusual spontaneous regression reported in this case. We do not have any solid information of a cytotoxic effect of the herb used, so we speculate that the regression was spontaneous probably mediated by the immunologic system of the host. However, this effect was short termed as the patient has died from further progression and metastasis of the tumor.Written informed consent was obtained from the next of kin for reporting of this case, the copy of consent is available with editor in chief.The authors declare that they have no competing interests.MB and ARA were involved in the concept and design, preparation of draft and editing of final manuscript. Both authors read and approved the final manuscript.


Invagination is a rare cause of mechanical intestinal obstruction in adults, but half of their causes are malignant. A diagnosis of invagination in an adult patient strongly suggests presence of a malignant pathology. Moreover some benign conditions may resemble malignant disorders like inflammatory fibroid polyp. Inflammatory fibroid polyps are rare benign lesions of uncertain origin that may occur in various parts of the gastrointestinal tract, with gastric antrum being the most common site, followed by the small intestine.A 31-year old male patient was admitted to our emergency department few hours after complaints of nausea and vomiting. Abdominal examination revealed distension and clanging intestinal sounds. Computed tomography showed intestinal obstruction without an obvious cause. The patient underwent diagnostic laparotomy. In this process, approximately 10 cm of invaginated mid-jejunal segment was seen. The pathologic segment was resected and end-to-end anastomosis was performed. The patient was discharged without any complication.Although IFP is seen very rarely in adults, it is one of the probable diagnoses that should be considered in obstructive tumors of the small intestine causing invagination. Inflammatory fibroid polyp (IFP) is a rare benign condition originating from submucosa . HistoloIFP is first described by Vanek as “submucosis granuloma with eosinophilic infiltration” . AfterwaIFP may manifest with a gamut of symptoms depending on localization of lesion. Intestinal IFPs may cause obstruction. Herein, we presented a case of IFP presenting as intestinal invagination in an adult patient.3), sodium: 134 mmol/L, potassium: 3.2 mmol/L. Plain films of abdomen disclosed multiple intestinal air-fluid levels. Contrast enhanced computed tomography showed intestinal obstruction without an obvious cause.A 31-year old Caucasian, Turkish male patient was admitted to our emergency department with the complaints of nausea and vomiting occurring few hours. It was learned that these complaints were repeating after meals for a week, episodically. On admission he looked severely ill and dehydrated. Abdominal examination revealed distension and clanging intestinal sounds. Laboratory investigation showed that leukocytosis  .Intestinal invagination is rarely seen in adults. When diagnosed, it strongly suggests presence of a malignant condition either primary or metastatic origin. Patients with invagination almost inevitably present with complaints of mechanical intestinal obstruction. Definite diagnosis is usually made by a diagnostic laparatomy. Treatment includes medical stabilization of patients’ condition and surgical resection at safe limits .IFP is an extremely rare condition in adults. It may manifest as invagination when located to intestine or as anemia and occult test positivity in the feces when located to stomach. Location of IFP in colon is extremely rare and may present with colicky abdominal pain and bloody feces. At the time of diagnosis most IFPs are in diameter of 3 to 4 cm, however, there is also a report of a case with 12.5 cm in size . Lesion The etiology and pathogenesis of IFP is unknown, but, it could be a consequence of extreme reaction of the body to an intestinal trauma or a localized variant of eosinophilic gastroenteritis given that it has a marked eosinophilic infiltration. ,3. HowevIn conclusion, IFP is one of the probable diagnosis in adults that should be considered in obstructive tumors of the small intestine causing invagination.


Schwannoma of frontoethmoid region is a rare presentation. We report a case of 11-year-old girl with a swelling at the root of nose and nasal dorsum. Based on clinical picture and radiological findings it was not possible to establish a definitive diagnosis. But the histopathological picture was suggestive of schwannoma. A novel surgical approach was adopted to facilitate complete removal of the tumor and provide best possible cosmetic results. Schwannoma of the frontoethmoid region is a rare entity. 25% to 45% of all schwannomas have been observed in the head and neck region. Out of all head and neck schwanomas, only 4% are seen in the nose and paranasal sinuses  [An 11-year-old girl from North-eastern part of India presented with 2 year history of progressive painless swelling at the root of nose. She had left sided nasal obstruction for one year and had occasional blood stained nasal discharge. Her vision was normal and had normal eye movements. There were no skin lesions/pigmentation over her any body part and had no signs of focal neurological deficits. Local examination revealed a smooth 4 × 3 cm swelling at the root of nose and bony nasal dorsum causing nasal deformity and telecanthus. The skin over swelling was normal and on palpation it was firm, non- tender and non compressible. The nasal bones appeared to be splayed and thinned out in the middle through which tumor was palpable. Anterior rhinoscopy revealed a pinkish mucosa covered mass filling the upper half of left nasal cavity and nasal septum was pushed to the opposite side.Contrast enhanced high resolution computed tomography (HRCT) of the paranasal sinuses revealed soft tissue expansile lesion of 42 × 36 × 20 mm size in left nasal cavity, left frontal sinus and bilateral anterior ethmoid area causing widening of nasal cavity and leading to thinning and erosion of right nasal bone. The mass was reaching upto but not eroding the cribriform plate. There were scattered areas of increased attenuation and mild post-contrast enhancement. Magnetic resonance imaging (MRI) showed a mass lesion of intermediate intensity on T1-weighted images and the T2-weighted signal without the cystic characteristics of the lesion . It can present at any age with no sex or race predisposition and local recurrences are infrequent.Clinically these tumors present like any other benign mass or polyp and the signs and symptoms depend on the site of tumor and the subsequent involvement of surrounding structures or its pressure effects. The most common symptoms are nasal obstruction, epistaxis, hyposmia and pain. A radiological investigation is essential to know the site and extent of the tumor. The lesions usually have a mottled appearance with peripheral intensification on contrast-enhanced CT scans. MRI is superior to CT scan in differentiating the tumor from inflammatory changes and very useful in evaluating intracranial extension. Sinonasal schwannomas have an intermediate to high intensity on T1-weighted images and the T2-weighted signal depending on the cellularity of the lesion and the cystic characteristics of the lesion .The differential diagnosis includes a spectrum of lesions ranging from angiomas and polyps to malignant tumors, such as melanoma or olfactory neuroblastoma. Since it is an expansile lesion without bone destruction it can be differentiated from malignancy and shows enhancement in CT unlike a mucocele. Electron microscopy and immunohistochemical analysis (S100) is necessary to differentiate a schwannoma from a neurofibroma. Although schwannoma are seen in order of frequency: nasoethmoidal region, maxillary sinus, the nasal cavity, and the sphenoid sinus with only a few reported cases of frontal sinus involvement in conjunction with the ethmoid sinuses ,5. Our pComplete surgical excision of the tumor should be attempted in first sitting to prevent recurrence. The approach for the resection should be chosen according to the location and extension of the tumor. The aim is to have i) adequate exposure ii) good cosmetic appearance, which is probably the most important from patient's viewpoint. Various combinations of techniques, including lateral rhinotomy, external ethmoidectomy, Caldwell -Luc approach; midface degloving, and/or endonasal endoscopic resection can be used ,9. Our cAn informed written consent was received from the father (patient being minor) for publication of the manuscript and figuresThe authors declare that they have no competing interests.AR, data collection, wrote manuscript, assistant surgeon: RK, concept, wrote manuscript, surgeon: CVK, data collection: AJ, histopathological report, microphotograph: RCD, concept, editorial adviceAll authors have read and approved the final manuscript.


Spontaneous rupture of the esophagus (Boerhaave's syndrome) is a rare, well-defined clinical syndrome caused by a longitudinal perforation of the esophagus. It is a life-threatening condition that necessitates rapid diagnosis and treatment. Patients typically present acutely with a history of vomiting followed by chest or abdominal pain. However, the diagnosis may be difficult or missed when patients present with chronic symptoms that mimic other conditions.In this report, we present a unique case of Boerhaave's syndrome in a 53-year-old male patient. In contrast to the more common acute presentation, our patient developed non-specific symptoms in association with an intrathoracic cyst. In this report, we will also review the usual presenting signs, symptoms, and treatment of Boerhaave's syndrome.Our emphasis in this paper will be on the importance of recognizing and diagnosing Boerhaave's syndrome in an acute as well as a chronic state. Boerhaave's Syndrome is a condition of spontaneous, longitudinal esophageal tear due to the elevated intraesophageal pressure that that classically follows repeated episodes of vomiting. Since the distal third of the esophagus is inherently weaker than the rest of the esophagus, ,2 rupturFurthermore, presentations of esophageal perforation can be distinguished as acute, subacute, and chronic. Acute perforation presents with symptoms within twenty-four hours after rupture. In a subacute rupture, symptoms develop between twenty four hours to two weeks following perforation. With chronic perforation, the onset of symptoms is more insidious, often delaying presentation and diagnosis for weeks to months after rupture ,6.A history of forceful emesis, subxiphoid chest pain, and subcutaneous emphysema (termed the Mackler Triad) is a common triad of symptoms that should suggest acute esophageal rupture ,4. HowevTreatment of spontaneous esophageal rupture can be either non-operative or operative. Nonoperative treatment is best for patients with a contained perforation and the absence of clinical mediastinitis ,11. SuchA 53-year-old Caucasian male with a history of heavy nonsteroidal anti-inflammatory drug (NSAID) usage and chronic alcohol abuse presented to the emergency room with a four-month history of dysphagia, 45-pound weight loss, coffee-ground vomitus and upper abdominal pain. Physical examination was normal, except for nonspecific epigastric tenderness, as were routine laboratory studies including CBC, chemistries and liver function studies. Although chest X-rays showed only mild perihilar consolidation, esophagogastroduodenoscopy (EGD) confirmed the presence of a large, intrathoracic, cystic structure, partially filled with necrotic debris, arising from a large entry orifice just above the gastroesophegal junction. Computed tomography (CT) of the chest demonstrated bilateral pleural effusions, pneumomediastinum, and a 6.7 cm diameter, fluid-filled diverticulum extending from the esophagus into the posteromedial left hemithorax, Fluoroscopy, performed during injection of barium through a nasogastric tube positioned in the distal esophagus, showed contrast extravasation from the distal esophagus into this cavity in the left chest.The patient was treated with targeted drainage, intravenous antibiotics, nasogastric decompression, and enteral nutrition. However, he became septic two days after the EGD, and required emergency thoracotomy with distal esophagectomy following several days later. After ashort term of ventilator support and parenteral nutrition, he was weaned off of fboth, and was discharged with a PEG tube for enteral feedings, and is currently being evaluated for esophagostomy reversal.While a spontaneous esophageal rupture usually presents in an acute setting, it can also present in a subacute or chronic manner as well. Similarly, though patients usually have symptoms of vomiting, chest pain, tachycardia, and tachypnea, a patient with Boerhaave's Syndrome may also present with non-specific complaints and no major physical findings. Due to this diverse range of presentations, the diagnosis of Boerhaave's Syndrome can be difficult, and effective treatments are often delayed. Thus, the diagnosis of Boerhaave Syndrome must be considered in any clinical setting in which a patient presents with a highly suspicious history with or without specific complaints.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.UFM was involved in writing, reviewing and editing the manuscript. He was the corresponding author who drafted the initial article. He also finalized the manuscript. RY was involved in writing, editing and reviewing the manuscript. HDP reviewed and edited the manuscript. AMC: was involved in initial writing and editing of the manuscript. BS: was involved in writing, gathering and organizing pictures and final editing of the manuscript. RL was the chief gastroenterologist involved with the case. He also reviewed, edited and finalized the manuscript. AM was the chief of surgery and attending involved with the case. He also reviewed and finalized the manuscript.All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/10/29/prepub


Hepatocellular carcinoma (HCC) is the most common primary tumor of the liver worldwide. The incidence of HCC is increasing in North America secondary to rises in chronic liver disease from alcohol abuse and viral hepatitis. HCC most commonly metastasizes hematogenously or through lymphatics to the lungs and regional lymph nodes. Involvement of small bowel is rare and typically results from direct invasion and extension. We examined the molecular features related to this extremely rare case of isolated duodenal metastasis of HCC and noted p53 and Ki-67 positive staining. Here, we review the possible molecular and immunohistochemical studies that may aid definitive diagnosis and the evidence for the management of metastatic hepatocellular carcinoma. Hepatocellular carcinoma (HCC) is the 6th most common cancer and the 3rd most common cause of cancer death worldwide . It is mHCC most commonly metastasizes via blood vessels or lymphatics to the regional lymph nodes, lungs and bone. Other less common sites of spread that have been reported include the adrenal gland, stomach, peritoneum, kidney, spleen, heart, and brain. Metastasis to the small bowel is rare and the few cases of small bowel involvement have been reported only as case reports or case series. In most of the reported cases, small bowel involvement was located at the proximal duodenum as a result of direct extension from the primary tumor -6. HematThe patient discussed in this case report presented with an upper gastrointestinal bleed secondary to HCC metastases located at the distal duodenum, which to our knowledge, has been reported only once previously Here, weThis 53-year-old Canadian First Nations woman presented to emergency with abdominal pain, chest discomfort and melena. Her medical history included chronic hepatitis C, previous heavy alcohol consumption, cirrhosis and a recent diagnosis of multifocal hepatocellular carcinoma. Her other comorbid illnesses included type 2 diabetes mellitus, atrial fibrillation and valvular heart disease. At the time of initial diagnosis of HCC, a CT scan demonstrated multiple hepatic lesions and portal vein thrombosis. Although there was no evidence of tumor extension beyond the liver, her underlying heart disease and the presence of portal vein thrombosis deemed her an ineligible candidate for either surgical resection or transarterial chemoembolization. Since her diagnosis of HCC, she had been managed with long acting octreotide  20 mg intramuscularly given monthly for seven months when she presented with abdominal discomfort and melena.At presentation, she was hemodynamically stable and the physical examination was unremarkable apart from the presence of melena stool. Laboratory investigations showed hemoglobin 85 g/L, with MCV of 71.7 fl, and a normal serum AFP.Esophagogastroduodenoscopy (EGD) revealed two lesions in the third part of the duodenum: (i) a 2.5 cm soft nodule resembling the liver parenchyma with a dark red colour and hyperemic base, initially covered with a large clot (ii) a larger ulcerated lesion located distal to the first nodule, which was also covered in clot and more difficult to visualize Figure . These lThe biopsy fragments showed a moderately differentiated carcinoma growing in a sheet-like fashion with an indistinct sinusoidal pattern. The tumor cells had abundant pale pinkish or clear cytoplasm and vesicular nuclei with prominent nucleoli Figure . Mucin pKi-67 immunohistochemical staining was performed to assess proliferative activity in the tumor. One thousand cells were counted and 100 cells (10%) were positive. The expression of a tumor suppressor, p53, was also assessed. Twenty four percent of cell nuclei were either lightly or heavily stained.Hematogenous metastasis to the duodenum from any primary malignancy is generally uncommon. Hematogenous metastasis from HCC to the duodenum is exceedingly rare. There have been several primary tumors with reported hematogenous metastases to the duodenum including carcinomas of the lung, breast, and malignant melanoma -10. In tIn this patient with known multifocal HCC, duodenal lesions resembling liver parenchyma were found endoscopically, raising the suspicion that these lesions were metastatic deposits of HCC. The majority of cases of duodenal involvement with HCC are a result of local extension of the primary tumour, but the CT scan demonstrated no local invasion into the small bowel ,5,6. If In the setting of an unclear clinical presentation and small biopsy specimens, it is often necessary to confirm the histologic diagnosis using molecular and immunohistochemical studies. This is particularly true for endoscopically obtained biopsies, which are frequently small and often damaged with crush injury. Immunostaining of the biopsy fragments for Heppar 1 and AFP may help to resolve the histologic diagnosis of HCC. Heppar 1 immunostaining is present in 86% of hepatocellular carcinomas . HoweverThe presence of high levels of p53 and Ki-67 expression in this case correlates with a more aggressive tumor phenotype. Positive p53 and Ki-67 markers (positive staining in >10% cells), in addition to high serum AFP levels, have been suggested as predictors of poor clinical outcomes and earlier recurrence of HCC . Our fin131 lipiodol, hormonal therapy, immunotherapy, and octreotide. Meta-analysis of the randomized trials comparing chemoembolisation with conservative management has shown a survival benefit with chemoembolisation. The chemotherapy agents previously tested for chemoembolisation include doxorubicin and cisplatin. On the other hand, meta-analysis of tamoxifen compared with conservative management failed to show a survival benefit [With regards to treatment options, there is somewhat limited evidence to guide our management approaches, particularly for advanced tumour stages. Attempted treatments in the advanced setting include chemoembolisation, arterial or systemic chemotherapy, I benefit . A numbeIn this case, the patient's management was somewhat limited by the presence of portal vein thrombosis, which made her ineligible for chemoembolization, and her other medical comorbidities, which would pose significant risk with more aggressive treatment. Given the available evidence around palliative single agent chemotherapy, she was treated with six cycles of doxorubicin every three weeks at disease progression. She survived another seven months on this regimen. It is difficult to know whether this treatment extended her survival in light of her extremely rare presentation of isolated duodenal metastasis.This is the second reported case of isolated duodenal metastasis of HCC without evidence of direct invasion or widespread disseminated metastases. Given this rare presentation, the diagnostic challenges were reviewed with descriptions of possible molecular and immunohistochemical studies that may help establish a definitive diagnosis. A brief review of the evidence for various treatment options in the setting of metastatic HCC was also presented, which supported the management of this particular patient. This case demonstrates the variability in presentation of metastatic HCC but also emphasizes the need for further tools to improve diagnosis and treatment of metastatic hepatocellular carcinoma.AFP: alpha fetoprotein; CT: computed tomography; EGD: esophagogastroduodenoscopy; HCC: Hepatocellular carcinoma; VEGF: Vascular endothelial growth factor.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.CC and JAA collected the information and wrote the case history of manuscript. CC finalized and submitted the manuscript. DO collected the pathological information and contributed to the pathological component of the case history and discussion of the manuscript. AW contributed to the endoscopic photos for the case history. EMY and IT contributed to the discussion and edited the final version of the manuscript. All authors read and approved the final manuscript.


Lipoma is by far the most common of all benign soft-tissue tumors which far outnumber malignant tumors. Soft-tissue sarcomas are malignant tumors and are usually named for the type of tissue in which they begin. Liposarcoma (LPS), which arises in the fatty tissue, is rather an uncommon soft-tissue tumor. Multiple histologic subtypes of liposarcoma are recognized, including myxoid liposarcoma, and correspond to tumors of very different prognosis. In two-third of the cases, this tumor occurs in the muscle while often demonstrating a misleading benign appearance as observed in the majority of soft-tissue sarcomas.We report the case of a 50-year-old man operated on for a fat tumor of the thigh initially diagnosed as lipoma but revealing to be a myxoid liposarcoma after histopathological examination. The initial incomplete tumor excision required the need for a re-excision with adjuvant chemotherapy and complementary radiotherapy.When any suspicious soft-tissue tumor is diagnosed, the combined information gathered from accurate preoperative radiographic planning and X-rays or surgical biopsy is of tremendous value for establishing the most appropriate therapeutic program, highly adapted to the histopathological findings. Lipomas account for 50% of all benign soft-tissue tumors. Malignant tumors or sarcomas comprise approximately 1% of all soft-tissue tumors. They are also rare among the malignant tumors that occur in adult, reporting a prevalence lower than 1% and an incidence of 30 cases per million population . LiposarMr A. Oma, a patient aged 50 years, with a 5-year history of a small tumorous lesion, and regularly followed after a polytraumatism, presents on 23 December 2003, with the complaint of a gradually growing mass in his thigh for three months. This small tumor involving the antero-lateral aspect of the thigh within the upper one-third, has a soft consistency, no adherences to the surrounding structures and is well circumscribed. Neither functional nor general symptoms are associated and routine blood tests are normal. Such features suspects a common adipose tumor of lipoma type and its excision is scheduled on 27 January 2004. This simple and rapid surgical procedure includs the excision of an intramuscular, subaponeurosis tumor, very well defined within the vast external of the crural quadriceps. The tumor of approximately 8 cm is sent to the laboratory of pathological anatomy. The excised specimen is a 7.5-cm, well defined, flexible, ovoid tumor of gelatinous aspect and brownish appearance, with some more fleshy zones, without necrotic or hemorrhagic changes.Histologic examination reveals a myxoid-type tumor, consisting of an amorphous mucoid material, slightly colored with small dark oval cells and no evidence of atypia or mitotic figures fig. . An exte2), Ifosfamide (2500 mg/m2), Dacarbazine (300 mg/m2) and Mesna (6000 mg)] combined with radiotherapy delivering 50 Gy on the left thigh followed by 10 Gy focused on the operating site.Postoperative management is simple: from March to July 2004, the patient is treated with adjuvant chemotherapy consisting of six cycles of MAID-type [Doxorubicine  reveal normal with minor orthopedic sequelas; a schedule of radio-clinical follow-up is established (MRI Scan and TAP-CT-Scan); the patient is in clinical remission at five years.Despite the misleading benign appearance of this malignant tumor, complete preoperative X-Ray investigations and biopsy should have been performed since any deep tumor of soft tissues and/or which size exceeds five centimeters is considered as being suspect and requires biopsy prior to any excision procedure. Biopsy plays a crucial role in accurate histopathological diagnosis, and proper staging would enable the oncology committee to implement the most appropriate therapeutic protocol . The firThe genesis of soft-tissue sarcomas has not yet been clearly defined but several contributing factors that increase the likelihood of developing these tumors have been identified: external radiation therapy and genetic factors are the most well-established risk factors for soft tissue sarcomas. Approximately 1% of patients treated with radiation therapy for a malignant tumor, might develop, in previously irradiated tissues, an osseous or soft tissue "radiation-induced sarcoma", which may appear from three to ten years later . Some geIt is often well defined with little adherence to the adjacent structures. The clinical diagnosis of malignancy of this adipose tumor is thus difficult but findings of an important size > 5 cms) and rapidly growing mass should alert and lead to the realization of an appropriate preoperative X-Ray investigation (U/S-Scan then MRI-Scan and biopsy) ,2,14. Th cms and The operative specimen must be located and oriented, to accurately define the margins of the excision. It is sent to the pathologist, in a fresh state, along with a thorough clinical information mandatory for proper histological analysis ; the type and objective of the surgical procedure are detailed . The myxThe present case clearly illustrates the problems associated with the management of these rare tumors and which might be encountered by any surgeon, whatever the surgical field. Any soft-tissue tumor diagnosed in the adult, particularly when its size exceeds five centimeters and/or when of subaponeurotic origin, typically requires the need for a careful preoperative X-ray investigation combined with a diagnostic biopsy. In case of malignancy, an early accurate medico-surgical treatment should be implemented, associated with a systematic histopathological examination. The prognosis for patients with soft tissue sarcoma largely depends on the quality and coherence of the initial management protocol based on a close multidisciplinary cooperation within the oncology committee.The authors declare that they have no competing interests.FL prepared the draft manuscript. CB helped in preparation of the draft manuscript. FC contributed pathological part of the manuscript and the photomicrographs. All authors read and approved the final manuscript.Patients consent was obtained for the publication of this case report.


Although synovial cell sarcoma is a common tumor of the extremities, its occurrence in the thorax has been less frequently documented.A 46-year-old Pakistani man presented with a 2 month history of progressively increasing cough and left lower chest pain. Initial evaluation was done using a chest x-ray; the patient was found to have a large mass involving the lower portion of the left chest. A computed tomography scan was performed next which showed a large mass involving the left chest wall with invasion into the pericardium and left hemidiaphragm. En bloc surgical resection of the tumor was undertaken. Final pathology showed synovial cell sarcoma of the thorax. At one-year follow-up, the patient has shown no recurrence of the disease.We have described a rare case of a large synovial cell sarcoma of the thorax. Surgical resection appears an appropriate modus operandi for managing giant synovial cell sarcomas of the thorax. However, there is a need to clearly define post-operative strategies for cases with extensive involvement of surrounding structures. Synovial cell sarcoma (SCS) is a common tumor of the extremities. However, SCS of the thorax is an overall rare clinical entity. We report here a case of SCS in a 46-year-old man with extensive involvement of the chest wall, lung and diaphragm.A 46-year-old Pakistani man presented to our hospital with a 2 months history of progressively increasing cough and left lower chest pain. Initial evaluation was done using a chest x-ray and he was found to have a large mass involving the lower portion of the left chest. Computed tomography (CT) scan was obtained next to better delineate the disease process Figure . It showth ribs was seen. It was also involving the left lower lung lobe, left hemidiaphragm and pericardium (Figure Trucut biopsy of this mass showed features of SCS. In the absence of effective chemotherapy and obvious metastasis, the patient was planned for a complete resection of this tumor. The tumor was exposed via a left posterolateral thoracotomy approach. Intra-operatively, a large tumor with extensive involvement of the left lower chest wall from the 4th to 10m Figure . The masSCS is a malignant tumor that has been termed such because of histological resemblance to developing synovium . It is tSCS of the thorax represents a diagnostic challenge because of the diverse array of competing diagnosis and rarity of incidence . The symTreatment of choice for SCS sarcomas of the thorax is excision, radiation therapy and adjuvant chemotherapy following resection. In our case, successful surgical resection was performed for a large synovial sarcoma of the left chest wall with involvement of left lung, pericardium and left hemidiaphragm. The diaphragm and left chest wall had to be reconstructed with prolene mesh and muscle flap after complete resection of tumor. Our patient is not receiving any radiation therapy because of proximity of the bare heart to the chest wall. Also, the need for adjuvant therapy was obviated because of achievement of histologically negative margins. As SCS is known to recur, the patient needs to be followed up carefully.We have documented a rare case of a giant SCS of the thorax with extensive involvement of surrounding structures. This is a rare clinical entity and there is a need to clearly define post-operative strategies for a case with extensive involvement like ours.CT: computed tomography; MRI: magnetic resonance imaging; SCS: synovial cell sarcoma.Written, informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the consent form is available for review by the Editor-in-Chief of the journal.The authors declare that they have no competing interests.TS was involved in data collection, interpretation and drafting the manuscript. SHF was involved in study design and conception, data interpretation, drafting the manuscript and providing overall supervision in the project. All authors read and approved the final manuscript.


An air filled lesion can be a diagnostic dilemma and a careful investigation must be following to clarity any underlining pathology.A 62-year old male, ex smoker, with a history of chronic cough was examined with helical CT tomography and an air filled lesion was demonstrated at the right paratracheal region at the thoracic inlet. A narrow connection to trachea lumen was also visible, a critical element to establish the diagnosis of ttracheal diverticulosis.This malformation is a rare anomaly with two types, the congenital and the acquired one. It must be included into the differential diagnosis of any air filled lesion at the thoracic inlet. Computed tomography scans (with thin section and reconstructed images) seem the proper imagine. Bronchoscopy can also visualize the diverticulum although sometimes the connection with trachea can't be detected. In most cases is asymptomatic and needs no special treatment. A possible danger of repeated respiratory infections and insufficient intubation and/or ventilation must be in mind. An air filled paratracheal lesion is a rare radiological finding and several entities must be included in the differential diagnosis. Although in most cases the physician's suspicion is oriented to the digestive system, some rare anomalies arising from the respiratory system could be the cause. One of these is tracheal diverticulosis. This malformation has two forms-the congenital and the acquired one- and although some patients remain asymptomatic, others may have a history of repeated respiratory infections or unsuccessful intubation.A 62-year old male, ex-smoker, with a history of chronic cough was referred for helical chest computed tomography. Until then physical examination, spirometric values and routine laboratory results were unremarkable. On scanogram an ovoid air filled lesion originating from the trachea was detected at the right paratracheal region, at the level of the thoracic inlet figure . The conThe differential diagnosis of an air filled paratracheal region includes a laryngocele, a pharyngocele, a Zenker's diverticulum, tracheal diverticula, an apical hernia of the lungs and apical paraseptal bullae. Radiological barium swallow study, endoscopic techniques and computed tomography can be used to characterise the lesion -3.Tracheal diverticulum is rare and usually found post-mortem. The frequency of the tracheal diverticulum in some autopsy series has been estimated to 1%. On the other hand, Goo et. al. reported sixty four proven cases in a single study among livings ,4. In moTracheal diverticulum is commonly asymptomatic and incidentally detected. Nevertheless, it can act as a cavity full of secretions and manifest clinically with chronic cough, repeated episodes of respiratory infections, haemoptysis, stridor or dyspnoea. Compression of the vocal cords by the diverticulum can cause dysphonia -4,7,8. FComputed tomography examination, including thin sections or reconstructed images, is the proper imaging method for the study of tracheal diverticulum. It can demonstrate the connection between the diverticulum and the tracheal lumen and the possible damage of lung's parenchyma as the result of chronic illness. Although bronchoscopy can establish the diagnosis, diverticula with a narrow opening or just a fibrous connection with the trachea can be bronchoscopically missed -3,6,7.Surgical treatment of tracheal diverticulum is rarely advocated. Conservative measures, such as antibiotics, mycolytics agents and physiotherapy are proposed especially in older patients ,2,5,7.The authors declare that they have no competing interests.All authors were involved in patient's care. IK, IK and HM prepared the manuscript, DH, SB edit and SK coordinated 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


A rare case of grade IV frostbite is presented resulting in bilateral below knee amputations. This case highlights the importance of early versus late amputation as well as the importance of close collaboration between the rehabilitation, surgical, psychosocial, and public health disciplines in this rare and challenging problem that still may be encountered in the United Kingdom. This is the case of a 50-year old homeless man who sustained grade IV frostbite injuries to both feet after sleeping out in sub-zero temperatures. The patient first presented with ischaemic feet with well demarcated areas around the ankle Figure . The feeDelayed amputation had a positive effect on psychological acceptance of bilateral amputations by the patient as well as preparation for rehabilitation.Bilateral below-knee amputations are rarely performed. The predisposing factors include diabetic foot sepsis, atherosclerosis, frostbite, burns, trauma, calciphylaxis-related gangrene, suicide attempt, sensory loss and calf-wound healing failure after coronary revascularisation ,2. One oFrostbite can be graded into stages I to IV depending on tissue depth involvement. Two main approaches can be adopted with deep frostbite, either (a) early necrectomy with subsequent local treatment of wounds and (b) long-term conservative treatment until formation of demarcation line of necrotic tissue with subsequent resection and formation of a stump to facilitate rehabilitation.This case highlights the importance of careful consideration of early versus late amputation as well as the importance of close collaboration between the rehabilitation, surgical, psychosocial, and public health disciplines in this rare and challenging problem that still may be encountered in the United Kingdom.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 author declares that he has no competing interests.MJR is the sole author to this article and has written and researched the case himself.


The finding of umbilical metastasis has historically been called a “Sister Mary Joseph Nodule”. A few case reports of lymphoma presenting in this manner have been documented. We report a case of relapsed mantle cell lymphoma (MCL) presenting as a Sister Mary Joseph's nodule. Although the few reports of lymphoma exhibiting umbilical metastasis suggest that patients may still expect a reasonable response to chemotherapy, this patient experienced multiple relapses, despite aggressive chemotherapy regimens. This clinical course is characteristic of the mantle cell form of non-hodgkin's lymphoma and illustrates a need to seek out more effective therapies. Umbilical malignant metastasis is a rare sign of advanced malignancy and usually associated with cancers of the gastrointestinal and reproductive tract . The terInterestingly, reports of hematological malignancies presenting in this manner are exceedingly uncommon and when reported, do not necessarily represent intractable disease. In a review of 407 cases of SMJ nodules, only 1 was noted to be of lymphomatous origin , 6. FurtA 72-year-old male with a history of previously treated blastic variant (Ki-67 60%–70%) mantle cell lymphoma (MCL) presented to his outpatient oncology clinic with new complaints consisting of diffuse abdominal pain and distention, night sweats, fatigue, and a new mass within the umbilicus. The patient was noted to have a Mantle Cell Lymphoma International Prognostic Index (MIPI)  of 6, pr Recurrence was noted by PET scan to have occurred in areas of previous involvement, including several areas above the diaphragm, the retroperitoneum, and the right pelvis. However, abdominal examination revealed a new, nontender, violaceous mass projecting from the umbilicus . ComputeMCL is characterized by frequent extranodal involvement with a predilection to the aerodigestive tract, though ocular, central nervous system, breast, skin, and testicular involvement have been reported –17. StudIn general, MCL is characterized by lack of sustained responses to conventional therapies and poorer outcomes when compared to other lymphomas. While five-year survival after standard R-CHOP therapy was recently demonstrated to have improved from 22% to 47% , worse o


We report an unusual case of Ewing's sarcoma / primitive neuroectodermal tumor (PNET) of the sacral nerve plexus in a 9-year-old boy who presented with a soft tissue swelling and severe piercing pain in the lower back region. MRI of the lumbosacral spine showed a lobulated soft tissue mass with clubbed finger-like projections along the path of the sacral nerves, which had caused widening of the spinal canal and the sacral foramina (S2–S4 level). There was presacral extension and posterior scalloping of the sacral vertebrae. Histopathology of the lesion confirmed Ewing's sarcoma / PNET of the sacral spinal nerve plexus. The patient responded favorably to chemotherapy and radiotherapy, showing clinical and radiological improvement. Extraosseous Ewing's sarcoma (EES) / primitive neuroectodermal tumor (PNET) of the sacral nerve plexus is an extremely rare primary soft tissue malignancy. It has been grouped along with Ewing's sarcoma of the bone (ESB) under the Ewing's sarcoma family of tumors (ESFTs). Many casA 9-year-old apparently healthy boy presented with a gradually increasing painful swelling of 4 months' duration over his lower back. The pain was localized to the lower back, severe in intensity, and piercing in nature; it did not radiate and increased with movements. It was associated with urinary incontinence for the same duration. There was no history of trauma. There was no history of any significant medical or surgical illness in the child or his family.Local examination of the site revealed a small tender swelling in the lumbosacral region, with no signs of inflammation, no visible scar / sinus, and no abnormal pulsations. The swelling was fixed to the underlying structures and the overlying skin was freely mobile. The child was anemic, with hemoglobin of 10.2 gm%; the ESR was 62 mm in the first hour (Westergren's method). The other routine investigations were within normal limits.Radiographs of the pelvis and lumbosacral spine revealed posterior scalloping of the sacral vertebrae and widened ill-defined neural foramina; there was an overlying right-sided soft tissue haze. MRI of the lumbosacral spine revealed a lobulated soft tissue mass with both intraspinal and extraspinal components in the sacral region S2–S4). It was isointense to pelvic muscles on T1W and hyperintense, with areas of necrosis, on T2W images  and 2; t–S4. It wAn open biopsy of the lesion revealed a proliferative growth. Microscopically, there were monomorphic-appearing small round cells arranged in sheets, with dispersed congested vessels. The cytoplasmic borders were indistinct, producing a syncytial appearance. Mitoses were seen. The nuclei appeared to be round to cleaved. These features are suggestive of Ewing's sarcoma / PNET . Based oThe patient was started on chemotherapy , which was followed by radiotherapy to the tumor bed. Radionuclide scan of the whole body (Tc-99m-MDP) after six cycles of chemotherapy revealed no evidence of metastases. The patient responded well to the chemotherapy and radiotherapy and became asymptomatic. Repeat scans showed almost complete resolution of the mass.et al. in 1969[EES / PNET is a rare malignant small round cell neoplasm of undifferentiated mesenchymal origin.6 It was . in 1969 in four . in 1969 The site. in 1969In the present case, the tumor arose from the sacral spinal nerve roots; this is infrequently reported, althoughThe classical histopathological features of ESFT consist of uniform round cells, with irregularly shaped chromatic nuclei surrounded by scanty cytoplasm. Mitotic figures may be seen. Special cellular arrangements, like rosettes or differentiations, are not often seen. The cells often show immunohistochemical positivity for various neurofilaments, CD99, and S-100.5Ewing's sarcoma / PNET arising from the sacral nerve plexus is only rarely reported and there is a need to differentiate it from other tumors arising in this region. The benign nerve sheath tumors such as neurofibromas are well-defined lesions, with a ‘target’ appearance due to a hyperintense rim and a hypointense center on T2W and contrast-enhanced T1W images (due to collagen and condensed Schwann cells); they arDepending on the site of the tumor and its extension, treatment can be with surgery, chemotherapy, and radiotherapy, used separately or in various combinations.4 Our patIn conclusion, the present case highlights a rare case of EES / PNET presenting in the first decade of life and arising from the sacral nerve plexus. The mass was seen to grow along the sacral nerves and the diagnosis could he established only after a biopsy. The complaints resolved following therapy.


Tumor recurrence following radical cystectomy for a low-grade superficial transitional cell carcinoma (TCC) is exceedingly uncommon and has not been reported previously.We describe a case of a young male presenting with anorexia, weight loss and a large, painful locally destructive pelvic recurrence, ten years after radical cystoprostatectomy. The pathology was consistent with a low-grade urothelial carcinoma. After an unsuccessful treatment with cisplatin-based chemotherapy, the patient underwent a curative intent hemipelvectomy with complete excision of tumor and is disease free at one year follow-up.A literature review related to this unusual presentation is reported and a surgical solutions over chemotherapy and radiotherapy is proposed. Low-grade papillary (Ta) urothelial carcinomas have the lowest risk of progression to invasive disease and death of all the superficial tumor types, with 50–70% recurrence rate after transurethral resection of bladder tumor (TURBT) and progression to invasive disease in 2.4–3.3% of cases . In compA 48 year old male with a long history of smoking presented with weight loss, anorexia and pelvic pain. He had a significant past history of a radical cystectomy ten years prior for a large multi-focal non-invasive, low-grade papillary (Ta) transitional cell carcinoma. The stated indications for cystectomy were large size of the mass and the anticipated inability to perform a complete resection. The pathological specimen which was reviewed at our institution was described as a low-grade non invasive papillary multifocal transitional cell carcinoma (TCC). The margins were clear and fourteen uninvolved lymph nodes were submitted. Postoperatively the patient recovered well and was under surveillance without any disease till above mentioned complaint. The patient's past history was also significant for a straddle injury requiring open surgical repair that occurred approximately 2 years prior to the diagnosis of bladder cancer.Physical examination revealed a thin uncomfortable male with no other abnormal findings. Basic laboratory investigations were within normal limits. Imaging studies with CT scan of abdomen and pelvis revealed a right sided large heterogeneous pelvic mass with an area of central necrosis and evidence of bone destruction (right acetabular invasion) and distal rectal involvement Figure . There wen bloc resection represented the best option for patient.A CT guided biopsy of this mass revealed a low-grade urothelial carcinoma. Cisplatin based chemotherapy along with growth factor support was administered . After 3 uncomplicated cycles no tumor response was achieved. It was then decided that a curative intent The patient underwent surgical resection of the mass requiring a right hemipelvectomy, end colostomy and a myocutaneous flap closure with penile and scrotal reconstruction. The final pathology revealed an urothelial cell tumor with predominantly low-grade morphologic features, with focal areas of high grade tumor seen , extent of invasion (T) and lymph node involvement (N) are the most widely recognized, beside others . Herr etsurvival . Data resurvival . The samVarious site of metastasis such as skin, lung, orbit metatarsal bone, penis, posas muscle and calcaneum have been reported in the literature in patients with superficial bladder cancer -9.Saito reported a case of solitary subcutaneous scrotal metastasis 18 months following initial treatment with TURBT and intravesical instillation of Bacillus Calmette-Guérin (BCG) with no tumor recurrence on repeat cystoscopy. The histology of scrotal lesion was consistent with the primary bladder tumor showing intermediate grade transitional cell carcinoma (pT1a) disease .et al., reported a case of delayed recurrence 20 years following radical cystectomy for a low-grade muscle invasive disease with skin and pelvic metastasis. The histology from skin recurrence was consistent with well-differentiated TCC. Subsequently patient developed a pelvic recurrence in spite of chemotherapy [Ku otherapy . In our et al., reported a case of vaginal and omental metastasis six years after TURBT for a well-differentiated superficial TCC. Subsequent evaluation revealed no visible tumor in the bladder, but large omental deposit and left obturator lymph node mass engulfing the ureter. The report does not document the grade of recurrent TCC [Kumar rent TCC .et al. [Recently Dougherty et al. , reporteet al. .There are many similarities of the above cases in the literature to our case. To our knowledge our case is the first reported case of a non-invasive low grade urothelial carcinoma treated with cystectomy with a late recurrence of the same low-grade disease. The value of the cystectomy in our case is high, because of the well known problem of clinical understaging in urothelial carcinomas Table .The mechanism responsible for such a delayed presentation in our case is unknown. It is very likely that the tumor was seeded in the pelvic area over 10 years prior, and considering the location of the tumor and its low-grade, it did not become symptomatic for many years. The history of saddle injury and/or the repair of this injury may have played a role in this case. Traumatic implantation of the cancer cell is supported by a report of similar implantation metastasis following laparoscopic bladder biopsy for bladder cancer . Thus a Modern cisplatin-based combination chemotherapy regimens are associated with 40–60% objective response rates in metastatic high-grade urothelial carcinomas. The regimen used in our case is associated with an overall response rate of 62% . Our intWe present an exceedingly rare occurrence of a pelvic recurrence of a low-grade superficial TCC after cystectomy. Delayed presentation with recurrent low-grade urothelial carcinoma is an unusual entity and potential mechanism of traumatic implantation should be considered. Characteristically low-grade tumor's are resistant to systemic chemotherapy and curative-intent surgical resection of the tumor should be considered.TURBT: Transurethral resection of bladder tumor; TCC: Transitional cell carcinoma; MVAC: Methotrexate, vinblastine, doxorubicin and cisplatin.The authors declare that they have no competing interests.PPD – concept and design, collection and assembly of data, analysis and interpretation of data and preparation of manuscript. WPW – provided study material and patient, editing of the manuscript and approval of final draft. JM – provided study material and patient, editing of the manuscript and approval of final draft. AM – provided study material and patient, editing of the manuscript and approval of final draft. PM – Conception and design, provided study material and patient, data analysis and interpretation and preparation and editing of manuscript. All authors read and approved the final manuscript.Written informed consent was obtained from the patients for publication of this case report and any accompanying images. A copy of written consent is available for review by the Editor-in-Chief of this journal.


Teratomas of anterior mediastinum are rare tumors and are often slow growing, asymptomatic and detected incidentally on chest imaging. Results of surgical resection are very satisfactory.A 19-years old male presented with an asymptomatic cystic neck mass. X-ray and CT scan of chest and neck showed an extrathyroidal multi-septate, predominantly cystic neck mass, that was continuous with a solid intrathoracic mass extending up to the level of right atrium and which contained areas of calcification and cystic necrosis. The mediastinal structures did not show any features of compression or infiltration. Fine needle aspiration cytology from the neck mass was suggestive of a dermoid cyst. In view of the extent and uncertain pathological nature of the tumor, it was excised via a combined cervical and trans-sternal route. Histo-pathology of the resected specimen confirmed the diagnosis of a mature cystic teratoma. The patient made an uneventful recovery, and after five years of follow-up, he has been symptom free with no clinical or radiological evidence of recurrent disease. We discuss the role of imaging and the need for surgical treatment to avoid possible catastrophic complications in patients with cervical and mediastinal masses of uncertain histological nature.A mediastinal teratoma may rarely present as a cystic neck swelling due to its cephalad extension. This entity needs to be considered in cases where clinical and investigative work-up fail to provide a convincing clue to a primary neck pathology as cause of a cystic neck swelling. Teratomas of the anterior mediastinum account for 8–13% of tumors in this region -4. TheseWe report the case of a young adult male who presented with a cystic neck mass, due to degeneration of a cervical extension of a mature teratoma of the anterior mediastinum. This is an unusual presentation of an uncommon pathology.A 19 years old male presented with a rapidly progressing painless neck swelling of 3 months duration. There were no complaints of fever or weight loss or symptoms suggestive of compression of adjacent structures. The patient was afebrile and had a cystic, non-tender, non-translucent and smooth swelling occupying the whole of the neck anteriorly, extending from the hyoid bone to the suprasternal notch. The lower limit of the swelling could not be reached. There were no neck nodes or dilated tortuous neck or chest wall veins.99mTc-thyroid scan ruled out a thyroid lesion. Contrast enhanced CT of neck and mediastinum  declare that they have no competing interests.GA conceived the case report, contributed to collection of clinical details and writing, reviewing and finalization of the manuscript; DKK prepared the first draft besides contribution to collection of clinical details and illustrations. Both authors reviewed and finally 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.


Pneumobilia is mostly observed on computed tomography (CT) following surgical biliary-enteric anastomosis and biliary manipulation through endoscopic procedures. Although pneumobilia can be seen in pathological conditions, post-surgical pneumobilia is typically not associated with morbidity. In the present article, we report a case in which blunt abdominal trauma led to the evacuation of pre-existing pneumobilia causing pneumoperitoneum. Given that the subsequent laparotomy proved to be non-therapeutic, this report adds to the few cases of intra-peritoneal free air not helped by surgical intervention. Pneumoperitoneum in blunt abdominal trauma is typically associated with the rupture of a hollow viscus and remains a surgical emergency. We present a case of pneumoperitoneum that was not helped by therapeutic laparotomy due to its unique pathophysiology arising from hepatic fracture and pneumobilia.A 65-year-old woman presented as an unrestrained driver in a frontal impact motor vehicle crash. Upon arrival to our emergency department, her pulse was 100, blood pressure was 125/76 mmHg, Glasgow Coma Score was 15, and she complained of right upper quadrant pain. Physical examination revealed previous surgical scars, moderate abdominal distention, and right upper quadrant localized tenderness. The secondary survey was negative for additional examination findings.The patient’s past medical history was significant for chronic pancreatitis, which was refractory to medical management. Due to her severe pain, the patient underwent a cholecystectomy with a combined Roux-en-Y longitudinal pancreaticojejunostomy and hepaticojejunostomy, 14 months prior to the current presentation. As a result of this biliary-enteric anastomosis, the patient was noted to have asymptomatic pneumobilia . Additio3/mm3, hemoglobin 11.3 g/dL, Aspartate aminotransferase (AST) 1698 U/L, alanine transaminase (ALT) 783 U/L, alkaline phosphatase 376 U/L, total bilirubin 1.2 mg/dL, direct bilirubin 0.4 mg/dL, albumin 3.1 g/dL, and troponin 0.19 ng/mL. Her chest X-ray, head computerized tomography (CT), and cervical spine CT examinations were negative for any acute injuries. A CT of her abdomen and pelvis at this admission showed a large amount of pneumobilia, moderate amount of peritoneal free air around the liver, perihepatic free fluid, and an American Association for the Surgery of Trauma (AAST) grade II liver laceration[Laboratory examination revealed the following: WBC 15.3 × 10aceration. There wA midline incision incorporated the scar from the patient’s previous abdominal surgery. Extensive abdominal adhesions were encountered and care was taken not to injure the abdominal viscera while efficiently attempting to access the peritoneal cavity. Inferior to the umbilicus, a moderate amount of hemoperitoneum was encountered; however, no enteric contents were appreciated. There were no injuries on any intestinal surface to explain the free air. The Roux limb from the patient’s hepatico- and pancreaticojejunostomies was dissected free; however, it too had no evidence of injury or perforation. Upon examination of the right upper quadrant, pockets of free air surrounded the liver. A minimally bleeding liver laceration was encountered on the dome of the liver, just anterior to the inferior vena cava. Upon further examination, air was tracking under the liver capsule into the right lobe. When the liver was manipulated, small air bubbles arose from within the parenchyma and exited through the laceration. Hemostasis was achieved with FloSeal  and Surgicel . The abdomen was copiously irrigated and closed after leaving a 10 mm flat Jackson-Pratt drain in the upper right quadrant adjacent to the lateral aspect of the liver.Post-operatively, the patient recovered in the Trauma Intensive Care Unit for 3 days before transfer to a general surgical floor. Her post-injury course was complicated by the development of fevers, leukocytosis, and hypotension on post-operative day 9 which necessitated transfer back to the Intensive Care Unit. A CT of the abdomen/pelvis showed a 6.9 × 8.1 cm hepatic abscess . This waThe patient was seen in follow-up on several occasions and has continued to do well. A follow-up CT of the abdomen prior to drain removal demonstrated both healing of the liver laceration and resolution of the intra-hepatic infection .et al. simulated hepatic frontal deceleration injuries using cadaveric livers and found that rotation around the axis of the inferior vena cava and differential motion of the right and left lobes caused lacerations in the caval area.[et al. proposed that traumatic pneumobilia occurred due to increased duodenal intraluminal pressure forcing bowel gas through the sphincter of Oddi.[Pneumobilia is an uncommon radiographic finding and may occur secondary to surgical biliary-enteric anastomosis or biliary manipulation through endoscopic procedures. In the trauma literature, it has been described as a rare consequence of blunt abdominal injury.–4 In theval area. In congr of Oddi. Our patiet al. reported that non-surgical treatment of blunt liver trauma increased from 0% of cases in 1985–1989 to 65% in 1995–1999. The same period saw a consistent decline in both total and liver-related death rates.[While our patient did not receive any therapeutic benefit from our exploratory laparotomy, it was required given the free intraperitoneal air following traumatic mechanism and abdominal tenderness. From before the time of Pringle until the early 1990s, surgical intervention was considered almost always necessary in blunt hepatic trauma. Richardson et al. from Greece described a case of right-sided blunt thoracoabdominal trauma in which a hemidiaphragm rupture with hepatic herniation led to copious pneumoperitoneum as demonstrated by CT.[et al. reported a case in which a motorcycle accident resulted in urogenital lacerations and a handlebar hernia; atmospheric air dissected via the subcutaneous tissue from the lacerations to the hernia and into the peritoneal cavity.[et al. from the University of Toronto reported a series of 118 consecutive abdominal CT scans for blunt trauma in which seven patients had free abdominal air.[Although abdominal free air in blunt abdominal trauma is generally associated with hollow viscus perforation, other causes have been described. Polychronidis ed by CT. Ferrera ed by CT. Interestl cavity. Hamiltoninal air. Of theseP < 0.001).[As shown in Figures 1113< 0.001). These no< 0.001).–19 FortuTo our knowledge, this is the first case in the English literature of what appears to have been pneumoperitoneum originating from preexisting pneumobilia following a traumatic mechanism. Pneumobilia is an uncommon finding, although its prevalence may increase with biliary-enteric drainage procedures.The patient reported here presented with a rare cause of traumatic pneumoperitoneum that did not receive therapeutic benefit from surgical exploration. In retrospect, this scenario could have been managed non-operatively, however, this was not an appropriate option given the patient’s traumatic mechanism and degree of abdominal tenderness on presentation. In our current age of reliance on CT scan, we must continue to be mindful of a differential diagnosis in our utilization of highly sensitive imaging technology.


The management of concurrently occurring abdominal aortic aneurysm and another intra-abdominal pathology is controversial and represents a difficult management problem for the surgeon. Most surgeons are reluctant to perform a second non vascular procedure at the time of the aneurysm repair because of the risk of graft infection. Some evidence suggests that the one-stage elective surgical treatment in selected patients with concomitant abdominal aortic aneurysm and other pathologies; especially Gastro-Intestinal malignancies, is safe with superior cost effectiveness. However, there is a major dilemma in the management patients with large aneurysm which require an urgent repair and presented with concomitant pathologies that carry a high risk of sepsis. In this case report, we describe an unusual presentation of a large aneurysm with concomitant Acute Appendicitis where both needed an urgent surgical intervention. To our best knowledge, there has been no similar case report published in literature.A 66 years old Caucasian male presented with a dual pathology of large abdominal aortic aneurysm and acute appendicitis. The diagnosis was confirmed by Computerized Tomography scan of his abdomen. He underwent a 2-stage operation; open Appendicectomy followed by open repair of his aneurysm to avoid the risk of graft infection. He had an uneventful recovery period with a full return to normal life.The incidence of patients with abdominal aortic aneurysm and coexistent intra-abdominal surgical pathology is increasing, and the surgical strategy for those patients remains controversial. There are not enough studies that looked directly into the management of large abdominal aortic aneurysm which require an urgent repair and presented with concomitant pathologies that carry a high risk of sepsis. In such situations, simultaneous operations should be avoided because of the risk of prosthetic graft infection and priority should be given to the symptomatic or most life threatening condition. The second pathology should be dealt with as soon as possible; preferably within the same admission. More studies are needed to look into this issue; however, this would be rather difficult because of the uncommon and complex nature of such presentations. The management of concurrently occurring abdominal aortic aneurysm (AAA) and another intra-abdominal pathology is controversial. It is unclear whether to treat the aneurysm or the other pathology first, or both at the same time. Most clinicians agree that if one of the lesions is symptomatic it must be treated first . OperatiThere are some reports of performing that one-stage elective surgical treatment in selected patients with concomitant AAA with gastric or colorectal cancer, with safety and superior cost effectiveness . MoreoveA 66 years old Caucasian male presented with a 3 days history of nausea, vomiting, right lower abdominal pain and lower back pain. He had been complaining from upper abdominal discomfort for few months before his admission, this was treated as gastritis by his General PractitionerThe patient was known to have chronic obstructive air way disease . He was a smoker for years, who smoked about 25 cigarettes a day.On clinical examination he was fully orientated, alert and not pale. His vital signs chart showed a temperature of 36.6°C, pulse of 85 and blood pressure of 153/61. Abdominal examination revealed a tender expansile mass in the upper abdomen with peritonism in the right iliac fossa.His blood tests showed elevated WCC of 13000 and CRP of 64 with normal Hb and other parameters. Due to his abdominal signs, an immediate abdominal Computerized Tomography (CT) scan was arranged. This showed a 6.3 cm infra-renal AAA which was not leaking. In addition, there were radiological signs of acute appendicitis  antibiotics; Cefuroxime and Metronidazole (the first doses were given on induction). He had an uneventful post operative recovery; however, he was kept in the hospital for close observation and preparation of his AAA repair. The histopathology report showed features of acute appendicitis; with no other abnormality.The possibility of endovascular repair of the AAA was excluded after discussion with an experienced endovascular radiologist; due to the very short neck of the AAA.th post-operative day the patient had appeared to have recovered completely from the appendicectomy, and his inflammatory markers were back to normal limits. So he underwent an open AAA repair with a 20 mm straight Dacron graft. He had one dose of IV Cefuroxime (1.5 g) on induction. There were no signs of peritoneal soiling or infection in the abdominal cavity. The aneurysmal sac was closed securely over the graft to decrease the risk of infection. He had an uneventful post-operative recovery period and he was discharged home 6 days after his AAA repair. He had been seen in outpatient clinic for routine follow up at 3 months, 6 months and 1 year and no problem was reported.By the 10The incidence of patients with AAA and coexistent intra-abdominal surgical pathology is increasing, and the surgical strategy for those patients remains controversial . There iHowever; and to our best knowledge, there are not enough studies that looked directly into the management of large AAA which require an urgent repair and presented with concomitant pathologies that carry a high risk of sepsis. In such scenarios, simultaneous operations should be avoided because of the risk of prosthetic graft infection. This case raises awareness of a rare combination of acute appendicitis and a large tender AAA.AAA: Abdominal Aortic Aneurysm; CT: Computerized Tomography; IV: Intravenous; WCC: White Cell Count; CRP: C – Reactive Protein; Hb: Haemoglobin.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.AALS was involved in the major parts of writing the paper and performing the literature search. He was also involved actively in the patients care in the pre and post operative period. JM contributed to the paper writing and to the literature search. He had also performed the open Appendicectomy and assisted in the AAA repair. VB is the responsible vascular surgeon who had set the management plan and the team leader. All authors have read the manuscript and agreed to its contents.


Neuroendocrine carcinoma accounts for less than 5% of cancers of unknown primary site. When primary pancreatic cancer is diagnosed, 95% of cases are classified as aggressive adenocarcinoma and the remaining 5% of cases are caused by indolent behaving neuroendocrine carcinoma. We present a rare case of well-differentiated neuroendocrine carcinoma of the pancreas extending to the transverse colon, small bowel, stomach, and lymph nodes with metastasis to the caudate lobe of the liver. The patient had a two-year history of midepigastric abdominal pain that eventually sent him to the Emergency Department and after extensive tests we removed the entire tumor with margins free of growth. Included in this case report are many illustrations to show the severity and we further emphasize the importance of examining patients thoroughly especially when vague symptoms are chronic.A 58-year-old African American male presented to the Emergency Department by ambulance complaining of a constant, sharp, and worsening midepigastric pain that radiated posterior for approximately 30 minutes. The pain was perceived to be 10 out of 10 and was described as an inflated balloon that was squeezing from the inside out. The paramedics found the patient in his house hypotensive and lying in a right lateral decubitus position. The patient was given a bolus of normal saline and his blood pressure responded appropriately. Upon examination, the patient claimed to of had a two-year history of worsening mild midepigastric and left upper quadrant abdominal pain and was seen by his primary care provider whom prescribed him a proton pump inhibitor (PPI) which partially relieved some symptoms. In addition, the patient complained of being nauseated, feeling bloated after meals, having a history of bloody stools one week prior to admission, and increased pain after eating. He denied vomiting or early satiety. Vital signs on admission were stable.Past medical history included gastroesophageal reflux disease (GERD), hyperlipidemia and impaired fasting glucose. His medications consisted of a PPI, H2-blocker and simvastatin. The only past surgical history was an appendectomy. The patient was a truck driver for several years. He denied alcohol, drug use, and smoked approximately ½ packs of cigarettes a day for the past 40 years. He reported his two nieces having an unspecified type of cancer but no other known family history of cancer or heart diseases. Review of systems was negative except for gastrointestinal complaints as described above. The patient denied weight loss, fatigue, fever, shortness of breath, and/or chest pain. On physical examination, the abdomen was hard, guarding was noted, and there was direct and rebound tenderness noted in all four quadrants but worse in the epigastric area.On admission, the patient had a complete laboratory workup involving all systems. The abnormalities found mildly decreased were hemoglobin of 12.9 g/dl, hematocrit of 39.6%, albumin of 3.1 g/dl, and sodium of 132 mEq/l. The only abnormalities found mildly increased was the red blood distribution width of 16%, white blood cell count of 15,100 ul, corrected calcium of 11.32 mg/dl, glucose of 129 mg/dl, prothrombin time of 12.2 seconds, and the international ratio of 1.2 seconds. Stool guaiac was negative.Computed tomography (CT) of the abdomen and pelvis on admission revealed a large 4.7 centimeter (cm) lobulated hypodense mass in the region of the porta hepatis probably arising from the liver with the possibility of adjacent morphologic tubular lymph nodes or satellite lesions . ExtendiUtilizing CT guidance, biopsy of the large mass in the left upper quadrant was performed by Interventional Radiology on the same day of admission. Pathology results two days later showed a well-differentiated neuroendocrine carcinoma. The architecture revealed insular and trabecular morphologies, and the Ki-67 index was approximately 30% positive . MicroscOn hospital day number six the surgery service performed a diagnostic laparoscopy, exploratory laparotomy with distal pancreatectomy, splenectomy, partial gastrectomy, left colectomy, and resection of the caudate lobe of the liver . Pre andOverall, the surgery was successful. At the end of the procedure two 10 ml Jackson-Pratt drains were inserted into the lesser sac and left upper quadrant.Gross specimens were sent to pathology in three parts: frozen section of the periaortic lymph node measured 1.0 by 0.8 by 0.4 cm, the caudate lobe of the liver measured 4.0 by 3.5 by 2.5 cm, and the massive left upper quadrant tumor that consisted of an en bloc resection of the spleen, partial transverse colon, partial small bowel, pancreatic tail, partial distal stomach, adipose tissue, and lymph nodes. The spleen measured 12 by 7.5 by 2.5 cm, the colon measured 33 cm in length and 4 cm in diameter, the pancreatic tail measured 12 by 8 by 6 cm, a portion of the small bowel measured 15 cm in length and about 3 cm in diameter, and a small portion of the distal stomach measured 9 by 6.5 cm . The masMicroscopic sections were analyzed and found the perioarotic lymph node to be negative for metastatic carcinoma. Microscopic sections of the caudate lobe of the liver showed metastatic well-differentiated neuroendocrine carcinoma . SectionStreptococcus pneumoniae, Haemophilus influenza and Neisseria meningitides were administered. Twenty days post-admission the patient was discharged from the hospital with instructions not to lift more than 15 pounds, and to follow-up with surgery in one week.Overall, the patient tolerated the procedure and hospital stay well. During the course of hospitalization his pain was well controlled with morphine, and his glucose was well controlled after being weaned off of an insulin drip and placed on a sliding scale. Vaccinations for encapsulated organisms including pneumococcal, When the initial work-up, including physical examination, laboratory and radiographic studies fails to identify the primary site of a tumor with symptoms of referable to a metastatic site, light microscopic evaluation of biopsy material is necessary. Cancer of unknown primary site is a common entity and based on five histological categories: adenocarcinoma, poorly-differentiated carcinoma, poorly-differentiated neoplasm, squamous cell carcinoma, and neuroendocrine carcinoma . NeuroenNeuroendocrine carcinomas falls into three broad categories: poorly, moderately and well-differentiated neuroendocrine carcinomas. Poorly-differentiated neuroendocrine carcinomas usually have a high grade malignancy and are characteristically small cell undifferentiated or morphologically anaplastic by light microscopy . ModeratWith well-differentiated neuroendocrine carcinomas encompassing such a broad spectrum of neoplasms it is pertinent to further classify them depending on whether they have a neural or an epithelial origin. Paragangliomas are of neural origin and the epithelial group can be subdivided into the remaining types of neuroendocrine carcinomas . In our The majority of well-differentiated neuroendocrine carcinomas involve the gastrointestinal tract, and therefore are referred to as gastroenteropancreatic neuroendocrine carcinomas, however neuroendocrine carcinomas have been found to occur in almost every organ in the body . GastroePancreatic endocrine tumors or islet cell tumors may be nonfunctional or functional depending on whether or not the tumor was secreting excessive amounts bioactive substances such as insulin, gastrin, somatostatin, vasoactive intestinal peptide, or glucagon and further causing associated symptoms . InitialWe looked into reasons of why metastasis occurred in the caudate lobe of the liver and not the other three lobes. Interesting, the caudate lobe is the only part of the liver that has hepatic venous drainage encompassing a few sizeable and several smaller branches that flow directly into the inferior vena cava . WhetherAlthough there is abundant literature on neuroendocrine carcinomas ,7,9, to Well-differentiated neuroendocrine carcinomas have variable and most often indolent biologic behaviors, and microscopically show uniform and small cells organized in organoid or trabecular architecture. Biopsy and immunohistology is necessary to distinguish the various types of neuroendocrine carcinomas whether they are epithelial versus neural origin or poor versus well-differentiated and additional laboratory workup should be done to differentiate between functional and nonfunctional types of pancreatic endocrine tumors which may be associated with neuroendocrine syndromes. More importantly, neuroendocrine carcinomas of all kind have been capable of local recurrence and distant metastasis, and therefore, close clinical correlation and appropriate treatment are important factors attributed to improving the overall survival rate.


Bizarre parosteal osteochondromatous proliferation, otherwise known as Nora’s lesion, is surface tumor of bone with high probability of local recurrence. The author reports a case of Nora’s lesion of the proximal phalanx of the second toe, successfully managed by en bloc excision of the swelling. At four-year follow-up there was no evidence of recurrence. Bizarre parosteal osteochondromatous proliferation (BPOP) is a rare tumor affecting proximal and middle phalangs of the hands and feet.124169nd web space , BPOP is a benign surface tumor from the small tubular bones of the hands and feet.1011First described by Nora Mostly a tumor of the middle and proximal phalanges of the hand,236912410BPOP arise directly from the cortical surface of the bones, as if they are “stuck” on the periosteum.129These are single or multiloculated lesions, macroscopically, with a diameter ranging from 05-40 mm.129There is bizarre intermingling of cartilage, bone and fibrous tissue. The cartilage is composed of hyper cellular chondrocytes, some of which are binucleated. There are no atypical fibrous spindle cells, thus differentiating BPOP from parosteal osteosarcoma.A differential diagnosis of parosteal osteosarcoma, osteochondroma, myositis ossificans, and benign florid periostitis is considered. Initial diagnosis in many reports is osteochondroma because of the parosteal location, cartilage cap and histological presence of chondrocytes.91It is a highly aggressive tumor with local recurrence reported as high as 50%.1368