In a 53-year-old man with superior vena cava obstruction syndrome an atypical carcinoid of the thymus (During his first year of follow-up a Somatostatin Receptor Scintigraphy was recommended. An area of abnormal concentration of the radiopharmaceutical was revealed in the mediastinum at this time.A thorough understanding of the mechanisms of the radiopharmaceutical uptake and of the various clinical settings in which uptake can occur are essential for a proper evaluation of the scintigraphic findings and result in the optimal use of this valuable modality.The literature review provides an overview of this rare type of tumor and insight into the clinical significance of Somatostatin Receptor Scintigraphy. Primary neuroendocrine tumors of the thymus, previously known as thymic carcinoids, are unusual tumors that account for less than 5% of all anterior mediastinal neoplasms. They affect patients over a wide age range, with the median age being 43 years. Men are more frequently affected than women, with a male to female ratio of 3:1 [Clinically, these tumors manifest in one of four ways: 1) as an incidental finding on routine chest radiography, 2) with symptoms of thoracic structure displacement or compression, 3) with symptoms related to an associated endocrinopathy or 4) with symptoms and signs relating to a distant metastasis, most commonly in the liver, lung, pancreas, pleura or bone. At least 20% of affected patients have metastatic disease at presentation, with the frequency of extra-thoracic metastasis being 20% – 30 % [Thoracic CT and MRI and nuclear medicine imaging, including Somatostatin Receptor Scintigraphy (SRS), meta-iodobenzylguanidine (MIBG) scintigraphy with I-123 or I-131 and bone scan, are useful studies in the evaluation of these patients . Radio lHowever, pentetreotide has also been shown to concentrate in primary and metastatic thymic tumors, including thymoma, thymic carcinoma, and TNET -9 where 111In-Pentetreotide has yielded tumor growth inhibition in animal models and in patients with somatostatin receptor positive tumors, this therefore could be a clinical application in this kind of tumor [SRS also may have a role in the follow-up of these patients . Anotherof tumor .A 53-year-old man noticed some swelling of his face. A chest x-ray showed a large mass projecting over the right lung field fig . A CT scHe underwent excision of the tumor, which required resection of the superior vena cava and insertion of a graft, resection of the right phrenic nerve and plication of the diaphragm.The finding at surgery was an enormous spherical tumor with an extremely vascular capsule due to the venous collaterals. The tumor was 20 cm from pole to pole and 15 cm in diameter. On the left it bulged into the left pleural space, displacing the phrenic nerve laterally. Superiorly it obliterated the left innominate vein and extend into the strap muscles. There was an enlarged lymph node. On the right it had obliterated the confluence of the innominate veins and the first centimeter or so of the superior vena cava. It enclosed the phrenic nerve over its complete length and was adherent to the medial aspects of the upper and middle lobes of the right lung. There was some thickening of the visceral pleura in this area and there appeared to be some superficial infiltration. Posteriorly it was adherent to the pericardium and there was some thickening of the ascending aorta, consistent with longstanding pressure effects. There was a small bloody pericardial effusion and a rather large right pleural effusion. There was no evidence of any pleural deposits or intrapericardial deposits.neuroendocrine carcinoma of intermediate grade 2).The histology of the resection specimen showed a tumor weighing 2.1 kg. The tumor appeared encapsulated with an irregular surface. The tumor invaded the surrounding fat and pleura. There was infiltration into the underlying lung tissue lymphatics. There was some residual thymic tissue within the surrounding fat. Metastatic deposits were found in the right hilar lymph node and the node removed from the left cervical horn of the thymus. The tumor was classified as an atypical carcinoid of the thymus . In these cases total blood count and ESR remain normal.Pentetreotide is accumulated in surgical trauma because of the healing process in which macrophages are recruited. This fact makes a SRS unable to distinguish the disease from the healing process. This applies for two months after the surgery.In the case of our patient, the SRS was performed one year after the surgery, so this possibility should be excluded, although the radiotherapy, which our patient had undergone, may have prolonged the healing period.Two years after the SRS, the patient is free of disease according to his clinical condition, biochemical data and imaging modalities, including a MRI scan. A second SRS that could clarify our finding was not performed since the MRI scan was negative, and a further study of our finding was not possible. Thus, the SRS finding has to be interpreted, based on the above follow up data, as a radiopharmaceutical accumulation at the site of an inflammatory process and not as a mass remnant or a recurrence of the disease.To our best knowledge, 28 cases of TNET in which SRS was performed have been reported to date in the literature table  5,7-10,,10,15-18Since CT and MRI are more accurate than SRS in the detection of TNET, the main role of pre-operative SRS is in the planning of follow up and possible therapeutic approaches for these patients. The post-operative SRS should be performed at the right time, so that the healing process is over and a possible remnant or recurrence can be detected. All the radiopharmaceutical accumulation mechanisms have to be taken into account in order to proceed to a valid differential diagnosis and give precise information to the clinician.The author(s) declare that they have no competing interests.


Idiopathic sclerosing encapsulating peritonitis  is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations.A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up.Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management. The idiopathic form  was established, due to intraoperative findings and by ruling-out any other condition explaining the patient's pathology. Postoperatively, a forgotten small bowel follow-through performed elsewhere in 2000 was brought to our attention by the patient, showing "ileal loops bunched and confined in the lower abdomen and pelvis apparently due to adhesions" . He had no surgical or other medical history. On examination, he was in distress, but afebrile and haemodynamically stable. His abdomen was distended but non-tender, with increased bowel sounds in pitch and frequency. No palpable abdominal mass or organomegaly and no external hernias were present. Laboratory blood analyses were within normal limits. Plain abdominal X-ray showed few air-fluid levels centrally located, without free intraperitoneal gas. Ultrasound of the abdomen did not reveal any abnormalities. Contrast-enhanced abdomen computed tomography, performed on the same day, confirmed the diagnosis of small bowel ileus without providing any diagnostic clues. The patient was admitted to the general surgical ward and ileus resolved by day 3 conservatively. Endoscopy of upper and lower gastrointestinal tract and biopsies from the duodenum and colon provided normal findings. Although symptoms of obstruction had abated, the history of multiple relapses, the patient's complaints for poor quality of life and multiple admissions, as well as the undefined origin of the underlying pathology led to an exploratory laparotomy on day 7. On surgery, a fibrous capsule covering all the abdominal viscera was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. The liver, stomach, appendix, right and left colon, as well as the sigmoid, were also covered and the greater omentum looked hypoplastic and encased in fibrous tissue  declare that they have no competing interests.CS and IK designed the case report, researched the article and drafted the manuscript.CS, SV, GR, GG, NL, GP carried out the surgery and were involved in all investigations.All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Leiomyosarcoma of the inferior vena cava is a rare tumor that presents in an insidious manner with non-specific symptoms. Given its rarity, there are no consensus guidelines to its management. The aim of this study was to report the clinical experience in the management of patients presenting to our institution during a 12 year period.Four patients with leiomyosarcomas of the inferior vena cava were managed at our institution during the period reviewed. Patient details were identified through a search of the pathology department computerized database, and case notes were retrospectively reviewed to obtain details of presentation and management.There were 3 females and 1 male with a mean age of 59 years. All tumors were identified within 2 months of first symptoms. Three of the 4 had localized tumors whilst 1 patient had lung metastases at presentation. The three patients with resectable tumors underwent radical surgical excision of the tumor, and two patients had postoperative radiotherapy. One patient died of recurrence at 7 months, and another at 30 months. The third patient is currently well and disease free at 16 months. The fourth patient with metastatic disease was treated with chemotherapy alone and survived 36 months.Leiomyosarcoma of the inferior vena cava is an uncommon tumor that presents with non-specific symptoms. At the time of presentation, tumors are usually large and resection is challenging but probably offers the best opportunity for long-term survival. Leiomyosarcomas of the inferior vena cava (IVC) are rare as illustrated by the fact that an International IVC Leiomyosarcoma registry established in 1992 collected, by means of literature review and personal communication, only 218 cases by the time of publication of its first report in 1996 .en-bloc resection with a view of achieving clear margins. However, in spite of an overall 50% 5-year survival, half of the tumors recur either locally or metastasize [IVC Leiomyosarcomas are classified anatomically according to their relationship to the liver and renal vessels: lower or Segment I ; middle or Segment II  and upper or Segment III (suprahepatic IVC) . The manastasize ,3.We present our experience with four cases of IVC leiomyosarcoma managed at our institution between January 1993 and December 2004.From January 1993 to December 2004, 4 patients were diagnosed with leiomyosarcomas of the IVC. There were 3 females and 1 male with a mean age of 59 years (range: 57–61 years). The demographic details and symptoms at presentation are summarized in Table Clinical data was obtained from review of the patient notes, whilst radiological and histological results were obtained from prospectively maintained departmental computerized databases.All 4 patients were symptomatic at presentation although there was significant variation in symptoms between patients Table . PatientThe diagnosis of a caval tumor was made on an ultrasound scan (USS) in 3 of the 4 cases and on computed tomography (CT) in the fourth. CT scans were performed to further characterize the origin of the tumors, demonstrate any contiguous invasion, and to exclude extra-abdominal metastases Figure . Patienten-bloc in all 3 cases. Patient 3 also underwent a thoracotomy and excision of a tumor embolus from the right atrium. In this patient, the point of origin of the tumor on the IVC was narrow and there was enough mobility to primarily close the IVC. Whilst in the remaining 2 cases, the tumor had a broad base. In these cases, the tumor was excised with a generous cuff of normal IVC wall, the defect was patched with a portion of long saphenous vein in patient 4  undergoing resection received postoperative radiotherapy.Patient 1 had a variety of chemotherapy protocols as well as palliative therapy, and survived 36 months. Disease recurrence occurred in patients 2 and 3. Patient 3 presented with widespread metastases and died at 7 months, whilst patient 2 developed disseminated disease 2 years after his surgery and died at 30 months post resection. Patient 4 is alive and disease free at 16 months post surgery.The first reported case of leiomyosarcoma of the inferior vena cava was documented in the German literature by Perl  in 1871 Leiomyosarcomas of the IVC are rare malignant tumors originating from the smooth muscle cells of the media of the cava and demonstrate three growth patterns – extraluminal, intraluminal or both . The tumThis tumor commonly presents with a few months history of vague upper abdominal symptoms including pain, anorexia and vomiting; but may also present with more distinct signs such as an abdominal mass, deep venous thrombosis due to IVC occlusion, or less commonly as an acute Budd-Chiari Syndrome. However metastasis may be the first presentation of the tumor such as in case 1.Given the rarity of the tumor, and in the absence of symptoms and signs of IVC compression, the identification of an IVC leiomyosarcoma is often an unexpected finding on imaging with USS or CT. In 3 of the 4 cases in this series, the tumor was identified on USS and a CT scan was performed to determine the origin, evaluate any local invasion of the tumor and exclude pulmonary metastases. In all 3 cases undergoing surgery, a diagnosis of leiomyosarcoma was suspected preoperatively. Other imaging modalities that aid the diagnosis of IVC sarcomas include vena cavography, which allows biopsy of the tumor. Nevertheless, in some cases, the precise origin of the tumor cannot be confirmed preoperatively. Furthermore, percutaneous biopsy to confirm histology of tumors in this region is often not possible due to the anatomical location of the IVC. Thus the surgeon is often faced with a tumor of uncertain type and origin at laparotomy.en-bloc surgical excision with a view to obtaining negative resection margins. A number of techniques have been reported for dealing with the IVC following excision including IVC ligation; primary repair of IVC; patching of IVC and interposition grafting with a synthetic conduit. In the largest single institute series of 25 patients over a 20 year period, Hollenbeck et al [Whilst there is no strong evidence base for the management of IVC leiomyosarcomas, the treatment of choice based on the available literature is radical ck et al , concludWhen radical surgery is performed, 5- and 10-year results of 49.4% and 29.5% respectively have been reported . Tumors The role of chemotherapy and radiotherapy in the treatment of IVC leiomyosarcoma, either as a primary modality or as an adjuvant, remains to be proven. In this series, different chemotherapy protocols provided good palliation for a patient presenting with advanced disease. In the remaining three patients, only two underwent radiotherapy with a view to obtaining local control and prevention of local recurrence.Whilst IVC leiomyosarcomas are certainly rare, long-term survival depends on obtaining an early diagnosis and performing extensive surgery. The more frequent use of CT in the investigation of patients with abdominal symptoms may aid the earlier diagnosis of these tumors.The author(s) declare that they have no competing interests.SA: writing the article and literature reviewGJM: writing the discussion and searching the literatureDS: operative procedure and care of one of the studied casesIW: reviewing the article and operative procedure and care of one of the studied casesAGD: contributing the pathological aspects of the manuscriptMCP: overall supervision of the article writing


Leiomyomas of the deep soft tissue are quite uncommon and occur even more rarely in upper extremity.A 32-year old manual laborer man presented with a two-year history of numbness, tingling and burning pain in the palmar surface of the left hand and fingers. His medical history was unremarkable and no trauma episode was reported. According to the clinical examination and the result of median nerve conduction study (NCS) the diagnosis of carpal tunnel syndrome was established. Operative release of the transverse carpal ligament was subsequently performed but the patient experienced only temporary relief of his symptoms. MRI examination revealed a deep palmary located mass with well-defined margins and ovoid shape. Intraoperatively, the tumor was in continuity with the flexor digitorum superficialis tendon of the middle finger causing substantial compression to median nerve. Histopathological findings of the resected mass were consistent with leiomyoma. After two years the patient was pain-free without signs of tumor recurrence.Despite the fact that reports on deep soft tissue leiomyoma are exceptional, this tumor had to be considered as differential diagnosis in painful non-traumatic hand syndromes especially in young patients. Leiomyomas are uncommon smooth muscle tumors and they account for 4.4% of all benign soft tissue neoplasms . They arWe describe an unusual case of secondary carpal tunnel syndrome due to a misdiagnosed, deep-seated, soft tissue leiomyoma. Surgical excision led to uneventful clinical result and resolution of symptoms.A 32-year old manual laborer, right-hand-dominant man presented in the Orthopaedic Outpatient Clinic of the local hospital with a two-year history of numbness and tingling sensation in the palmar surface of left hand and fingers. During the last six months the patient reported also burning pain in the same area – causing him to wake up frequently during the nightime hours – and progressive inability to perform his regular occupation. No episode of trauma or other medical problems were mentioned. All the symptoms were attributed to carpal tunnel syndrome and the diagnosis was confirmed with median nerve conduction study (NCS). Through a classical open palmar approach subsequent release of the transverse carpal ligament was performed. According to surgeon's operative report, there wasn't any reference for any pathology in the dissected area except from the severely compressed median nerve. However, postoperatively, the patient experienced only partial and temporary relief of his symptoms without improvement of hand function.Three months after the surgical procedure, the patient was referred to our department for further investigation. On physical examination, the palmar aspect of the left hand and the area of the previous incision were tender to palpation and swollen while a slight atrophy of the thenar muscles was apparent. There was a sensory loss in the area innervated from the median nerve as compared with the controlateral hand and Tinel's sign was evoked over the median nerve at the wrist. The patient had decreased grip strength while passive flexion and extension of fingers were painful. As a result, additional investigation was deemed necessary and magnetic resonance imaging (MRI) was selected for delineating any potential pathology in the carpal tunnel or the surrounding tissues. MRI showed a misdiagnosed, volarly placed soft tissue mass with well-defined margins and an ovoid shape which was in direct contact with the flexor tendons Figure .Because of the persistent symptoms and the radiographic abnormalities, surgical exploration and tumor resection were scheduled. Under axillary block a well-circumscribed, encapsulated mass in the deep palmar tissues was identified causing significant compression of the median nerve. The tumor was adhered to the third flexor digitorum tendon superficialis and it was moving within the tendon during flexion or extension of the middle finger Figure  See add. No vascAt the two-year follow-up evaluation, the patient showed no evidence of recurrence of the mass and no pain or sensory dysfunction. He had a significant improvement in hand grip strength and he returned to his previous occupation.Leiomyomas are benign soft tissue tumors that occur wherever smooth muscle is present. They are classified into three groups: cutaneous leiomyomas that arise from the erector pili muscle; vascular leiomyomas that arise from smooth muscle of the vein and leiomyomas of deep soft tissues . In handDeep soft tissue leiomyomas are uncommon and only sporadic cases have been reported in the literature -3,8,9. MThe tumor may not be easily recognised until become painful and it is rarely diagnosed before surgery as imaging techniques, including MRI, are not specific for tumor diagnosis . LipomasIn the herein case, patient symptoms were attributed to idiopathic carpal tunnel syndrome and no further investigation was taken place. Even during the open dissection of the carpal tunnel, no pathology around median nerve was suspected and no meticulous observation of the area was performed. As space occupying lesions were found only in 5.5% of the patients with unilateral carpal tunnel syndrome routine imaging for the investigation of unilateral carpal tunnel syndrome is not essential . HoweverTypically, leiomyoma lacks mitotic activity altogether and the usual treatment is simple excision of the tumor due to its benign character. Billings et al  were repThe case is presented due to its rarity and the risk of tumor misdiagnosis. Deep soft tissue leiomyoma is an uncommon tumor and its appearance in upper limb or hand constitutes a rare issue. The potential of tumor connection to flexor tendons and the development of compression neuropathy make the diagnosis even more difficult as the mass may be mistaken for a giant cell tumor of the tendon sheath or a ganglion cyst. Further awareness of the existence of atypical leiomyomas in hand among clinicians may lead to better evaluation and understanding of similar soft tissue lesions.The author(s) declare that they have no competing interests.BC: Preparation and submission of manuscript.CD: Data collection, preparation of figures and video.Both authors have read the final manuscript and agree to its publication.Adhesion of tumor to the third flexor digitorum tendon superficialis.Click here for file


Goblet cell carcinoid (GCC) of the appendix is a rare neoplasm that share histological features of both adenocarcinoma and carcinoid tumor. While its malignant potential remains unclear, GCC's are more aggressive than conventional carcinoid. The clinical presentations of this neoplasm are also varied. This review summarizes the published literature on GCC of the appendix. The focus is on its diagnosis, histopathological aspects, clinical manifestations, and management.Published studies in the English language between 1966 to 2004 were identified through Medline keyword search utilizing terms "goblet cell carcinoid," "adenocarcinoid", "mucinous carcinoid" and "crypt cell carcinoma" of the appendix.Based on the review of 57 published papers encompassing nearly 600 diagnosed patients, the mean age of presentation for GCC of the appendix was 58.89 years with equal representation in both males and females. Accurate diagnosis of this neoplasm requires astute observations within an acutely inflamed appendix as this neoplasm has a prominent pattern of submucosal growth and usually lacks the formation of a well-defined tumor mass. The mesoappendix was involved in 21.64% followed by perineural involvement in 2.06%. The most common clinical presentations in order of frequency were acute appendicitis in 22.5%; asymptomatic in 5.4%; non-localized abdominal pain in 5.15% and an appendicular mass in 3.09%. The most common surgical treatment of choice was appendectomy with right hemicolectomy in 34.70% followed by simple appendectomy in 24.57%. Concomitant distant metastasis at diagnosis was present in 11.16% of patients with the ovaries being the most common site in 3.60% followed by disseminated abdominal carcinomatosis in 1.03%. Local lymph node involvement was seen in 8.76% of patients at the time of diagnosis. The reported 5-year survival ranges from 60 % to 84%. GCC's of the appendix remains a neoplasm of unpredictable biological behavior and thus warrants lifelong surveillance for recurrence of the disease upon diagnosis and successful surgical extirpation.GCC of the appendix is a rare neoplasm. Due to its wide range of presentation, this tumor should be considered as a possible diagnosis in many varied situations leading to abdominal surgery. Histopathological features such as increased number of Paneth cells, increased amount of mucin secretion and presence of pancreatic polypeptide may predict a more aggressive behavior. The advocated plan of management recommended for patients with tumors that involve the adjacent caecum or with high-grade tumors with histological features such as an increased mitotic rate involve initial appendectomy with completion right hemicolectomy due to the high possibility of local recurrence with intraperitoneal seeding prior to lymph node involvement and a 20% risk of metastatic behavior. In female patients with GCC of the appendix regardless of age, bilateral salpingo-oophorectomy is advocated. In cases with obvious spread of the disease chemotherapy, mostly with 5-FU and leucovorin is advised. Cytoreductive surgery with adjuvant intraperitoneal chemotherapy can offer improved survival in cases with advanced peritoneal dissemination. Cancer of the appendix is an uncommon disease . Appendiet al described the first report of GCC in 1974 [Subbuswamy  in 1974 . This ty in 1974 ,14 and h in 1974 . The his in 1974 . Some au in 1974 . Yet, in in 1974 . The tum in 1974 . Anatomi in 1974 . Preoper in 1974 . The GCCet al described 3 unusual tumors of appendix with common features of 1) association of nests of entrochromafin cells with mucus secreting glandular structures; 2) integrity of the appendiceal mucosa, and 3) an infiltrative pattern similar to carcinoid tumors but with the propensity to invade nerves [et al in 1974 [et al reported microscopical demonstration of the cells of the other known cases with GCC of the appendix [et al reported the first case of appendico-vesical fistula due to appendix abscess with GCC in 1976 [et al reported 39 cases of GCC and revealed more aspects about this neoplasm [et al described the origin of these cells as being endodermal in origin and explained that these cells arise from crypt base stem cells [et al explained this neoplasm as amphicrine (endo-exocrine) neoplasia [Appendiceal tumors that share the histological characteristics of carcinoids and adenocarcinomas have been recognized since the 1960s, with early reports by Gibbs, Bates and Butler, Rosai and Rodriguez . In 1969e nerves . In 1974e nerves  but the  in 1974 . After tappendix . DiPaola in 1976 . In 1978neoplasm . In 1979eoplasia ,7,24. Duet al explained that the mean age for GCC is 58.8 years, whereas that for carcinoid tumor is 35.9 years [GCC accounts for less than 5% of primary tumors of the appendix . Based o.9 years . The mea.9 years . The mea.9 years . In addi.9 years . In cont.9 years .GCCs of the appendix are amphicrine tumors arising from a pluripotent cell with divergent neuroendocrine and mucinous differentiation and with dysregulation of the cell cycle . There iIn conclusion, based on the review of all published papers for nearly 600 diagnosed patients, the mean age for diagnosing GCC was 58.89 years with equal representation in both genders with male to female ratio of 1:0.84. GCC of the appendix occurs later in life in comparison to conventional carcinoid. The etiopathogenic factor that most authors agree is the occurrence of p53 mutations and this is confirmed in most of the reported analyzed cases.Although GCC was previously considered a variant of carcinoid tumor, current evidence suggests that GCC is a distinct tumor with a histogenesis different from that of typical carcinoid . AppendiGCCs represent a composite biphasic neoplasm of two morphologically differentiated cells, each with a common endodermal embryogenic origin . In earlThe two types of granules (neuroendocrine and mucinous) that normally exist in GCC cells are not mixed with one another in the same cell and are completely separate ,21 thougBecause GCC is considered to be derived from crypt cells, there are other immunohistochemical techniques for their diagnosis including Cytokeratin 20 (CK20), CK7 (with the positivity of CK20 more than CK7), neuron-specific enolase (NSE), chromogranin A, serotonin, lysozyme, PGP 9.5, IgA and vimentin ,27,33,34et al mentioned that SC-, lyzozyme- and CEA-reactivity was strictly limited to the exocrine tumor areas and was not found in endocrine cells of the normal mucosa [Carcinoembryonic antigen (CEA) is one of the markers that are diffusely positive on the plasma membrane of tumor cells in GCC . Höfler l mucosa . In one l mucosa . Tumor cl mucosa . Most cel mucosa  and antil mucosa . Sometiml mucosa .The characteristics of GCCs that somewhat simulate ordinary carcinoids are: 1) intramural position; 2) continuity with basiglandular cells of the mucous membrane; and 3) generally well-differentiated nature as well as the presence of goblet cells, argentaffin or argyrophil cells and Paneth cells in varying proportions . Paneth In summary, GCCs are derived from undifferentiated stem cells and are different from typical carcinoids that originate from endocrine cells in the mucosal stroma  and the et al explained features of GCC as 1) isomorphous tumor structure, 2) the presence of adenoid tumor cell groups in the intestinal wall, 3) intact mucosa overlying the tumor, and 4) the presence of endocrine cells [When microscopically evaluated, atypical cells show a prominent submucosal growth pattern with diffuse infiltration from the submucosal layer to the subserosal layer forming a microlumen structure. The growth pattern of atypical cells is within the lamina propria and extends through the muscularis propria into the subsequent serosa without destroying the appendiceal structure . Generalne cells .Macroscopically, in most cases, there is no well-defined mass. There maybe areas of indurations in the wall of the appendix or stenosis of the lumen with diffuse fibrous thickening of the appendix, which draw attention to the possibility of tumor ,40. The Regarding the anatomical location of the tumor, one study mentioned that the tumor was present in 70% in the distal segment of the appendix . RegardiThe majority of the metastasizing GCCs extend to the serosa of the appendix though serosal involvement is not necessarily an ominous finding . MetastaReview of all diagnosed patients has shown that the mesoappendix was involved in 21.64% of cases and perineural involvement as the second most common tumoral extension was seen in 2.06% of all patients.Clinical diagnosis of GCC is seldom made preoperatively. Most of the patients present with signs and symptoms of an acute appendicitis due to luminal obstruction ,6. The tet al reported one GCC with mucosal ulceration [In those patients where the tumor metastasizes to the ovaries, half of them primarily presented as an ovarian mass . Some raceration . Althougceration . Carcinoceration . Sometimceration . A very ceration .Review of the literature has shown that the most common clinical presentations for GCC of the appendix in order of frequency are as follows: acute appendicitis 22.5%, asymptomatic 5.4%, non-localized abdominal pain 5.15% and abdominal mass in 3.09%.GCC is included under the general heading of mixed (composite) glandular-endocrine-cell carcinomas . The diaGCC should be sharply distinguished from ordinary carcinoid tumors of the appendix, since they could behave biologically like carcinoma . The modThe resemblance of some areas in GCCs to Brunner's glands has been noted. However, the mucin in the latter is essentially neutral, whereas it is predominantly acid in GCC of the appendix . In addiIn most reports of metastasizing GCCs, the patients present with spread beyond the appendix at initial diagnosis . In seveThe white blood cell count is generally elevated with predominantly polymorphonuclear leukocytes and a "shift to the left," especially in patients presenting with acute symptoms ,20. HoweX-ray of the abdomen shows dilated small bowel loops with fluid levels when obstruction occurs . Cross-set al emphasized that in patients with no concomitant caecal involvement and low-grade tumor's histology; a simple appendectomy is enough [The site of the tumor within the appendix is not important for the choice of treatment . In genes enough .Some studies recommend that because GCCs are among the semi high-grade malignant carcinoids with unpredictable behavior, in addition to simple appendectomy a right hemicolectomy should also be performed ,42. The In addition to right hemicolectomy, some authors also advocate bilateral oophorectomy in female patients because of the possibility of ovarian metastasis ,48. ConvEven though the GCC of the appendix is not a complete high-grade malignant tumor, it can recur and metastasize so complete removal is imperative . It is eReview of all reported GCCs showed that simple appendectomy was performed in nearly 24.57% of cases especially in the early reports. The most common surgical treatment of choice was appendectomy with right hemicolectomy (RHC) especially in the recent reports (34.7%). Oophorectomy was also performed in nearly 11.39% of female patients. In conclusion, because of the moderately aggressive behavior of the tumor, it is recommended that in all patients in addition to appendectomy a right hemicolectomy is also performed. As the mean age of the patients is over 50 and ovaries are the most common sites of metastasis, and bilateral involvement of the ovaries is the commonest metastatic behavior, bilateral salpingo-oophorectomy with total abdominal hysterectomy (TAH-BSO) is also advocated in selected female patients. Peritoneal carcinomatosis from GCC origin is as invasive as peritoneal surface malignancy from colorectal adenocarcinoma. Patients in whom complete or near-complete surgical removal is possible should be considered for cytoreduction in combination with intraperitoneal chemotherapy .et al reported the first case of GCC on peritoneal fluid cytology in a woman with a prior history of carcinoid tumor of the appendix and the patient underwent 11 courses of Adriamycin and alpha-interferon with unknown documented follow-up [As GCC's have this capacity to seed diffusely into the peritoneal cavity, innovative chemotherapy regimens similar to gastric cancer chemotherapy is advocated though in one study chemotherapy was administered without successful response . One stuollow-up . A regimollow-up . There iollow-up . In geneollow-up . In one ollow-up . A recenollow-up . A summaEven though it was not prescribed routinely, most protocols include 5-FU. As peritoneal seeding can occur before lymph node metastasis and there is proven advantage of chemotherapy in metastatic patients, 5-FU is therefore highly recommended in all GCC patients. Radiotherapy was only ordered in 4 cases without any specific consequence.Metastasis occurs in nearly 20% of the cases . In geneIn-labeled octreotide scintigraphy is the most sensitive imaging modality in the diagnosis and staging of metastatic disease. Plasma chromogranin A is the most important blood marker available with levels being raised in 80–100 percent of patients with neuroendocrine tumors including GCC. Chromogranin A levels correspond to tumor load and levels above 5000 μg/l predict a poor outcome. Therefore, all patients with GCCs warrant investigation with plasma chromogranin A concentration, 24-h urinary levels of 5-HIAA, CT and In-labeled octreotide scintigraphy . Where aReview of the literature has shown that 11.2% of the patients were diagnosed with concomitant distant metastasis with ovaries being the most common site (3.60%) followed by abdominal carcinomatosis (1.03%). In addition, local lymph node involvement was seen in 8.7% of patients at the time of diagnosis. After surgical treatment, metastasis occurred in 4.1% of patients most commonly with abdominal carcinomatosis in 1.37% followed by ovaries in 0.85%. In conclusion, when GCC is suspected, both the ovaries and the peritoneum should be examined both during surgical extirpation and during follow up.The prognosis of these tumors is estimated to be somewhere between carcinoids and well-differentiated adenocarcinomas of the appendix . PrognosHistological features such as nuclear pleomorphism and mitotic activity show no significant correlation with staging or prognosis . On the As a rule, survival for GCC of appendix is not significantly worse than that for patients with malignant carcinoid when adjusted for age and extent of disease at presentation, and is similar to survival after treatment of a low-risk adenocarcinoma . One repGenerally, the behavior of GCC is unpredictable and cannot be anticipated from the tumor's diameter, as in the case with carcinoid tumors . AverageGCC of the appendix is a rare neoplasm that has varied clinical presentations ranging from an asymptomatic patient that is recognized during investigations for another problem, to acute appendicitis, the most common presentation, and toward the other end of the spectrum the metastatic presentation like Krukenberg's tumor or widespread peritoneal metastatic disease. So with this wide range of presentation, GCC should be always considered as a differential diagnosis in patients with "suspected appendicitis" with or without a mass leading to abdominal surgery. After the tumor is histologically confirmed, some factors need to be evaluated histologically as predictive factors for the biological behavior, e.g., the increased number of Paneth cells, increased mucin secretion and positivity for pancreatic polypeptide show a more aggressive and metastatic behavior and such cases should be considered for long-term follow-up. As nearly 20% show metastatic behavior and high possibility of intraperitoneal seeding before lymph node involvement, appendectomy with completion right hemicolectomy is recommended for all patients with caecal involvement and or high-grade tumors. Moreover, as the most common sites of metastasis are ovaries, in female patients, regardless of age and involvement, bilateral salpingo-oophorectomy is also recommended. In the presence of metastasis, combination chemotherapy regimes include 5-FU and leucovorin. It should be kept in mind that despite different terminology the biological difference between adenocarcinoid and carcinoma is not significant. Peritoneal carcinomatosis from adenocarcinoid of appendiceal origin is as invasive as peritoneal surface malignancy from colorectal adenocarcinoma. Patients in whom complete or near-complete surgical removal is possible should be considered for cytoreduction in combination with intraperitoneal chemotherapy. Based on the tumor histological predictors, long-term follow-up should be routinely advocated in all cases for possible metastasis. In this context, In-labeled octreotide scintigraphy is recommended for diagnosis and long-term follow-up of this neoplasm.The author(s) declare that they have no competing interests.PSP: Is the first and the corresponding author, Conception and design, Literature review with references, Drafting the article and revising, provided statistical analysis of the reviewed data, preparation of table.RK: helping in the preparation and drafting the manuscript, literature update, supervision, proof-reading and revising the manuscriptClick here for file


Pyometra is the accumulation of purulent material in the uterinecavity. Its reported incidence is 0.01−0.5% in gynecologic patients; however, as far as elderly patients are concerned, its incidence is 13.6% [ A plain chest X-ray film showed free air under the diaphragm on both sides. The abdominal X-rayrevealed no evidence of intestinal obstruction. Computed tomography scan of abdomenreported the presence of fluid within the abdominal cavity.A 92-year-old woman with severe abdominal pain and vomiting for24-hour duration was admitted to our hospital. Her gynecologichistory was unremarkable having undergone an eventful menopause.She had no history of postmenopausal bleeding or increased vaginaldischarge. On the physical examination, her abdomen was verytender, distended, and showed muscle rigidity. Rebound tendernesswas absent. Bowel sounds were hypoactive. Her blood pressure was110/65 mmHg, pulse rate was 114 beats/min, and axillary temperaturewas 36.9°C. Laboratory studies demonstrated a white cellcount of 5100/mmEscherichia coli andBacteroides fragilis. Histological examination revealedpyometra with no evidence of malignancy.Emergency explorative laparotomy was performed under the diagnosisof perforation of the gastrointestinal tract. The investigation ofthe gastrointestinal tract and gallbladder failed to reveal aperforation. The uterus was found to have two perforations,approximately 1 cm in diameter each, both in the uterinefundus, and purulent material exuding from the uterine cavity wasidentified. The uterus was soft and slightly enlarged. Bothparametriums were thickened and inflammatory changes were present.The fallopian tubes and the ovaries were normal. A total abdominalhysterectomy and bilateral salpingo-oophorectomy were performed.Culture of the pus grew She was observed in the intensive care unit with strict management ofrespiration and circulation for postoperative three days. On thethird postoperative day, she was transferred to the gynecology unit.Under the antibiotherapy with cefepime and metronidazole, hercondition improved postoperatively. However, on the tenthpostoperative day, wound dehiscence occurred and secondary woundclosure was performed. No other complications have occurred, and asthe patient completely recovered, she was discharged on theeighteenth postoperative day.Pyometra, or pyometrium, is defined as the accumulation of pus inthe uterine cavity resulting from interference with its naturaldrainage. It is an uncommon condition that occurs mainly inpostmenopausal women and is rare in the premenopausal age group. The claPyometra is a rare event in general population but more common inelderly women. It is caused by impairment of natural drainage ofthe cervix as a result of benign or malignant diseases. A detailedpelvic examination should be performed to rule out the associatedmalignancies. The diagnosis of pyometra is difficult, because itis usually asymptomatic.Ruptured pyometra should be kept in mind in elderly women presenting withacute abdomen as an unusual but serious condition.


Carcinosarcoma of the colon is a rare histopathological entity with uncertain histogenesis, that shows both epithelial and mesenchymal malignant differentiation. Carcinosarcoma rarely affects the gastrointestinal tract and only few cases are reported in the colon. Herein we describe a carcinosarcoma of the ascending colon, with morphological, ultrastructural and molecular analysis.p53 gene mutation (R282W in exon 8) and identical polymorphisms in p53 exon 4, in EGFR exons 20 and 21, and in c-kit exon 17. Microsatellite markers analysis revealed a common loss of heterozygosis on 18q. Overall, the data are consistent with a common origin of the two tumor components. The patient was treated with 8 cycles of oral capecitabine (1250 mg/m2 twice a day for 14 days repeated every 28 days) and two years after surgery is alive with liver metastases.An 81-year-old man was hospitalised for asthenia, weight loss and iron-deficiency anaemia. The patient underwent colonoscopy and adenocarcinoma was diagnosed by endoscopic biopsy. A right hemicolectomy was performed and, during surgical operation, liver metastases were detected. Histological examination of the surgical specimen revealed areas of both carcinomatous and sarcomatous differentiation, completely separated by fibrous septae. The sarcomatous component exhibited areas of smooth muscle and osteoblastic differentiation, with focal osteoid material deposition. Molecular analysis conducted separately on the epithelial and mesenchymal components revealed the same Carcinosarcoma of the colon is a rare tumour with both epithelial and sarcomatous components. Molecular analysis of the current case suggests the histogenesis from a common cell progenitor. Carcinosarcoma is a rare histopathological entity, exhibiting both epithelial and mesenchymal malignant differentiation, with uncertain histogenesis. Carcinosarcoma has been described in various organs, although head and neck, and female urogenital system are the most frequent sites of occurrence . In the 2 orally twice a day for 14 days repeated every 28 days for 8 cycles. Capecitabine was chosen for adjuvant therapy in this case since it has been reported to be as effective as 5-fluorouracil but with milder side effects in stage III colon cancer [An 81-year-old man with a past medical history of atherosclerotic heart disease, arterial hypertension, mitral insufficiency, bilateral carotid artery disease and early chronic renal failure was hospitalised for asthenia and weight loss. Laboratory investigation revealed iron-deficiency anaemia, with low haemoglobin (Hb) concentration , median cellular volume (MCV) level  and serum ferritin concentration . Serum levels of carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 19-9 and alphafoetoprotein (AFP) were within normal limits. Stool guaiac was positive for occult blood. Colonoscopy demonstrated an exophytic mass in the ascending colon and multiple diverticula in the entire colon, especially in the sigmoid region. Endoscopic biopsies were performed and at histological examination, adenocarcinoma was diagnosed. Chest x-ray was negative for metastatic disease. A week later a right hemicolectomy and regional lymph node dissection were performed. During surgery, liver metastases were detected. Computer tomography (CT) scan carried out after surgery identified 3 liver nodules in segment 4, in segment 8 and in segment 5, measuring 15 cm, 10 cm and 10 cm in their greatest dimension, respectively. The patient was subsequently treated with capecitabine at 1250 mg/mRepresentative sections of the tumour and all lymph nodes isolated from the surrounding adipose tissue were fixed in 10% buffered neutral formalin, embedded in paraffin and routinely processed. From each block, 5 μm-thick sections were cut and stained with haematoxylin and eosin (H&E).For immunohistochemical studies, the avidin-biotin peroxidase complex method was used with the following antibodies: cytokeratin 20 (CK20), desmin, vimentin, S-100, c-kit, CD34, sarcomeric actin, smooth muscle actin, osteonectin. The antibodies used are detailed in Table 4), dehydrated and embedded in epoxy resin. Ultrathin sections were counterstained in uranile acetate and lead citrate and observed in a Jeol JEM 1010  electron microscope operating at 80 kV.For ultrastructural examination, small samples were retrieved from paraffin-embedded material, deparaffinised in xylene, rehydrated in ethanol, post-fixed in osmium tetroxyde (OsOp53 gene (exons 4 through 9), the c-kit gene  and the EGFR gene  were evaluated by denaturing high-performance liquid chromatography (DHPLC) technique , followed by direct sequencing when required. K-Ras gene mutation status was assessed by direct sequencing. Distinct formalin-fixed paraffin-embedded material from both carcinomatous and sarcomatous components of the tumour was isolated by macrodissection. Genomic DNA was extracted by the phenol-chloroform method. Fifty ng of DNA were used for the specific amplification of all the exons listed above, as previously described [® Terminator v1.1 Cycle Sequencing Kit chemistry, purified by DyeEx 2.0 Spin kit  and analysed by capillary electrophoresis on Abi Prism 310 . Sequences obtained were aligned with normal sequences and examined for detection of mutations. For allelic status testing, 10 microsatellite markers on chromosomal arms 9p, 11p, 13q, 17q and 18q were investigated . Specific PCRs with fluorescent primers were conducted followed by loss of heterozygosis (LOH) analysis with GeneScan software .For the molecular detection of somatic genetic alterations, the The surgical specimen included a 4 cm long ileal segment, a 13.5 cm long colonic segment, and the caecal appendix. At the ileocaecal valve, on the colonic side, an exophytic, centrally ulcerated mass, measuring 7 cm in its greatest dimension, was documented. Twenty-two lymph nodes were isolated from the adipose tissue surrounding the bowel wall.Microscopically, about 70% of the tumour displayed features of moderately differentiated adenocarcinoma, invading the subserosa (pT3). The carcinoma was composed of neoplastic epithelial cells, arranged in nests forming glandular structures with areas of central necrosis. Neoplastic epithelial cells exhibited rare mitotic figures, an increased nuclear/cytoplasmic ratio and prominent nucleoli. Approximately 30% of the tumour presented mesenchymal features. The sarcomatous component was completely separated from its carcinomatous counterpart by fibrous septae Fig. . In the The epithelial component was immunoreactive for CK20 in approximately 30% of neoplastic cells, whereas it was negative for vimentin and osteonectin. In the mesenchymal component, spindle cells were diffusely immunoreactive for smooth muscle actin  investigated, 7 were considered informative and four of them displayed an identical pattern comparing normal and the two tumoral components. An almost complete loss of the second allele was appreciated for D18S585, D18S35 and D18S51 markers (18q21) in both tumor' parts . The molecular analysis performed in the current case supports the hypothesis of a common cell precursor , since the same mutation R282W in exon 8 of the Studies conducted on carcinosarcomas of nasopharynx , uterus p53 gene and identical LOH for D18S585, D18S35 and D18S51 in both carcinomatous and sarcomatous components, supporting the hypothesis of a common cell progenitor.Herein we describe a case of carcinosarcoma of the colon, with epithelial and mesenchymal components, completely different and separate at the morphological, immunohistochemical and ultrastructural levels. Molecular analysis revealed the same mutation R282W in exon 8 of the The author(s) declare that they have no competing interests.AAS revised the literature and drafted the manuscript. VV conducted the molecular analysis and participated in writing the manuscript. PB carried out the ultrastructural examination. MR performed the surgical operation and clinical follow-up. SF and SB devised the study and revised the manuscript. All the authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Carcinoid tumors rarely arise in the extrahepatic bile duct and can be difficult to distinguish from carcinoma. There are no reports of clear cell carcinoid (CCC) tumors in the distal bile duct (DBD) to the best of our knowledge. Herein, we report a CCC tumor in the DBD and review the literature concerning extrahepatic bile duct carcinoid tumors.nd portion duodenectomy and a lymph node dissection. The surgical specimen showed a golden yellow polypoid tumor in the DBD (0.8 × 0.6 × 0.5 cm in size). The lesion was composed of clear polygonal cells arranged in nests and a trabecular pattern. The tumor invaded through the wall into the fibromuscular layer. Immunohistochemical stains showed that neoplastic cells were positive for neuron-specific enolase (NSE), chromogranin A, synaptophysin, and pancreatic polypeptide and negative for inhibin, keratin, CD56, serotonin, gastrin and somatostatin. The postoperative course was uneventful and he is living well without relapse 12 months after surgery.A 73-old man presented with fever and occult obstructive jaundice. Ultrasonography, computed tomography (CT) and magnetic resonance cholangiopancreaticography (MRCP) demonstrated a nodular tumor projection in the DBD without regional lymph node swelling. Under suspicion of carcinoma, we resected the head of the pancreas along with 2Given the preoperative difficulty in differentiating carcinoid from carcinoma, the pancreaticoduodenectomy is an appropriate treatment choice for carcinoid tumors located within the intra-pancreatic bile duct. Extrahepatic bile duct carcinoid tumors are extraordinarily rare lesions and account for only 0.32% of all carcinoids of the gastrointestinal tract . Since 1We report herein a clear cell carcinoid tumor of arising from the distal (intra-pancreatic portion) common bile duct as the first report of a clear cell carcinoid tumor of the extrahepatic bile duct. Because of the rarity of carcinoid tumors in the bile duct as well as the rarity of clear cell carcinoid tumors, it is difficult to adequately assess their malignant potential, their responsiveness to particular treatments and their overall prognosis. Improved understanding can only be obtained by analyzing the collected data from reported cases; accordingly, we reviewed the literature concerning carcinoid tumors originating from the extrahepatic bile ducts.A 73-old man was visited our hospital with high fever (39°C), epigastric pain and reduced consciousness. He had suffered from poorly controlled diabetes mellitus, hypertension and multiple brain infarctions during the previous 3 years. On physical examination, no abdominal mass was palpable. Laboratory tests revealed signs of inflammation, including a WBC count of 34,400/mm3 and a CRP 10.4 of mg/dl, and abnormal liver function results with signs of biliary obstruction . The tumor markers were within the normal range: CEA 4.0 ng/ml and CA19-9 128 U/ml (slight elevation). The patient's blood sugar was elevated at 311 mg/dl with an elevated HbA1C of 7.9%, but no ketone bodies were detected in the urine. Contrast-enhanced abdominal CT revealed a slight dilatation of the intra/extra-hepatic bile ducts, a slightly enhanced protruding nodular lesion in the intra-pancreatic common bile duct and no lymph node swelling neither around the head of pancreas nor within the hepato-duodenal ligament. Ultrasonography demonstrated a slightly thickened gallbladder wall without calculi. Endoscopic retrograde cholangiopancreatography (ERCP) and MRCP showed a nodular mass with an irregular surface protruding into the bile duct lumen from the left wall at the distal end of the common bile duct Fig.  . AbdomiDuring laparotomy, a polypoid lesion was palpated in the common bile duct at the head of the pancreas. However, whether tumor was invading the pancreas or not was unclear and regional nodes were soft and slightly swollen. Given the suspicion of distal bile duct carcinoma without node metastasis (less than stage II); the patient underwent a modified pancreaticoduodenectomy. Taking into account possible complications, we tried to preserve not only the pylorus, but also the first and third portions of the duodenum and performed a regional lymphadenectomy.Macroscopically, the surgical specimen showed a golden yellowish polypoid mass, measuring 1.0 × 1.2 × 0.7 cm in size that was projecting from the surface of the intra-pancreatic portion of the bile duct. The superficial yellow discoloration extended to the proximal epithelium from the tumor base. The thickening of the bile duct wall at the tumor bed suggested longitudinal and vertical tumor invasion Figure . MitoticRecently, the World Health Organization (WHO) developed a new classification system that gives a better description of the characteristics and biological behavior of the carcinoid tumors. These tumors can be malignant and the histopathological differentiation of carcinoid tumors ranges from well differentiated  to poorly differentiated malignancies with neuroendocrine features, typified by small cell carcinoma. Carcinoid tumors derived from neuroendocrine cell compartments and their frequency of occurrence correlate with the site-density of neuroendocrine cells. Most over 60%) of carcinoid tumors arise from the gastrointestinal system, followed by the bronchopulmonary system 25%). Carcinoid tumors of the extrahepatic bile duct, excluding Vater, intrahepatic bile duct, gallbladder and cystic duct, are extremely rare. The incidence in all gastrointestinal carcinoid tumors is 0.32%, according to the recent analysis of 13,715 carcinoid tumors 5%. Carci. Pilz re0% of carOnly two clear cell carcinoid (CCC) tumors of the gallbladder have been described as originating from the biliary tracts; however, there have been no case reports of CCCs arising from the extrahepatic bile ducts. One case has been interpreted to be a CCC tumor of the gallbladder and appeared to be a distinctive manifestation of von Hippel-Lindau (VHL) disease with diffuse expression of inhibin in the tumor . Those and portion duodenectomy was successfully performed with an en bloc resection of the tumor and surrounding structures including regional lymph nodes. In the event of middle to proximal bile duct involvement, an en bloc excision of the bile ducts from the main hepatic ducts down to the upper margin of the pancreas and adjoining lymph nodes (with construction of a Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy) will be appropriate to preserve the patient's prospects for a cure.Carcinoid tumors are composed of multipotential cells with the ability to secrete numerous hormonal substances and vasoactive peptides. These substances cause the clinical features that constitute carcinoid syndrome; however, within the extrahepatic bile duct, only one carcinoid tumor has been associated with the syndrome . The mosAccurate pre-and intra-operative histological diagnosis may be problematic because the discrimination between carcinoid tumors and carcinoma is difficult at times, particularly with frozen section specimens. In some cases, frozen section diagnosis is inconclusive and resection must be undertaken with an indeterminate diagnosis. In our case, cytological examination failed to demonstrate carcinoid cells. However, imaging diagnostic findings obtained from CT, MRCP and ERCP and abdominal angiography were actually regarded as sufficient evidence to precede radical surgery since those images demonstrated a protruding nodular tumor in the distal bile duct and no evidence of metastasis in the regional nodes, distant metastasis and vascular involvement.Carcinoid tumors are slow-growing malignancies; however, prognosis after treatment depends on the evidence of degree of malignancy. Mitotic counts and the degree of anaplasia cannot reliably determine malignancy; rather, it is microscopic local invasion or gross distant spreads that differentiate the benign from the malignant forms of this disease. Metastases to distant organs (chiefly the liver), regional lymph nodes and direct invasion into the perineural lymph space, microscopic vessels and adjacent structures were found in 21 of 49 reported cases (including our case and excluding three cases which lacked adequate information). Out of those 21 patients with extended tumors, only two deaths),12 were Definite diagnosis is difficult preoperatively but may be improved with the assessment of neuroendocrine markers in suspected cases. Disease-free survival is prolonged following complete and safe resection of the tumor. Because carcinoid tumors are slow growing and less aggressive than adenocarcinoma, even if these patients have metastases, they should be treated aggressively. Continued reporting of single cases and long-term follow-up should be encouraged with reporting of the results of treatment.The author(s) declare that there are no competing interestsTS carried out the pathologic studies, participated in the sequence alignment and drafted the manuscript. SY participated in surgical operation as one of assistants for TT and took charge of post-operative managements together with KT. NH, NT and RM carried out the preoperative diagnostic work-up and corporate design of the study in cooperation with TM.All authors read and approved the final manuscript.showing case review of carcinoid tumors of the extrahepatic bile ducts.Click here for file


Purpose. To document a case of myxoid liposarcoma in which PET scan was less sensitive than MRI in detecting spinal metastasis. Materials and Methods. The case of a 65-year-old female with a history of myxoid liposarcoma (MLS) of the thigh resected 5 years previously and now presenting with low back pain is presented. Her medical oncologist ordered an FDG-PET scan to evaluate distant recurrence. Subsequently, an MRI of her spine was obtained by her surgeon. Results. The FDG-PET scan was obtained 1 week prior to the MRI, and it did not show increased glucose uptake in the spine. Her MRI did show increased signal intensity in her lumbar spine. CT needle biopsy confirmed the lesion to be metastatic MLS. Conclusion. FDG-PET scans are utilized to detect distant recurrence of cancerous lesions. Myxoid liposarcoma has a unique propensity to metastasize to the spine. Previous reports have documented the unreliability of bone scintigraphy to diagnose these metastases. Our report demonstrates that FDG-PET may also lack the sensitivity needed to detect these lesions. We advocate total spine MRI when screening for metastases in this population when they present with back pain. Liposarcoma is one of the most common soft tissue sarcomas and myxoid liposarcoma (MLS) is the second most common subtype. Soft tissue sarcomas tend to metastasize to the lungs, however myxoid liposarcoma may metastasize to unusual extrapulmonary sites including bone –3. Severblack arrow). Subsequent axial MRI (white arrow). Sagittal MRI (A 65-year-old female with a history of an AJCC stage III popliteal myxoid liposarcoma developed mild low back pain 5 years after her initial surgery. The primary tumor had beentreated with wide resection followed by 6500 cGy to the popliteal fossa. She had been disease-free and was seen by her medical oncologist for a routine check when she complained of back pain. Whole body FDG-PET  did not xial MRI  showed attal MRI  of the lScreening for metastatic liposarcoma is controversial. Chest CT misses a significant number of metastases as MLS has a propensity to metastasize to extrapulmonary sites such as the one in our case report. Bone scintigraphy is not a reliable diagnostic test for metastatic myxoid liposarcoma –6. This The negative PET scan may reflect low metabolic activity within the myxoid/paucicelluar regions of these tumors. It is possible that the absence of appreciable glucose uptake reflectsthe inability of the PET scans to detect those cells that are actively utilizing glucose within the myxoid stroma. The radiolabeled glucose may not be able to reach the cells embedded in the matrix. However, our case represents a high-grade liposarcoma with at least a 5% round cell component. One would expect that these areas could be detected by FDG-PET. Further study is necessary to determine whether the ratio of round cells relative to myxoid stroma is important.Spine metastasis should be considered when one is evaluating a patient with a history of MLS presenting with back pain. Bone scintigraphy and FDG-PET do not seem to be sensitive enough to detect these lesions. We recommend MRI of the spine when one is confronted with a patient complaining of back pain with a history of myxoid liposarcoma. While MRI seems to be more sensitive than FDG-PET,neither is specific, and biopsy should be considered for histologic confirmation.


Rectal carcinoids comprise only about 1% of all anorectal neoplasms. In addition, ganglioneuroma of the gastrointestinal tract is a rare tumor composed ganglion cells, nerve fibers, and supporting cells. Multiple carcinoid tumors with diffuse ganglioneuromatosis limited to the rectum are quite unusual.A 69-year-old man was referred to us because of about 100 small submucosal rectal tumors. He underwent abdominoperineal resection. Pathology revealed carcinoid tumors for about 30 submucosal nodules and diffuse ganglioneuromotosis. To date (6 months later) he remains well with no recurrence.Although the optimal treatment for the multiple rectal carcinoids remains to be clearly established, it is believed that not all patients with multiple rectal carcinoids (measuring less than 1 cm in diameter) need to have a radical operation. However, the treatment plan for each case should be individualized and a careful follow-up is mandatory. Carcinoid tumors were initially described as a morphologically distinct subset of small intestinal neoplasms with a less aggressive behavior than that of the intestinal adenocarcinoma. Carcinoid tumors of the rectum comprise only about 1% of all anorectal neoplasms .Gastrointestinal ganglioneuromas are also rare tumors that are generally well differentiated and benign tumors. They commonly occur in the retroperitoneum and posterior mediastinum. Though they may be found anywhere in the body, particularly in the distribution of the major sympathetic ganglia, their involvement in the gastrointestinal tract is a rare occurrence. Some reports have indicated that ganglioneuromas of the gastrointestinal tract have been found in patients with several systemic disorders including multiple endocrine neoplasia IIB (MEN IIB), von Recklinghausen's disease, tuberous sclerosis, Cowden's disease, juvenile polyposis, filiform polyposis, and colonic adenocarcinoma ,4.Multiple carcinoid tumors with diffuse ganglioneuromatosis limited to the rectum are quite unusual. The relationship between multiple carcinoid tumors and gastrointestinal ganglioneuromatosis of the rectum is herein discussed.A 69-year-old man was referred to us because of about 100 small submucosal rectal tumors detected at examination by his private physician. Multiple biopsies reported to be a tentative diagnosis of multiple carcinoid tumors. He had never been diagnosed as having multiple endocrine neoplasia (MEN) or other multiple tumor syndromes. His family history was not contributory. Physical examination revealed no abnormalities. Serum serotonin level was within normal range, 221 ng/ml. Tumor markers were within normal limits, CEA (carcinoembryonic antigen) 2.3 ng/ml, CA (carbohydrate antigen) 19-9 <2.0 U/ml. Computed tomographs of the brain, chest, abdomen and pelvis did not show any abnormality. He underwent abdominoperineal resection. Pathology revealed carcinoid tumors for about 30 submucosal nodules, which especially concentrated in the lower rectum Figure  and diffCarcinoid tumors of the rectum are considered to be a frequent primary site . One-halOn the other hand, gastrointestinal ganglioneuromas may be classified into three major categories: diffuse ganglioneuromatosis, ganglioneuromatous polyposis, and polypoid ganglioneuromas . DiffuseCarcinoid tumors have been reported to be part of hereditary cancer syndrome . The mosThe surgical treatment for carcinoid tumors is generally dictated to a degree, but not absolutely, by the size . Tumors Although the optimal treatment for the multiple rectal carcinoids remains to be clearly established, it is believed that not all patients with multiple rectal carcinoids (measuring less than 1 cm in diameter) need to have a radical operation. However, the treatment plan for each case should be individualized and a careful follow-up is mandatory.The author(s) declare that they have no competing interests.MH, MK, NT, TK and JF carried out the surgical procedures; MH and JF contributed to the design of the study; HK gathered the data form the literature search; YI performed the histological analysis of all surgical specimens and provided histological sections as figures for the manuscript; TK revised and finally approved the manuscript for been published. All authors approved the final manuscript.


Teratoma is a rare developmental neoplasm that arises from totipotential tumor stem cells. Head and neck teratomas constitute about 10% of all cases. Only two cases of mature teratoma of the nasal septum have previously been documented in the world literature.We present a case of histologically confirmed mature teratoma arising from the nasal septum in an eighteen month old Nigerian female who presented with a history of noisy breathing associated with recurrent rhinorrhea since birth. Physical examination revealed obstruction of the right nasal cavity by a pale fleshy mass. She underwent a total surgical excision and to date, after thirty one months follow-up, she is free from recurrence.The prognosis for benign teratoma of the nasal septum is good following total surgical excision. Teratoma is a developmental and sometimes congenital neoplasm that displays gross and microscopic differentiation into a wide variety of tissues representative of all three germ layers – ectoderm, mesoderm and endoderm. The presence of tissues within the lesion that are foreign to the affected sites is a distinctive feature of this rare tumor. The incidence of teratoma worldwide is about 1 in 4,000 live births ,3. A MEDAn eighteen month old Nigerian female presented to our health facility with a history of noisy breathing since birth, associated with intermittent mucoid rhinorrhea, especially from the right nasal cavity. Protrusion of a pale mass in the right nasal cavity was observed by the parents prior to presentation. There was no epistaxis, fever or difficulty with feeding. The child was the product of a term pregnancy and delivery was said to be uneventful. The developmental milestones were within normal expectations.Examination revealed a well-fed young girl with noisy respiration, afebrile and not pale. The external nasal pyramid appeared splayed; the right nasal cavity was completely obliterated by a lobulated pale mass which was firm, sensitive to touch, non-hemorrhagic and attached to the nasal septum. It was partly covered by mucoid secretions. Test aspiration of the mass was dry. The left nasal cavity, ears, throat and neck appeared normal. A nasogastric tube was easily passed through the left nasal cavity. No lesion was seen in any other parts of the body.X-ray  revealed radio-opacity of the right nasal cavity, extending posteriorly into the nasopharynx. There was no evidence of calcification of the soft tissue shadow or bony erosion of the nasal bony framework. The plain radiographs of the soft tissue neck and the chest were within normal limits. Complete blood and platelet counts were within normal range.Examination under general anesthesia  revealed that the right nasal mass could neither be delivered via the anterior nares nor the post nasal space, prompting a right lateral rhinotomy for adequate exposure of the mass Figure . The staThe excised mass appeared irregular, but smooth-surfaced, measuring 6 × 5 × 3 cm and weighing 27 g. The cut surface showed a lobulated tan colored appearance. Histological examination revealed haphazardly arranged heterogeneous tissues, including cysts lined by epidermis with associated hair follicles and sweat glands, gastrointestinal epithelium, respiratory epithelium and columnar epithelium reminiscent of mammary glands. Lobules of mature adipose tissue and fibrocollagenous connective tissue were also present , immature  and monodermal  teratomas . In our etal  and Streetal . All theetal . This inis tumor .Beside ultrasonography, there are other radiological tools that are necessary to confirm or rule out other possible differential diagnoses prior to the histological diagnosis. Plain radiograph (X-ray) of the upper aero-digestive system would reveal a soft tissue shadow as seen in this case. Teratoma shows some tissue calcification in about 16% of cases . The calin utero and postpartum diagnosis because of the good resolution for the soft tissues. In the developing countries, like ours, poverty is limitation to the use of these high resolution radiological investigative tools. For instance, the index case was requested to do a cranio-facial CT-scan as part of the work-up for surgery, but could not afford it.Computed tomographic scan would exclude bony erosion or defect at the base of the skull as it would be seen in encephalocele or in malignant tumors. The origin of antro-choanal polyps would better be defined by computed tomographic scan. Magnetic resonance imaging is useful in both Fine needle aspiration cytology and tissue histology would further differentiate nasal teratoma from other neoplasias, such as nasal glioma, choristoma, rhabdomyoma, and granulomatous lesions. Histological examination remains the gold standard for the confirmation of this lesion.The principles of treatment for nasal and other upper airway teratomas include early intervention, through establishment and maintenance of airway, total surgical excision of the tumor, adjuvant therapy (chemo-radiation) with aggressive monitoring of the patient within the first 2 years and then close monitoring for life in adults if there is evidence of malignant transformation .Over three decades ago, Hawkins and Park  reportedet al [Our patient had benign unilateral nasal teratoma, which was managed through surgical excision alone with a good outcome. An external approach  was used here, since we lack the equipments for less invasive and more sophisticated transnasal endoscopic technique. This is similar to the mode of management adopted by Takehara et al  in the mStreetharan and Prepageran  managed The author(s) declare that they have no competing interests.TI conceived the idea of reporting the case.DDK – assisted during the surgery and in the literature search.BAN – the pathology Senior Registrar that prepared the histology slides and contributed in literature search and manuscript preparation.OAA – the consultant anesthetist that anesthetized the patient and also contributed in the manuscript preparation.OGN – the principal surgeon, who also proof-read and made necessary corrections on the manuscript.EEA – read the slides and processed the microfilms for the slides to illustrate the various components of the teratoma.All authors read and approved the final manuscript.


Idiopathic segmental infarction of the greater omentum (ISIGO) is an uncommon cause of acute abdomen in children and adults and its etiology is rather vague and speculative. The clinical presentation is usually with atypical acute or subacute abdominal pain. In a number of cases radiologic imaging allows proper preoperative diagnosis and treatment.We report a case of ISIGO in a 31 year old patient, who presented with acute abdominal pain, nausea, vomiting and leukocytosis. Radiologic investigation was non-specific. The patient underwent surgical resection of the infracted omentum with compete recovery.ISIGO should be considered in the differential of acute abdomen especially when presentation is atypical and all other causes have been excluded. In cases with non-specific radiologic findings, laparotomy is necessary for proper diagnosis and treatment. Surgical resection of the infracted omentum results in uneventful recovery in the majority of cases. Primary or idiopathic segmental infarction of the greater omentum (ISIGO) is an uncommon cause of acute abdomen in adults and children and has a vague and rather speculative etiology -3. Less A 31-year-old man was admitted to our department because of acute abdominal pain, nausea, vomiting and anxiety of ten hours duration. On physical examination severe abdominal tenderness was marked, localized to the right lower abdomen aggravated on walking and coughing. His temperature was 37.8 degrees celcius and his pulse rate 92 bpm. Laboratory investigation disclosed a white blood count of 16,800/mm3, neutrofils 72% with left shift and elevated erythrocyte sedimentation rate (ESR) and C reactive protein (CRP).All other hematological and biochemical parameters were within normal range. Clinical presentation and laboratory findings suggested acute appendicitis and laparotomy was performed. Through a right paramedian subumbilical incision the right iliac fossa was explored and a grossly inflamed omental mass was revealed and resected. Fig. .A small amount of fluid was seen. The appendix was macroscopically normal and no other cause of acute abdomen was identified. The pathology of the surgical specimen reported primary omentitis. The patient made an uneventful recovery.Segmental infarction of the large omentum is an uncommon condition that may present as acute abdomen in any age group, mainly in the fourth and fifth decade of life, with a male to female ratio of 2:1 . It is sAlthough controversial, as has been suggested but not proven, an infectious cause has also been reported for the spontaneous segmental infarction of the greater omentum ,8.In the majority of the published cases, omental infarction is located to the right side probably due to the greater size and mobility of the right sided omentum in relation to the left side . PrecipiClinical presentation is generally atypical. Cardinal manifestations are acute or subacute abdominal pain, commonly of right lower quadrant or right paraumbilical in location and severe abdominal tenderness, which is no radiating and progressively increasing in severity. Fever is usually absent or of low grade. In general, there is no nausea, vomiting, anorexia or other gastrointestinal complain. Laboratory investigation discloses mild leukocytosis and elevated ESR and CRP ,3. AbdomLaparotomy or laparoscopy discloses a fatty hemorrhagic mass in the abdominal cavity accompanied by free serosanguineous fluid ,12. CareResection of the infracted omentum during laparotomy or laparoscopically is considered to be mandatory. Nevertheless, there are reports of effective non-operative treatment of omental torsion and segmental infarction of the greater omentum correctly diagnosed by imaging techniques, when the patient's condition is stable. The authors suggest that in these uncomplicated cases conservative treatment is acceptable . HoweverIn conclusion, ISIGO should be considered in the differential of acute abdomen, especially in cases with atypical presentation. Abdominal CT scan may reveal the underlying pathology. However, in a number of cases surgical intervention is necessary for definite diagnosis. Resection of the infracted omentum during laparotomy or laparoscopically is the treatment of choice, prevents future complications and is associated with fast recovery.ISIGO: idiopathic segmental infarction of the greater omentum;US: ultrasound;CT: computer tomography;ESR: erythrocyte sedimentation rate;CRP: C-reactive proteinThe author(s) declare that they have no competing interests.MS: Surgeon who performed the operation and edited a part of the manuscript.MS: Surgeon who performed the operation and prepared the draft.KG: Doctor who performed the pathological examination and edited part of the manuscript.AS: Surgeon who contributed to the performance of the operation.PS: Literature search, revision of bibliography.All authors have read and approved the final manuscript.


A 26-year old man presented with a 3-month history of a progressively enlarging palpable parieto-occipital mass. A CT scan indicated the lesion arose from the dura with bony destruction. A stealth assisted craniotomy was performed with the provisional diagnosis of osteoblastic meningioma. Further histopathologic analysis of the intracranial mass was consistent with leiomyosarcoma. Staging evaluation, including CT and PET scans, demonstrated no other sites of disease. Despite complete surgical resection and radiotherapy to the resection site, the disease recurred locally and systematically 5 months later. Primary intracranial mesenchymal tumors are rare and few cases have been previously reported. Outcomes have been universally poor and current therapeutic approaches appear to have only limited benefit. Intracranial neoplasms of mesenchymal origin are uncommon. Themajority of these tumors represent metastatic disease from otherprimary sites. Primary intracranial soft tissue sarcomas can,however, arise from mesenchymal cells of the dura matter or thecerebral blood vessels. These tumors often mimic meningioma onpreoperative MRI and, although rare, should be included in thedifferential diagnosis of dural-based lesions. Immunohistochemicalstains can help distinguishing these tumors from meningiomas.Postoperative radiation and chemotherapy should be considered,however, the prognosis has been universally poor. We describe thecourse of a patient with a primary intracranial leiomyosarcoma andreview the literature.A 26-year old previously healthy male presented with a 3 monthhistory of progressively enlarging parieto-occipital mass. Therewere no constitutional or neurological symptoms and no symptomssuggestive of other sites of involvement. There was nohistory of smoking, IV drug abuse, or sexual promiscuity. Physicalexamination was remarkable for a palpable 5 cm, fixed,nontender mass over the right parieto-occipital region. Completeblood counts and metabolic panel were normal. Viral serology wasnegative for both human immune deficiency virus (HIV) andEpstein-Barr virus (EBV). A CT scan of the brain demonstrated alarge highly vascular soft tissue mass involving the meninges withinvasion of the parietal bone and displacement of the brainparenchyma . A cliniSoft tissue sarcomas are rare tumors and account for only onepercent of all cancers . Most inAn increased incidence of leiomyoma and leiomyosarcoma has beenobserved in immunocompromised patients. The association of theseneoplasms with Epstein-Barr virus infection and AIDS is welldocumented in the literature , 6. Our Our review of primary intracranial myomatous tumors found thatonly one out of 29 reported cases demonstrated smooth muscledifferentiation. The other cases were pure mesenchymal or mixedneural and mesenchymal tumors showing skeletal muscledifferentiation. Tumors with rhabdomyomatous elements were morecommon than tumors containing leiomyosarcomatouscharacteristics .In another study of 3829 patients with soft tissue sarcoma, 21patients presented with and 19 patients subsequently developedbrain metastases. In this study the most frequent tumor type withmetastatic brain involvement was leiomyosarcoma .The diagnosis of leiomyosarcoma is confirmed by ultrastructuralfeatures of smooth muscle cells and immunohistochemistry. Thetumor cells are elongated with tapering cytoplasmic processes withelongated, convoluted nuclei, pinocytic vesicles, and basementmembrane material around the cytoplasmic membrane. Thedifferential diagnoses, which include malignant astrocytoma,malignant fibrous histocytoma, and meningioma, were excluded byimmunohistochemical testing. Our patient's tumor was negative forS-100 protein and epithelial membrane antigen and positive forsmooth muscle actin and cytokeratin staining.Although the pathologic and radiographic examination indicated aprimary intracranial leiomyosarcoma in our patient, anotherprimary site could not be excluded with complete certainty. Withinthe limits of CT scan and other imaging modalities, the evaluationand the clinical course of our patient were consistentwith a primary intracranial location.The prognosis for primary intracranial leiomyosarcoma is poor withthe longest reported survival being 32 months , 11.PatA primary intracranial tumor of mesodermal origin is rare and themajority of these tumors are rhabdomyosarcoma. Leiomyosarcomas maymimic meningiomas on preoperative MRI and, although extremelyrare, must be included in the differential diagnosis ofdural-based lesions


Castleman's disease is a rare lymphoproliferative syndrome. Its etiology and pathogenesis are unclear. The disease can be occasionally associated with a paraneoplastic pemphigus (PNP), an autoimmune mucocutaneous disorder commonly seen in neoplasms of lymphocytic origin.We present a case of a 63-year old male patient who was referred for surgical treatment of a lately diagnosed retroperitoneal pelvic mass. The patient had been already treated for two years due to progressive diffuse cutaneous lesions histologically consistent with lichen ruber verucosus and pemphigus vulgaris. Intraoperatively a highly vascularized solid mass occupying the small pelvis was resected after meticulous vascular ligation and hemostasis. After surgery and following immunosuppressive treatment a clear remission of the skin lesions was observed.Castleman's tumor should be always suspected when a retroperitoneal mass is combined with PNP. In a review of the literature we found 37 additional cases. Complete surgical resection of the tumor can be curative in most of the cases. Castleman's disease is a rare lymphoproliferative syndrome which was first described in 1956 .PNP is an autoimmune mucocutaneous disorder associated with neoplasms of lymphocytic origin, among them with Castleman disease . PNP canWe report a case of a patient with a retroperitoneal Castleman's tumor and paraneoplastic pemphigus that improved after tumor resection. A review of the literature is additionally presented.®) but skin lesions had remained stable or had shown a mild shortlasting remission only. The histological examination of the lesions was consistent with lichen ruber verucosus and pemphigus vulgaris.A 63-year-old male patient was referred to our Surgical Department from the Department of Dermatology with the diagnosis of a large retroperitoneal pelvic tumor for further treatment. The mass was morphologically consistent with a Castleman's tumor. Patient's symptoms had begun two years earlier with rapidly progressive diffuse cutaneous lesions all over the trunk and extremities as well as oral lesions with the form of erythematous plaques and superficial hyperceratosis. Erosive mucositis with lichenoid inflammation affecting the oral and penis glance mucosa as well as dystrophic nails associated with periungular erosions and onychorrexis had been observed. The patient had been initially treated with local corticosteroids and acitretin . As skin lesions had shown no signs of remission, further diagnostics was initiated in order to determine a possible paraneoplastic nature of skin pathology.A subsequently performed contrast-enhanced multislice CT demonstrated a highly vascularized retroperitoneal mass (10.3 × 9.2 cm) with significant contrast enhancement consistent with a sarcoma or a Castleman's tumor Figure  and 1b. On physical examination besides the above mentioned diffuse erosive muscositis with lichenoid inflammation, no lymphadenopathy or organomegaly was noted. Preoperative examination of the complete blood counts revealed the following results: hematocrite 41%, white blood cells 11.0/nl (80% neutrophils), PTL 562 k/m, PT 90% (INR 1.07).Following thorough preoperative diagnostics the patient was submitted to laparotomy for resection of the tumor. After catheterisation of both ureters with "pig-tail" catheters a midline laparotomy was performed. Entrance into the peritoneal cavity revealed a highly vascularized solid mass occupying the small pelvis extending to the right iliac fossa.After careful dissection of the right iliac vessels and right ureter off the mass, complete excision of the bulky tumor could be performed. Special attention was given to the meticulous ligation of the tumor vessels, mainly collaterals coming from the internal iliac artery, which was separately ligated.After surgery the patient was treated for four days on the ICU. Postoperative course was uneventful, so that the patient could be referred to the Dermatology Department for further treatment of his skin lesions. A complete evaluation of the patient's immune status was performed. Main results are as following: lymphocytes 5%, CD3+ 61% , CD3, CD4/CD3, CD8 0.91 % (0.9–3.6), CD3/CD19 12% (6.4–22.6), CD3/CD4 30 % (28.5–60.5), CD3/CD8 33 % (11.1–38.3), CD3/CD16+56 23% (5.6–30.9). Anti-Desmoglein 1-Abs were 59.96 (>20 positive), anti-Desmoglein 3-Abs 48.25 (>20 positive), BP 180-Abs 6.25 U/ml (>9 positive). Skin Abs were negative, but circulating IgG antibodies reactive with rat urinary bladder epithelia surfaces were detected positive (diagnostic of paraneoplastic pemphigus). New histological examination of biopsies taken from skin lesions, extremities, and the oral mucosa were consistent with the diagnosis of lichen ruber and highly suggestive of a pemphigus. The latest diagnosis was confirmed with direct immunofluorescence, which showed a high intracellular deposition of anti-IgG (antibodies against desmosomes) in the whole epidermis. Histological examination of the mass revealed a Castleman tumor of hyaline vascular type locally in sano removed.One month after surgery the patient was newly referred to us with a postoperative 15 × 11 cm pelvic abscess, which was percutaneously drained under CT control. Culture of the drained pus was positive for staphylococcus aureus (MRSA) and an intravenous treatment was initiated (linezolid and flucloxacillin). Percutaneous drainage was repeated two weeks later so that a rest fluid around the right psoas muscle could be successfully evacuated. Due to progression of the painful skin lesions an immunosuppressive treatment was initiated with cyclophosphamide and urbason (every two weeks) and cortison daily followed by a per os immunosuppression protocol with cyclophosphamide and endoxan.A dramatic improvement of the skin lesions could be seen in a period of three weeks' time. Following a removal of the left ureter catheter and change of the right one, the patient could be released in a good condition with almost complete remission of the mucocutaneous lesions four weeks later .Castleman's tumors are neoplasms of lymphatic origin, also known as giant lymph node hyperplasia or benign giant lymphoma. Histologically these tumors can be classified into 3 types: a) Hyaline-vascular type (80–90%), b) plasma cell type (10–20 %), and c) intermediate types . The mosPNP is a clinically, histologically and immunologically distinct autoimmune mucocutaneous disease. This entity was first described by Anhalt et al. . A varieSo far, it is unclear why Castleman's tumors are related to the pathogenesis of PNP. One hypothesis is that Castleman's tumors' proteins function as antigens and induce the production of autoantibodies in patients with paraneoplastic pemphigus. Meanwhile a general propensity for immune dysregulation has been proposed .Several cases of association between pemphigus and Castleman's tumor have been reported. In a review of the english language literature we found 37 additional cases -5,7-22. Treatment of the disease depends mainly on the histological type and the clinical symptoms . SurgeryIn the present case, although there was an initial deterioration of skin lesions after tumor resection, the lichenoid lesions dramatically improved after the initiation of immunosuppressive therapy, leaving a postinflammatory hyperpigmentation.Castleman's disease is a diagnosis, which should be considered in the presence of a hypervascular tumor, whatever its localization. Additionally PNP should be always considered when investigating atypical mucocutaneous lesions such as erosive lichen planus or pemphigus vulgaris non-responding to medication. In these cases a prompt search for an underlying tumor should be initiated. Complete surgical resection of the tumor can be curative in most of the cases.The author(s) declare that they have no competing interests.CM Member of operative team which performed the operation, Section "Discussion"CB Review of the literature, Section "Discussion"JH Collection of information about patient's previous dermatological history, writing of the section "Case report"CAJ Member of operative team which performed the operation, final form of the manuscript


Isolated splenic metastases from colorectal cancer are very rare and there are only 13 cases reported in the English literature so far. Most cases are asymptomatic and the diagnosis is usually made by imaging studies during the evaluation of rising CEA level postoperatively.th postoperative month necessitated further imaging studies including computed tomography of the abdomen which revealed a mass in the spleen that was subsequently confirmed by 18FDG- PET scanning to be an isolated metastasis. The patient underwent splenectomy 17 months after his previous cancer surgery. Histological diagnosis confirmed a metastatic adenocarcinoma with no capsule invasion. After an uneventful postoperative period, the patient has been symptom-free during the one-year of follow-up with normal blood CEA levels, although he did not accept to receive any further adjuvant therapy. To the best of our knowledge, this 14th case of isolated splenic metastasis from colorectal carcinoma is also the first reported case of splenic metastasis demonstrated preoperatively by 18FDG PET-CT fusion scanning which revealed its solitary nature as well.A 76-year-old man underwent an extended left hemicolectomy for synchronous colon cancers located at the left flexure and the sigmoid colon. The tumors were staged as IIIC (T3N2M0) clinically and the patient received adjuvant chemotherapy. During the first year follow-up period, the patient remained asymptomatic with normal levels of laboratory tests including CEA measurement. However, a gradually rising CEA level after the 14Isolated splenic metastasis is a rare finding in the follow-up of colorectal cancer patients and long-term survival can be achieved with splenectomy. From the first case report of isolated metastasis to the spleen in the setting of colorectal carcinoma by Dunbar et al.  in 1969,et al. -13. Twelet al.  reportedet al. . Most ca®, Roche) 5 × 500 mg capsule for 14 days with 7-day interval for a total of 8 courses. Blood CEA level decreased to the 3.54 ng/mL in the early postoperative period and remained normal up to the 14th month of follow-up. A rising CEA level  showed that the tumors located at the left flexure and the sigmoid colon and the endoscopic biopsies revealed the diagnosis of adenocarcinoma for both of the tumors. There was neither liver nor other remote site metastasis in the abdomen. The chest X-ray was normal. However, CEA level was higher  in the preoperative period , whereas it was in cecum and ascending colon in the remaining two. As previously stated by Avesani et al. , this ob [et al. ; a possiDiagnosis of splenic metastasis as well as other remote organ metastasis from colorectal cancer can be made by help of many current imaging studies such as US, CT or MRI. However, FDG-PET scanning provides a better detection of metastatic deposits, especially when they are too small to be shown by other radiological studies in the setting of postoperatively rising CEA level. In addition, PET-CT fusion may specifically suggest that the mass seen by the CT is tumoral in nature. On the other hand, solitary status of metastasis can be demonstrated and predicted preoperatively by PET-CT fusion scanning as in the present case. Therefore, the indication of splenectomy can be justified in such cases since the presence of multiple remote site metastases usually exterminates the chance of surgical intervention.Long-term survival rate following splenectomy in patients with solitary splenic metastasis from colorectal cancer is still unknown. However, the limited data extracted from the case reports in the literature indicate that these patients may survive up to 7 years ,12,13. TIsolated splenic metastasis is a rare finding in the follow-up of colorectal cancer patients and long-term survival can be achieved with splenectomy.CEA: carcinoembryonic antigen18FDG-PET: 18Fluorodeoxyglucose – positron emission tomographyPET-CT: positron emission tomography – computed tomographyUS: ultrasoundCT: computed tomographyMRI: magnetic resonance imagingThe author(s) declare that they have no competing interests.RG performed the operations and prepared the manuscript. FA and GK performed the histopathologic evaluations. NT participated in the redaction of the article. All authors read and approved the final manuscript.