Malignant small bowel tumors are very rare and leiomyosarcoma accounts for less than 15% of the cases. Management of these tumors is challenging in view of nonspecific symptoms, unusual presentation and high incidence of metastasis. In this case report, an unusual presentation of jejunal sarcoma and management of liver metastasis with radiofrequency ablation (RFA) is discussed.A 45-year-old male presented with anemia and features of small bowel obstruction. Operative findings revealed a mass lesion in jejunum with intussusception of proximal loop. Resection of bowel mass was performed. Histopathological findings were suggestive of leiomyosarcoma. After 3-years of follow-up, the patient developed recurrence in infracolic omentum and a liver metastasis. The omental mass was resected and liver lesion was managed with radiofrequency ablation.Jejunal leiomyosarcoma is a rare variety of malignant small bowel tumor and a clinical presentation with intussusception is unusual. We suggest that an aggressive management approach using a combination of surgery and a newer technique like RFA can be attempted in patients with limited metastatic spread to liver to prolong the long-term survival in a subset of patients. Malignant tumors of the small bowel are rare and accounts for the <2 % of total gastrointestinal (GI) malignancy ,2. The aA 45-year-old male was referred to our center with the diagnosis of suspected non-Hodgkin's lymphoma (NHL) of the bowel in December 1999. He had generalized weakness for 2-years along with recurrent vomiting, occasional constipation and melaena for last 2 months. The diagnosis was considered after an ultrasound (USG) guided fine needle aspiration cytology (FNAC) from the intra-abdominal mass done elsewhere and showed features suspicious of NHL.At presentation, patient's general condition was poor and he was dehydrated and pale. There was no peripheral lymphaedenopathy. Abdominal examination revealed an ill-defined, mobile, nontendor lump in left paraumblical region extending up to left lumber region. There was no hepato-splenomegaly. Examination of chest and cardiovascular system was normal. After initial resuscitation with crystalloids and blood transfusion, patient was further investigated.At presentation his hemoglobin was low (6.4 gm%) but rest of the routine haematological investigations were within normal limit. The chest X-ray was normal. Abdominal ultrasound (USG) showed a left upper abdominal mass lesion suggestive of bowel mass. The upper GI endoscopy was normal. The barium meal follow through examination was suggestive of intussusception of proximal jejunum and a suspected mass lesion at the leading edge of intussusceptum. An USG guided core needle biopsy of mass was performed because of earlier suspicion of NHL, which showed smooth muscle bundles with areas of necrosis. Based on the above findings and biopsy report, patient was taken up for exploratory laparotomy 3 weeks after initial presentation.Operative findings revealed an intussuscepted proximal jejunum loop 12 cm distal to duodeno-jejunal flexure and a vascular polypoidal growth measuring 6.5 × 5 × 3.5 cms on serosal surface of jejunum. Liver and spleen were normal. There was no evidence of mesenteric or retroperitoneal lymphaedenopathy, ascites or peritoneal disease. The resection of involved segment of jejunum with 5 cm margins and an end-to-end anastomosis was performed. The postoperative course was uneventful.Pathological examination of the specimen revealed two mass lesions measuring 4.5 cm & 3 cm in continuity  located on mucosal and serosal surface of jejunum respectively. Microscopically it showed spindle cell tumor with a mitotic rate of 5/10 high power fields (HPF). The tumor was negative for desmin, S-100, CD 34 and c-kit (CD117) but focally positive for actin on immunohistochemistry (IHC). The resection margins were free. The final diagnosis of malignant spindle cell tumor – leiomyosarcoma of jejunum was made. Due to negative margins and no evidence of disease elsewhere, no adjuvant therapy was planned and patient was kept on regular follow up. He was assessed clinically at three months interval and an USG of abdomen was performed six monthly. Patient remained disease free for 39 months. After that a follow-up USG showed a space-occupying lesion (SOL) in segment VII of liver and a mass lesion in left upper abdomen. The patient was asymptomatic and abdominal examination revealed no abnormality. A computerized tomographic (CT) scan of abdomen was performed which showed a 2 × 2 cms, brightly enhancing SOL in segment VII of liver  declare that they have no competing interests.SVSD, NKS: Surgical management and review of manuscript.AS, SH, SK: Review of literature and preparation of manuscript.ST, DKP: Radiofrequency ablation.All authors have read and approved the contents of manuscript.


Subcutaneous leiomyosarcoma is a rare condition that accounts for 1% to 2% of all superficial soft tissue malignancies. Approximately 10% of cases arise in the trunk, although the extremities are the most commonly affected.We report herein the case of a 31-year-old man with a subcutaneous leiomyosarcoma, measuring 124 × 105 mm, arising in the left inguinal region. A wide local excision (with a resection margin ≥ 20 mm) was performed. Histological examination of the resected specimen revealed a leiomyosarcoma with high cellularity and two mitoses per 10 high-power fields. The patient remains well with no evidence of disease 5 years and 8 months after the operation.This is the first reported case of subcutaneous leiomyosarcoma arising in the inguinal region and also one of the largest tumors reported. The experience of this case and a review of the English-language literature  suggest that a resection margin of ≥ 10 mm is recommended when excising this rare tumor. Subcutaneous leiomyosarcoma arises from smooth muscle in the walls of arterioles and veins. It is a rare tumor accounting for 1% to 2% of all superficial soft tissue malignancies . It usuaA 31-year-old man presented with a painless left inguinal tumor, which had gradually grown during the past six months. Physical examination on admission revealed a fist-sized subcutaneous tumor in the inguinal region. The overlying skin appeared normal without ulceration Figure . With a The resected tumor, measuring 124 × 105 mm, was solid, encapsulated, and a homogeneous yellowish white in color, without central necrosis and hemorrhage on its cut surface. Routine histological examination with hematoxylin-and-eosin revealed that the tumor comprised spindle-shaped cells with high cellularity in parts Figure  and two The patient had an uneventful recovery and was discharged on the 9th postoperative day. As the resection margin was negative, no adjuvant treatment was given. He remains well with no evidence of disease 5 years and 8 months after excision.Although subcutaneous leiomyosarcoma commonly arises in the lower extremities, it occasionally affects the trunk ,2,4. A ret al., [As subcutaneous leiomyosarcoma is resistant to radiotherapy and chemotherapy ,3,9, suret al.,  demonstrThe author(s) declare that they have no competing interests.KY, YS, NF, and DS took part in the operation, performed the literature search and drafted the manuscript for submission. HU performed histological examination. KH supervised the preparation of the manuscript and edited the final version for publication. All authors read and approved the final manuscript.


The adrenal gland is one of the common sites of metastasis from primary lung cancer. Adrenal metastases are usually unilateral however bilateral adrenal metastases are seen in 10% of all lung cancer patients; of these 2–3% occurs at the initial presentation of non-small cell lung cancer. Secondary tumors can disrupt the structure and function of the adrenal. This can lead to adrenal hemorrhage, which constitutes a life threatening hazard for the patient.A 59-year-old male presented with persisting abdominal pain. His initial work-up revealed significant anemia, an invasive process in the right upper lobe of the lung and large masses of heterogeneous texture, with hemorrhagic and necrotic elements in both adrenal glands. A biopsy confirmed it to be a large-cell carcinoma of the lungs. The patient developed severe leukocytosis akin to the paraneoplastic syndrome and died suddenly five days after the administration of chemotherapy.Intratumoral hemorrhage is a rare but life threatening complication of adrenal metastases and should be treated as soon as it has been diagnosed. If adrenalectomy is not feasible, combination chemotherapy should be applied as in metastatic disease. For choosing the appropriate chemotherapeutic regimen it is important to accurately achieve the diagnosis. The adrenal gland is a common site for metastases in breast, lung and renal cell carcinomas, melanoma, and lymphoma . AdrenalA 59-year-old male, working in a quarry, was admitted to the hospital complaining of abdominal pain persisting for 45 days, afternoon fever, fatigue and 8 kg weight loss during the last month. Previous medical history was non contributory. He was a heavy smoker for 45 years.At first assessment, the patient presented with disseminated dry rales and decrease of vesicular murmur in the right upper lung field. Following abdominal examination, palpable smooth surface masses were discovered in both renal areas. Laboratory tests demonstrated a significant anemia with normocytic features .Chest X-ray revealed a consolidated area in the right upper lobe of the lung. Abdomen ultrasound demonstrated large masses with foci of hemorrhagic necrosis in both adrenal glands Figure .Computed tomographic (CT) -scan revealed an invasive process in the right upper lobe of the lung Figure  and larg1 and T2 weighted sequences. Radiological features were suggestive of adrenal metastasis from lung primary  was performed which showed the masses with disseminated foci of high, heterogeneous signal intensity and pathologic enhancement after intravenous (i.v.) administration of contrast agent in both T Figures , 5. HistAdrenal metastases in the natural history of a malignant neoplasm occur in 20 – 45% of cancer patients, depending on the localization of the primary site . Up to 4The differential diagnosis of an adrenal mass incidentally discovered during imaging examination of the abdomen in patients with non-small cell lung cancer, usually comprises one or more of the characteristics detailed in Table More than 90% of the cases comprising this involve a nonfunctional adenoma or an adrenal metastasis.et al, observed that CT-scan technology has low specificity and sensitivity, which culminates in false positive and negative readings, given that 21% of true adenomas can be mistaken for metastases (density > 10 Hounsfield units) and 11% of true metastases may be mistaken for adenomas (density < 10 Hounsfield units) [Nonfunctional adenomas can be distinguished from adrenal metastasis with precision using modern imaging techniques, especially with MR imaging, where adenoma presents a low intensity signals in T2 sequences compared to hepatic parenchyma. In contrast, the adrenal metastasis presents higher intensity T2 sequence signals than that of the adenoma, relative to hepatic parenchyma. In CT-scan adenoma presents low intensity signals, below 10 Hounsfield units, compared to higher intensity signals in adrenal metastasis. Porte d units) . CT-scand units) .In presence of adrenal metastasis the disease is staged as stage IV, which demonstrates the aggressive biological behavior and systemic dissemination of secondary lesions. These are treated as any other metastatic neoplasm and the appropriate chemotherapy is started. In select patients, where the primary lung site is surgically resectable , with no involvement or the involvement of only the peribronchial and portal lymph nodes  and where the adrenal metastasis constitutes the unique indication of the disease, simultaneous adrenalectomy along with resection of the primary can increase overall survival . TechnicThe most important complication of adrenal metastases is intratumoral hemorrhage and only nine cases have been reported previously . When heA less common complication of bilateral adrenal metastases is primary adrenal insufficiency, caused by the destruction of adrenal gland architecture and function due to the development of the tumor. The main symptoms resulting from the decreased production of steroid hormones often overlap with the general indicators caused by the primary site, hindering the proper treatment of these patients. Primary adrenal insufficiency is diagnosed when serum cortisol basal values are low (<5μg/dl), and particularly when the values fail to increase within six hours following the i.m. administration of 1 mg of tetracosactride, the synthetic analog of ACTH. The treatment comprises daily administration of glucocorticoid and mineralocorticoid for life.Bilateral adrenal metastases can be found in a small number of patients with non-small cell lung cancer. Early identification of these lesions is necessary, since adrenalectomy may increase survival in select patients. Intratumoral hemorrhage is a rare but life threatening complication and should be treated as soon as it has been diagnosed. If adrenalectomy is not feasible, combination chemotherapy should be applied.The authors declare that they have no competing interests.CK – conceived the study, did the literature search and coordinated the preparation of the manuscript for submissionAT – helped in literature search, reviewed and edited the manuscript for final submissionSM and KV – helped in literature search and drafted the manuscript for submissionAll authors read and approved the manuscript.


Dedifferentiated liposarcomas represent heterogeneous tumors with lipomatous and nonlipomatous elements starkly juxtaposed. It is thought that the high grade nonlipomatous elements of the tumor portend a worse prognosis.A 19.8 kg heterogeneous retroperitoneal tumor was successfully and completely resected. Because of its extent, no additional treatment modalities were practicable. The tumor soon recurred. The recurrent tumor differed from the primary tumor in that it was more homogeneous, consisting mainly of nonlipogenic, calcific tissue.Dedifferentiated liposarcomas are known to have a very high recurrence rate. The biological behavior of dedifferentiated liposarcomas is likely dictated by the most aggressive element of these heterogeneous tumors. Sarcomas arising from the retroperitoneum are rare tumors, accounting for 10–15% of all soft tissue sarcomas . These fA 65-year-old male presented with a three-week history of progressively worsening abdominal distension. He denied any abdominal pain but stated that he noticed an increased frequency of bowel movements. His past medical history was unremarkable. On examination, he was afebrile and had a hugely distended abdomen with an immobile, nontender mass occupying all four quadrants of the abdomen. Computed tomographic (CT) scan revealed a large, heterogeneous lobulated mass occupying most of the abdomen Figure . The perWhile neoadjuvant chemotherapy and radiation comprise a frequent approach for retroperitoneal sarcomas at our institution, the extent of the tumor made this approach unfeasible. Resection was therefore planned unless an intraoperative biopsy revealed lymphoma. Resection was accomplished through a T-type incision Figure , and entThe tumor was submitted for histological examination in two parts measuring, 30.0 × 10.0 × 6.5 cm and 32.0 × 20.0 × 10.0 cm, weighing 19.8 kg in total Figure .The patient was discharged from hospital on the seventh postoperative day. He was followed in the surgical oncology outpatient clinic monthly. Four months after resection the patient had a follow-up CT scan, which demonstrated an intra-abdominal recurrence consisting almost completely of a calcified, nonlipomatous tumor Figure . The patde novo, although secondary dedifferentiation can occur with multiple recurrences of a well differentiated liposarcoma [The patient in our case manifested the dedifferentiated variant of liposarcoma. The term "dedifferentiated liposarcoma" refers to the development of a high grade nonlipogenic sarcoma juxtaposed to a well differentiated liposarcoma . The majosarcoma . CT and osarcoma . Calcifiosarcoma ,6. Otherosarcoma ,7. A furosarcoma .Among liposarcomas, the presence of features of the dedifferentiated variant strongly portends a worse prognosis. The overall 5-year survival of dedifferentiated liposarcomas is 20%; the 5-year survival of well differentiated liposarcomas is 83% . DediffeWhile generally the phenotype of the nonlipogenic component does not impact prognosis of dedifferentiated liposarcomas, the presence of calcifications has been identified as an adverse prognostic factor . In the Complete resection of the tumor is perhaps the most important factor determining long-term survival. Unfortunately, the rate of complete respectability is only about 53% . As illuDedifferentiated liposarcomas represent aggressive variants of liposarcomas. Each morphological element of these heterogeneous tumors may manifest completely different biology. The overall biological behavior of dedifferentiated liposarcomas is likely dictated by the most aggressive element, which typically resides in the nonlipomatous portion of the tumor.None declared.SK, HB, WT, and OB made substantial contributions to the intellectual content of the paper, in the interpretation of data, and in drafting the manuscript. All authors read and approved the final manuscript.


Gastrointestinal stromal tumors (GIST) constitute a large majority of mesenchymal tumors of the gastrointestinal (GI) tract, which express the c-kit proto-oncogene protein, a cell membrane receptor with tyrosine kinase activity. GI stromal tumors of the stomach are usually associated with bleeding, abdominal pain or a palpable mass.A 75-year-old male presented with upper abdominal pain and palpable mass. Computed tomographic (CT) scan of the abdomen showed a large mass arising in the posterior aspect of fundus, body, and greater curvature of the stomach. Second day after the admission, there was significant reduction in the size of the tumor, clinically as well as radiologically. Endoscopic biopsy showed large bulge in fundus and corpus of the stomach posteriorly with an opening in the posterior part of the corpus, and biopsy from the edge of the opening reveled GIST. Patient underwent curative resection.Spontaneous ruptured of giant gastric stromal tumor is very rare presentation of stomach GIST. Thorough clinical examination and timely investigation can diagnose rare complication. Gastrointestinal stromal tumors (GIST) are the most common form of mesenchymal tumors arising from the gastrointestinal (GI) wall, mesentery, omentum or retroperitoneum that express the c-kit proto-oncogene protein . This exA 75-year-old diabetic male presented with dull upper abdominal pain of one-week duration. He noticed swelling in left upper abdomen. There was no history of vomiting, fever or gastrointestinal bleeding. He had no significant medical or family history and was non-smoker and non-alcoholic. Physical examination showed a 14 × 10 cm mass palpable in epigastrium and left hypochondrium with minimal intrinsic mobility.Routine biochemical investigations were normal. Ultrasonogram and CT-scan of the abdomen showed large heterogeneous mass of 13 × 10 cm extending from the tail of pancreas to anterior pararenal space, lesser sac to gastrosplenic ligament enveloping the posterior aspect of fundus, body and greater curvature Figure . Fluid aGI stromal tumors express c-kit protein also known as CD 117, and is considered as highly specific marker that differentiates GIST from other mesenchymal tumors such as leiomyomas -10. The Assessment of malignant potential of a primary GIST lesion is difficult in many cases; even as small as less than 2 cm size GIST has certain malignant potentials . Currentet al [Imatinib mesylate, competitive inhibitors of certain tyrosine kinases including the intracellular kinases ABL and BCR-ABL fusion proteins present in some leukemia's, and platelet-derived growth factor receptors , is the et al  had repoet al . The optet al . Role ofSpontaneous ruptured of giant gastric stromal tumor is very rare presentation of stomach GIST. Clinical examination and timely investigations can diagnose this rare complication.The author(s) declare that they have no competing interests.RM, AJ: Preparation of manuscriptNR: performed upper endoscopy and biopsy and helped in preparation of draft manuscript.SOV, SS, PD: surgical management, manuscript revision for scientific contentPD, BV: Revision of manuscript and preparation of final manuscript.All authors read and approved the final version of the manuscript.


Gangliocytic paraganglioma are rare neoplasms that predominantly arise in periampulary region. Though considered benign the disease can spread to regional lymphatics.A 49 year old woman presented with melena and was found to have a periampullary mass. Endoscopic evaluation and biopsy demonstrated a periampullary paraganglioma. The tumor was resected with pylorus-preserving pancreaticoduodenectomy and was found to represent a gangliocytic paraganglioma associated with nodal metastases. In a controversial decision, the patient was treated with adjuvant external beam radiation therapy. She is alive and well one year following resection. The authors have reviewed the current literature pertaining to this entity and have discussed the biologic behavior of the tumor as well as the rationale for treatment strategies employed.Paraganglioma is a rare tumor that typically resides in the gastrointestinal tract and demonstrates low malignant potential. Due to rarity of the disease there is no consensus on the adjuvant treatment even though nearly 5% of the lesions demonstrate the malignant potential. This entity was first discussed by Dahl  in 1962 . The pat in 1962 . Long teA 49-year-old woman presented with a 6-month history of melena. During the preliminary consultation, she also complained of right upper quadrant pain, which radiated to her right lower quadrant and upper back. She underwent upper gastrointestinal (GI) endoscopy which demonstrated a 3 cm, ulcerated, ampullary mass figure . Endoscoth postoperative day.The patient underwent pylorus-preserving pancreaticoduodenectomy and lymph node dissection. The postoperative course was uneventful except for delayed gastric emptying diagnosed by frequent vomiting during postoperative days 7 to 10, and confirmed by gastrograffin swallow. These symptoms eventually resolved with conservative management and the patient was discharged from the hospital on 15Gross pathological evaluation of the resected specimen included a portion of duodenum with ampulla, measuring 16 cm. in length, and a portion of pancreatic head measuring 5 cm. in length. There was a polypoid periampullary mass protruding into the duodenal lumen, measuring 1.4 × 1.2 × 0.7 cm. On cut section, the mass was moderately firm and pink-tan. It was circumscribed but unencapsulated, and appeared to be covered by normal appearing duodenal mucosa. There was no evidence of pancreatic invasion by the tumor. A total of seven lymph nodes were also removed, 5 peripancreatic (3.0 cm in greatest dimension) and 2 periduodenal .Histologically, the tumor consisted of a complex neoplastic proliferation that included a component resembling carcinoid or islet cell tumor figure , admixedImmunohistochemical analysis demonstrated that the tumor stained positively for S-100, chromogranin, synaptophysin, and cytokeratin AE1 and AE3. No reactivity was observed with MART-1 or HMB 45. Staining for c-kit (CD 117) was performed on sections of the primary tumor and of one of the lymph nodes with metastatic tumor. The carcinoid-like epithelial cells and the spindle-shaped neurofibrillar cells stained negatively for the cell marker c-Kit. The gangliocytic cells stained strongly positive for c-kit.Due to the evidence of regional lymph node metastasis the patient was counseled regarding adjuvant therapeutic options. The treating physicians queried recognized experts in the field of radiation therapy for gastrointestinal malignancies via email and a consensus developed that external beam radiation therapy might be reasonable, although it was admitted that no data are available regarding the use of this modality for this disease entity. Ultimately, a decision was made to administer external beam radiotherapy to the abdomen in an effort to eradicate any possible residual disease not removed during surgery and to reduce the risk of locally recurrent disease. No chemotherapy was advised due to the rarity of distant metastases and the lack of response of these neoplasms to conventional systemic therapy.The patient was treated with intensity-modulated radiotherapy in 28 fractions of 180 cGy/fraction over 37 elapsed days; 6 mv photon beam energy was used. The target was a postoperative tumor bed with a 5–10 mm margin. The total dose was 5,040 cGy. She tolerated treatment well and is now symptom free more than one year following resection. Surveillance CT scans and endoscopy have been performed both of which reveal no evidence of recurrent disease.et al reported that there seems to be a slight male predominance and an average age of 54 at presentation [Gangliocytic paraganglioma is a rare, typically benign tumor of the gastrointestinal tract most commonly located in the second portion of the duodenum, with a few cases having involved the jejunum and pylorus -7. Burkeentation . This leentation .Histologically, our patient's tumor demonstrated the characteristic tricellular pattern of gangliocytic paragangliomas. These tumors are typically composed of an admixture of ganglion cells, spindle cells and epithelial cells ,10-13. TImmunohistochemically these tumors stain positive for a variety of markers as was demonstrated in this report. Such markers include those mentioned above as well as neuron-specific enolase, pancreatic polypeptide, somatostatin, myelin basic protein and neurofilament proteins ,12,13,17There is no data in the literature to guide clinicians on the use of adjuvant therapy despite the fact that approximately 5% of cases demonstrate malignant features . Since tGangliocytic paraganglioma is a rare duodenal tumor that can present with non-specific symptoms. Positive diagnosis can be obtained histologically by observing three characteristic cell types. Although this tumor is considered benign, the possibility exists for regional lymph nodal spread. Due to the rarity of the disease, no clear adjuvant treatment strategy has been determined in cases that demonstrate regional or distant metastasis.The authors(s) declare that they have no competing interests.AW wrote the original manuscript.AM performed surgical resection and prepared requested revisions of the manuscript.JM performed histopathological evaluation of the lesion and prepared photomicrographs.CT administered radiation therapy to the patient, made editorial suggestions, and supervised AW.


Lymphoma-associated hemophagocytic syndrome (LAHS) occurs in mostly extra nodal non-Hodgkin's lymphoma. LAHS arising from gastrointestinal lymphoma has never been reported. Here we report a case of gastric T-cell lymphoma-associated hemophagocytic syndrome.A 51-year-old woman presented with pain, redness of breasts, fever and hematemesis. Hematological examination revealed anemia. Gastroscopy revealed small bleeding ulcers in the stomach and the computed tomography scan showed liver tumor. She underwent total gastrectomy for gastrointestinal bleeding and the histopathology revealed gastric T-cell lymphoma. She continued to bleed from the anastomosis and died on the 8th postoperative day. Autopsy revealed it to be a LAHS.If Hemophagocytic syndrome (HPS) occurs in lymphoma of the gastrointestinal tract, bleeding from the primary lesion might be uncontrollable. Early diagnosis and appropriate treatment are needed for long-term survival. Hemophagocytic syndrome (HPS) in adults is characterized by reactive and systemic proliferation of benign histiocytes that phagocytose blood cells . It is o4/mm3, hemoglobin 10.3 g/dl (post transfusion), WBC 4,900/mm3, Platelets 51,000/mm3, serum albumin 1.5 g/dl, total bilirubin 0.6 mg/dl, AST 691 IU/l, ALT 187 IU/l, LDH 2976 IU/l, fibrinogen 134 mg/dl, FDP 10 μg/ml, and AT-III 40%. Bleeding from the stomach continued and did not stop with conservative treatment; therefore, two days later the patient underwent total gastrectomy and a partial liver resection. Histopathology of the resected specimen showed it to be a gastric lymphoma  with liver metastasis . There was no generalized lymphadenopathy. On gastroscopic examination multiple small ulcers were observed in the stomach. An abdominal computed tomographic (CT) scan showed liver tumor and a normal spleen. Hematological and biochemical examination at admission showed the following results: RBC 352 × 10in situ hybridization. The final diagnosis was EBV-related T-cell LAHS.On autopsy, malignant lymphoid cell infiltration and hemophagocytosis were observed in the liver, spleen, heart, small bowel, lung, both breasts, kidney, pancreas, uterus, and gastroduodenal lymph nodes Fig. . The bonet al, [et al, [HPS is a clinicopathological entity characterized by systemic proliferation of benign hemophagocytic histiocytes, fever, cytopenia, liver dysfunction, hepatosplenomegaly, and coagulopathy . This syet al,  are wideet al, ,3,4. On  [et al,  has propIn Japan, T-cell LAHS accounts for 48.5% of all adult LAHS . UncontrThe poor prognosis of LAHS, especially T-LAHS, is well known. The median survival time from the diagnosis is reported to be 143 and 69 days respectively in Japan . For LAHIn this case, the initial presentation was mastalgia and hence it took a considerable amount of time to reach a diagnosis. Furthermore, bleeding from the anastomosis continued leading to a rapidly progressive fatal clinical course.In HPS occurring in lymphoma of the gastrointestinal tract uncontrollable bleeding from the primary lesion might occur. Therefore, an earlier diagnosis of HPS should be made by bone marrow aspirates, and appropriate treatments should be started as soon as possible. Surgery if performed, must be performed with utmost caution.LAHS could also occur from lymphoma of the gastrointestinal tract. For long-term survival; early diagnosis and appropriate treatment are needed. Surgery if performed without a proper diagnosis could prove fatal.The author(s) declare that they have no competing interests.RF, FH, TY, RD, KO and KH were gastrointestinal surgeons.MO referred this patient to us.KK and MS performed pathological examination and the autopsy.KS was a member of the intensive care team.TH, YY, HN gave us helpful comments about the manuscript


Hepatocellular carcinoma is a common malignancy for which chronic hepatitis B infection has been defined as the most common etiologic factor. The most frequent metastatic sites are the lung, bone, lymphatics, and brain, respectively. Metastases to the chest wall have been reported only rarely.We report a patient with hepatocellular carcinoma who presented with an isolated metastatic mass on the left anterolateral chest wall in the axillary region.Metastasis of HCC should be included in the differential diagnosis of rapidly growing lesions in unusual localizations, particularly in patients with chronic liver disease even if a primary tumor can not be radiologically identified. Chronic hepatitis B has been described as the most frequent cause [Hepatocellular carcinoma (HCC) is the most frequent primary malignant tumor of the liver . It is unt cause -4. Hematnt cause ,4. Rarelnt cause -7.This report presents an unusual metastasis of HCC which presented as a mass on the left chest wall in the axillary region.3; Plt: 126 000/mm3; PT: 15.6 sec; INR:1.3; total protein: 6.5 g/dL (N: 6.4–8.5 g/dL); albumin: 2.2 g/dL (N: 3.4–4.8 g/dL); AST: 124 iu/L (N: 15–40); ALT: 52 iu/L (N: 10–40); ALP: 173 iu/L (N: 37–147); GGT: 213 iu/L (N:0–40); total bilirubin: 3.8 mg/dL (N: 0.1–1.2), and direct bilirubin: 1.57 mg/dL (N: 0–0.3). Hepatitis B virus surface antigen, IgG antibody to the core antigen, anti-HBe and HBV DNA with polymerase chain reaction were positive. HBe antigen, anti-Delta and serological markers of hepatitis C were negative.A 71-year-old male was admitted to our clinic with complaints of left shoulder pain, swelling in the left anterolateral chest wall, jaundice, weight loss, dyspnea and weakness. At three months prior to admission, he had noticed a less movable mass approximately 2 cm in diameter and then the tumor had grown rapidly, becoming increasingly painful during exertion. There was no history of expectoration, fever, or night sweats. The patient's physical examination revealed yellow discoloration of the sclerae, pitting edema up to the knees and a 5 cm × 6 cm fixed mass in the left axillary region. There was mild splenomegaly but no hepatomegaly, liver masses or ascites. Laboratory findings were as follows: sedimentation rate: 90 mm/hr; Hb: 11.5 mg/dL; WBC: 3900/mmAbdominal ultrasonography showed ascites, splenomegaly and diffusely nodular and heterogeneous echogenic patterns in the liver. There was no history of chronic liver disease. Upper gastrointestinal endoscopy was normal except for esophageal varices. Computerized tomography of the thorax revealed a mass on the left anterolateral chest wall Figure . CytologAlthough extrahepatic metastasis of HCC was reported in 18% of untreated patients in a retrospective study, metastatic lesions were found at a higher incidence in an autopsy study of deaths related to primary liver cancer . The mosAlthough bone metastases are seen in 10% of HCC patients , it infrth rib [The establishment of the diagnosis of metastatic HCC can occasionally be problematic, particularly when the primary tumor has not been identified. To the best of our knowledge there are only two reports in the literature describing cases of solitary metastasis to the chest wall from an unknown primary HCC. One patient had an HCC metastasis involving the sternum, and the other patient had a metastasis on the right 4th rib ,6.In the present patient, the etiology of HCC was due to chronic hepatitis B, and the HCC was diffuse in nature. Yuki et al. reported an association between HBs antigen positivity and diffuse-type HCC. Intrahepatic, hematogenous and lymphogenous metastases have been frequently observed in diffuse-type HCC . NeverthAFP, when elevated, usually correlates with tumor size. AFP doubling time is also closely related to tumor doubling time. A rapidly growing axillary mass, diffuse tumoral infiltration of the liver and a very high level of AFP in our patient are consistent with these observations. However, nearly one-fourth of patients with HCC may have normal AFP values .The need for liver biopsy might be questionable in the our patient. The presence of chronic liver disease with a very high AFP level was highly suggestive for HCC. However, there are several reports in the literature describing ectopic HCC without a liver origin. These patients can be treated with surgical resection and have a good prognosis ,20. FurtIn conclusion, metastasis of HCC should be included in the differential diagnosis of rapidly growing lesions in unusual localizations, particularly in patients with chronic liver disease even if a primary tumor cannot be radiologically identified.The author(s) declare that they have no competing interests.SC, involved in the patient active management and preparation of the figures. OY, carried out the literature search. SK, revised it for scientific content. KC, performed the cytological examination. MB, involved in the patient active management. AD, edited the manuscript and coordinated the submission. All authors contributed to the preparation of the manuscript. All authors read and approved the final version of the manuscript.The pre-publication history for this paper can be accessed here:


Umbilical metastasis is one of the main characteristic signs of extensive neoplastic disease and is universally referred to as Sister Mary Joseph's nodule.A 59-years-old Caucasian female underwent liver transplant for end stage liver disease due to hepatitis C with whole graft from cadaveric donor in 2003. After transplantation the patient developed multiple subcutaneous nodules in the umbilical region and bilateral inguinal lymphadenopathy. The excision biopsy of the umbilical mass showed the features of a poorly differentiated papillary serous cystadenocarcinoma. Computed tomographic scan and transvaginal ultrasonography were unable to demonstrate any primary lesion. Chemotherapy was start and the dosage of the immunosuppressive drugs was reduced. To date the patient is doing well and liver function is normal.The umbilical metastasis can arise from many sites. In some cases, primary tumor may be not identified; nonetheless chemotherapy must be administrated based on patient's history, anatomical and histological findings. Metastases to the umbilicus are universally referred to as Sister Joseph's (or Sister Mary Joseph's) nodule. Etiology is related to the presence of primary malignant disease in the abdominal cavity or occasionally in the chest and/or breast -5. HistoAlthough skin metastasis is rare and range between 5% and 9%, it is estimated that 1% to 3% of abdomino-pelvic tumors metastasize to the umbilicus ,3,5. TheSerous papillary cystadenocarcinoma has been documented previously as a cancer responsible for Sister Joseph's nodule because of the metastasis to the regional lymph nodes -9. Herei2 I.V) once a week for 16 weeks. To date, after 6 months of follow-up, the patient is doing well, the liver function is normal and tacrolimus serum level is 1 ng/ml. A recent whole body CT-scan showed regression of iliac and inguinal lymph nodes involvement.A 59-year-old Caucasian woman was admitted to our hospital for progressively enlarging but painless mass in the region of the umbilicus. The umbilical mass of approximately 5 cm in diameter and has developed two months after liver transplantation. The nodule was firm with irregular borders, wine-red shade and fixed to the surrounding tissues. The overlying skin was not ulcerated. She never smoked, consumed alcohol or used oral contraceptive pills. The gynecological history was unremarkable. The patient underwent a liver transplantation for end stage liver disease (ESLD) secondary to hepatitis C in mid of 2003. Prior to the transplant, her serum alpha-fetoprotein  and CEA concentration were measured and both were within normal limits. The transplant procedure and immediate post-transplant period were unremarkable. The post-transplant immunosuppressive regimen consisted of Tacrolimus (6 mg/day) and steroids (prednisone 10 mg/day). Two months after transplantation, during routine follow-up, physical examination revealed multiple subcutaneous satellite nodules in close proximity to the umbilical region and lower abdominal wall. There was bilateral inguinal lymphadenopathy. A plain anterior/posterior chest x-ray was normal. Ultrasound of the abdominal cavity showed a 4 × 4 cm mass below the umbilicus and additional smaller nodules confined to the lower abdominal wall. Paraaortic lymph nodes were not identified. The echogenicity of the mass consisted of alternative hyper- and hypoechoic with poorly defined edges. However, the general condition of the patient was good and the laboratory test showed: hemoglobin level 9.6 g/dl, total bilirubin 0.6 mg/dl, conjugated bilirubin 0.1 mg/dl, Alkaline Phosphates 273 U/l, GGT 40 U/L, AST 36 U/L, and ALT 56 U/L. There was serological evidence of past hepatitis C virus infection. The biopsy of the umbilical mass showed features characteristic for a poorly differentiated serous papillary cystadenocarcinoma. An extended resection of the umbilical and paraumbilical metastasis was performed. In addition, extensive investigation to detect the primary neoplastic origin was initiated. Tumor marker evaluation consisted of: CEA 0.22 [normal 0–3.7 ng/ml], alpha feto protein 1.49 , CA125 27.73 , CA 19.9 was 8.61 , CA 15.3 was 22.48 . Multiple tumor marker measurements were obtained and each time similar results were reported. A computed tomographic (CT) scan was unable to demonstrate any lesion that could be considered a primary source of the metastasis to the umbilicus. Trans-vaginal ultrasonography showed a normal uterus and ovaries; ultrasonographic examination of thyroid and breast did not show any pathological changes as well. In spite of laborious efforts, the tumor's primary site was not found at that time. However, considering the histological results, the umbilicus metastasis could be attributed almost entirely to the ovarian cancer stage 4, since the main primary site in females are the ovaries. In order to prevent further tumor extension and expansion, tacrolimus was reduced from 6 mg/day to 1.5 mg/day. In addition, chemotherapy with Taxol was initiated  declare that they have no competing interests.FP: conception and design, interpretation of data, drafting the article and revisingEA: interpretation of data, drafting the article and revisingSDD: acquisition of data and revisingNM: interpretation of data, design and coordination and helped to draft the manuscriptGB: collection of data, design and coordination and helped to draft the manuscriptRM: collection of data, design and coordinationMM: acquisition of data and drafting the articleTJ: drafting the article and interpretationFR: acquisition of data and designMC: interpretation of data, drafting the articleUV: drafting the article, revising and supervision


Gastro-bronchial fistula (GBF) is a rare and devastating complication following esophagectomy. Making the correct diagnosis is difficult and there is no agreement on the treatment for this rare condition.We report the case of a 56-year-old man who presented with features of repeated aspiration and chest infections six years following an esophagectomy for Barrett's esophagus. Despite extensive investigations the cause of symptoms was difficult to determine. The correct diagnosis of fistula from stomach to right main stem bronchus was made at bronchoscopy under general anesthesia. After ruling out local recurrence of cancer, a successful primary repair was carried out by resection of fistula and direct repair of gastric conduit and bronchus. He is well after 6 months of treatment.Late development of gastro-bronchial fistula is a rare complication of esophageal resection that may be difficult to diagnose.Surgical resection and direct closure is the treatment of choice, although the method of treatment should be tailored according to the anatomy of the fistula and the patient's condition. Gastrobronchial fistula (GBF) following esophagectomy for malignant disease is a recognized complication. However benign GBF are rare and the literature on this complication is limited consisting of a handful of case reports. Their mode of presentation and management are varied and there is no single treatment method, which is widely accepted. We report a case, which was successfully treated by direct primary repair. A brief review of literature is presented.A 67-year-old man with past history of Ivor Lewis esophagectomy 6 years previously for a Barrett's esophagus presented with dysphagia, recurrent cough, regurgitation and weight-loss. Endoscopy suggested a benign anastomotic stricture, for which he underwent dilatation. However his symptoms worsened and he continued to lose weight in spite of supervised dietary therapy. Aspiration was suspected and a barium swallow was performed. This demonstrated pharyngeal in-coordination, as the probable cause of aspiration. A repeat endoscopy was non-contributory. A feeding jejunostomy was inserted to prevent continued weight loss. In spite of this, he continued to have repeated chest infections resulting in extensive bronchiectatic changes in the right lower lobe.A tracheostomy was planned at this stage to prevent aspiration. Whilst he was being intubated for the procedure, air was noted to escape from the right bronchus. A bronchoscopy revealed the presence of the GBF, with the bronchial opening in the right main stem bronchus located about 1 cm below the tracheal bifurcation and the gastric opening midway down the lesser curvature of the gastric conduit. The temporal sequence of events suggests that fistulation had occurred at the time of anastomotic dilatation, and thus the worsening of symptoms following the procedure. The fistula was however, difficult to diagnose in spite of extensive investigations leading to unexpected finding in the anaesthetic room.Given his debilitated condition and poor respiratory function stenting was considered to occlude the fistula. However as the fistula was positioned in the middle of the gastric conduit Figure  it was nAt operation a right-sided re-thoracotomy was performed and careful dissection carried out to display the anatomy of the GBF Figure . The fisThe patient was discharged home after two weeks and remains well six months later.GBF is a rare complication in patients with an intra-thoracic oesophagogastric anastomosis after esophageal resection. Recurrent malignancy is the commonest cause of this complication. Non-malignant cases may occur early or late. In the early postoperative period GBF usually arises as a result of the extensive dissection, ischemia  or surgiThe usual modes of presentation are cough associated with eating, dyspnoea, haemoptysis and recurrent chest infections. The fistula may connect at any site in the respiratory tract, from trachea down to lobar bronchus. The fistula may be difficult to locate and may not be visualized at endoscopy of respiratory or upper GI tract. In such cases a methylene blue dye test may show bluish sputum when the patient is asked to swallow the dye. CT scan or upper GI contrast radiology is also helpful in arriving at the correct diagnosis. Multiple biopsies help to rule out recurrence of malignancy as the cause. Left untreated, GBF is usually fatal due to chronic pulmonary sepsis and one should not rely on conservative treatment .Patients often present in poor general condition with malnutrition and chronic pulmonary infection. Prompt institution of broad-spectrum antibiotic cover, gastric drainage, attention to fluid and electrolyte balance, nutritional support and chest physiotherapy are essential steps in preparation for definitive surgery. In acute cases with the presence of gross mediastinal sepsis cervical oesophagostomy and return of the stomach tube to abdomen with débridement is performed. The continuity can be restored at a later date with an alternative conduit e.g. colonic interposition.The ideal operation consists of re-thoracotomy and resection of fistula with direct closure of the openings in the esophagus and the respiratory tree, preferably with an intervening viable tissue. A variety of tissue and pedicles of muscles like pectoralis major, intercostal, latissimus dorsi  and sterLate development of GBF is a rare complication of esophageal surgery that may be difficult to diagnose. Surgical resection and direct closure is the treatment of choice. However where patients are debilitated or if there is insufficient length of airway available for resectional surgery, alternative, less invasive endoscopic stenting procedures may be used .Late development of gastro-bronchial fistula is a rare complication of esophageal resection that may be difficult to diagnose.Surgical resection and direct closure is the treatment of choice, although the method of treatment should be tailored according to the anatomy of the fistula and the patient's condition.GBF : Gastrobronchial FistulaThe author(s) declare that they have no competing interests.MPD collated the information, searched literature and wrote the manuscript.RJ assisted in literature search, writing of the manuscript, editing the final script and doing the final submission.SG and PK assisted in providing a critical appraisal and review of the manuscript.The pre-publication history for this paper can be accessed here:


Oncocytomas are benign tumors of the kidney that are usually diagnosed postoperatively due to differential diagnostic problems from renal cell carcinoma. Although the latter are neoplasms that have been associated with erythrocytosis in 3.5% of cases, there are no reports in the literature about a similar occurrence in oncocytomas.In this case report we present a unique case of a right lower pole oncocytoma associated with erythrocytosis. Erythrocytosis subsided after partial nephrectomy.Erythrocytosis can sometimes occur in association with renal oncocytomas. Under normal circumstances, erythropoietin is produced in the kidney along with a variety of other substances like renin and 1,25 dihydroxycholecalciferol. Paraneoplastic syndromes such as hypertension, anemia, pyrexia and hypercalcemia are described in up to 20% of patients with renal cell carcinomas, and most of them subside after radical nephrectomy. Erythrocytosis occurs as a paraneoplastic syndrome in 3.5% of patients with renal cell carcinoma. Polycythemia is usually attributed to pathologic erythropoietin production by the tumor or by the adjacent normal parenchyma in response to hypoxia induced by tumor growth [6/mm3. The mean corpuscular volume (MCV) was within normal levels: 88,9 fl. Oxygen saturation was 99%. The patient was a non-smoker. Hemoglobin was 21.7 g/dl, with all other hematological, biochemical and urine examinations being normal, apart from a small cholesterol (232 mg/dl) and GPT (72 U/L) level elevation. The patient was taking no medications and his medical and surgical history was clear.A 41-year-old man presented in March 2005 to the internist due to frequent headaches and plethoric face. The CBC results showed a hematocrit of 65.1%. The number of red cells was elevated: 7,32 × 10Findings of the abdominal ultrasound examination were normal, apart from a solid mass on the lower pole of the right kidney, measuring 8.1 × 6.1 cm, with calcifications. The left kidney was normal. On subsequent CT and MRI examination this mass was enhancing. It measured 6 × 6 × 7 cm and the differential diagnosis included oncocytoma and renal cell carcinoma. There was evidence of a tumor pseudocapsule with similar uptake to that of the normal renal parenchyma and a central part showing homogeneously diminished contrast uptake. No central scar was detected. Furthermore, the mass appeared to distort the lower pole calyces of the right kidney. No renal vein invasion or lymph node enlargement was noted. Erythropoietin level was 17.4 mIU/ml .Subsequently, after phlebotomy (twice) to prevent perioperative thrombotic complications, the patient was subjected to open partial nephrectomy via a flank approach. This decision was based on the fact that the patient was young and had a relatively higher probability to develop chronic renal insufficiency had he been subjected to radical nephrectomy. Another reason was the favorable peripheral location of the tumor and some recent evidence that tumors>4 cm can be treated by partial nephrectomy . Macroscth postoperative day after an uneventful recovery period. On follow-up examination 1, 3 and 6 months later the hematocrit remained normal  and after 9 months the hematocrit was 42.5%. Follow-up MRI at 9 months was normal and erythropoietin levels dropped to 14.5 mIU/ml one year after the operation.The hematocrit levels dropped to normal levels in the perioperative period (probably due to phlebotomy and blood loss during the operation). Because of the need to closely follow his postoperative status, the patient was discharged from the hospital on the 11About 7% of surgically excised renal neoplasms are oncocytomas . Most aret al, 33% of renal cell carcinomas had elevated serum erythropoietin, however, no correlation between erythrocytosis and elevated serum erythropoietin was found [Paraneoplastic phenomena have not been reported for renal oncocytomas. However, paraneoplastic manifestations are present in up to 20% of renal cell carcinomas, sometimes being the first clinical presentation, and most of them subside after surgical treatment of the tumor . Erythroas found . From a This is the first report of an oncocytoma with associated erythrocytosis. In general, erythrocytosis can have various causes like smoking, living in high altitude, sleep apnea, chronic lung disease, hemoglobin mutations or renal disease, which were excluded during diagnostic workup. The serum erythropoietin levels were within the normal range in this case and dropped by 17% one year after the operation, in parallel with stabilisation of the hematocrit to normal levels. The possibility that erythrocytosis was a result of the response to tumor-induced hypoxia can not be excluded. The contribution of erythropoietin to erythrocytosis in this particular case is unclear, however, the fact that the erythrocytosis was corrected after tumor resection strongly suggests that this was a tumor-related occurence.The author(s) declare that they have no competing interests.DR conceived of the case report, performed the operation and drafted the manuscript. KT was involved in postoperative follow-up. AT was involved in postoperative follow-up and drafted the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon fetal development anomaly of the terminal respiratory structures. The large cyst type usually occurs in stillborn infants or newborn infants with respiratory distress. Cases of CCAM have been previously described in adulthood, more often type I with multiloculated cystic lesions.We report a case of type I CCAM presenting as a single, expansive cystic mass in the middle pulmonary lobe in a 38-year-old man, revealed by persistent cough and haemoptysis. Computed tomographic scan showed a single cyst with air fluid level, occupying the lateral segment of the lobe. When the type I CCAM is a single cyst, other cystic pulmonary lesions must be excluded. The intrapulmonary localization and the absence of cartilage in the cyst wall are conclusive findings of CCAM. The pathogenesis, management and differential diagnosis with other lung malformations are discussed along with a review of the literature.The literature data confirm that surgical resection is the treatment of choice in all cases of CCAM and in the cases of cystic pulmonary lesions with uncertain radiological findings, in order to perform a histological examination of the lesion and to prevent infection and the potential neoplastic transformation. The development of the respiratory system begins at 3 weeks of gestation, and aberrations in developmental processes may give rise to a group of structural abnormalities collectively referred to as bronchopulmonary foregut malformations. These lesions include congenital cystic adenomatoid malformations (CCAMs), sequestrations and infantile lobar emphysema. All congenital malformations of the lower respiratory tract are usually diagnosed and managed antenatally, in the newborn period, in infancy or in childhood. In a small number of patients, such malformations may go unrecognized in infancy, childhood and rarely in adulthood ,2. In thWe report a case of type I CCAM presenting as a single, expansive cystic mass in the middle pulmonary lobe in a 38-year-old man.A 38-year-old man was admitted to the Division of Surgery in February 2006 because of persistent cough and haemoptysis. Bronchoscopic examination, bronchoalveolar lavage, sputum and bronchial aspirate were negative for malignancies. Contrast-enhanced computed tomography scan disclosed a single expansive cystic mass, 7 cm in diameter, with air fluid level, occupying the lateral segment of the right middle lobe of the lung with compression of the medial segment and of the adjacent segments of the lower lobe. The cystic wall showed intraluminal projections and a sessile nodule 1 cm in diameter  accounts for 50% to 70% of cases, is characterized by multiple large cysts, (up to 10 cm) or a single dominate often multiloculated cyst, with a pseudostratified ciliated columnar epithelium, resembling the distal bronchial tree and proximal acinus, with normal alveoli between the cysts. Radiographic analysis may preoperatively suggest the diagnosis, especially when a multicystic pattern is evident. When the cystic lesion is single, the differential diagnosis with the congenital parenchymal cysts and bronchogenic cysts is not possible only on the base of the radiological features;2. Type II (microcystic type) represents 20% to 40% of cases and shows multiple tiny cystic structures usually much less than 2 cm in diameter. This type can associate with high frequency to other congenital anomalies, and the prognosis is poor;3. Type III (solid type) represents more than 10% of cases and consists of a bulky firm, solid mass with cysts less than 0.5 cm in diameter, mimicking the terminal bronchioles and the alveolar ducts of the pseudoglandular period,10,11; iCCAM is most commonly found in the neonatal period and up to 90% of diagnoses are made within the first two years of life ,13.The adult form of CCAM shows a wide radiological expression with extreme difficulty of preoperative diagnosis . Some auAfter a complete revision of the 45 cases of post-natal and adult CCAM reported in the literature, we can make the following considerations:1. Type I is the most frequent CCAM type, representing the 64% of the described cases;2. Only once a single, unilocular cystic mass was reported as the unique clinico-pathological manifestation in the lower right lobe ;3. CT scan provides a morphological assessment of the lung cavities, but it is inadequate to differentiate CCAM from other cystic lung diseases with similar imaging features. The differential diagnosis is essential, since malignancy has been associated with large cyst-type CCAM, including rhabdomyosarcoma  and bronWhen CCAM type I consists of a single large cyst, the differential diagnosis includes lung and bronchogenic cysts. The exact localization of the disease and the histological examination can be crucial for the correct diagnosis. Bronchogenic cysts are generally extrapulmonary, usually located in the right paratracheal or carenal region and may cause symptoms by bronchial compression or when infected. The histology of bronchogenic and lung cysts shows a columnar to cuboidal respiratory epithelial lining, surrounded by a fibromuscular wall which contains islands of cartilage and nests of bronchial glands. The single cyst of CCAM type I is always intrapulmonary and the wall is free of cartilage. The pathogenesis of the lesion is unknown. The absence of bronchiolar cartilage in the cystic wall suggests that in the type I there is an embryological alteration before the sixteenth week of intrauterine life, when the cartilaginous bronchi are formed. The histological picture of the present case supports this hypothesis.In conclusion, the literature data confirm that surgical resection is the treatment of choice in all cases of CCAM and in the cases of cystic pulmonary lesions with uncertain radiological findings, in order to perform a histological examination of the lesion and to prevent infection and the potential neoplastic transformation ,21.The author(s) declare that they have no competing interests.LM, IP, SL, SD, AC and FDN participated equally in the design of the report and in drafting the manuscript. All authors read and approved the final manuscript.


The authors describe the case of a large hepatocellular adenoma diagnosed in a 30-year old woman who came to us complaining of acute pain in the upper abdominal quadrants. The patient had been taking an oral contraceptive pill for the last ten years. We present the clinical features, the diagnostic work-up and the treatment prescribed. During the last 30–40 years, there has been an increase in the incidence of hepatocellular adenoma (HCA), a rare primary benign hepatic tumour, in young and middle-aged women, which has been associated with the use of oral contraceptives (OCs) .These tumours may be found accidentally, or they may present with pronounced symptoms, such as acute abdominal pain or haemorrhage due to the rupture of the tumour.We describe the case of a 30 year-old female patient, who had been taking oral contraceptives for the last ten years and who came to our observation with severe upper abdominal pain as the main symptom of a large HCA.A 30-year-old woman was referred to us for the appearance of frequent attacks during the previous week of acute pain in the upper abdominal region; she also complained of nausea and had vomited after eating on several occasions. The patient had no history of abdominal disease and reported that she had been taking a contraceptive pill for the last ten years.Clinical examination of the abdomen revealed a painful, palpable mass with regular margins of about 6–8 cms. in diameter located in the upper quadrants, between the epigastric region and the left hypochondrium.6 × μl; Hb 12,9 g/dl), γGT, total and fractionated bilirubin, cholinesterase, glycaemia and serum electrolytes were all within normal limits; markers for hepatitis B and C were negative.Laboratory tests at hospital admittance showed a slight increase in serum transaminase , whereas haemochrome  revealed a neoformation of about 8 cms in the left hepatic lobe, with clear margins; the mass presented a dyshomogeneous echostructure, with hypoechogenic areas alternating with hyperechogenic zones.An abdominal CT scan using a contrast medium and triphasic techniques, performed 48 hours after admittance, confirmed the presence of a nodular lesion of about 10 cms in diameter in the left lobe of the liver compressing the gastric corpus and fundus but with no signs of infiltration. The lesion showed a dyshomogeneous density with irregular enhancement in the arterial phase and wash-out in the late phase. No infiltrations were observed, either in the parenchyma or within the vascular structure Fig. .The diagnostic work-up, together with the medical history of the long-term use of OCs, led us to suspect a large HCA.We therefore performed a liver biopsy for histopathologic examination which showed a shaft of hepatic tissue containing a few vacuolated hepatocytes within an area of widespread necrosis; no portal or biliary structures were present. Morphological examination of the specimen suggested a diagnosis of hepatic adenoma, although this could not be considered as conclusive.6 × μl; Hb 9,90 g/dl), together with a further increase in transaminase  and an increase of LDH (810 IU/l).Four days after admittance, the pain was still present and there was also a reduction of red blood cells and haemoglobin  was therefore performed with the use of a paramagnetic contrast medium, and this confirmed the presence of a lesion of 12 cms, with clear margins, taking up all the II and III segments of the liver Fig.  and alsoNot only was the tumour extremely large and causing considerable pain, most probably due to the distension of Glisson's capsule, but laboratory tests and imaging also revealed endotumoral bleeding, indicating a probable rupture of the neoplasia, and we thus decided that surgery was indicated.At this point it was thus decided to perform an angiographic study in order to check hepatic vascularisation. Catheterisation of the coeliac tripod, performed during preoperative angiographic examination of the hepatic vascularisation, showed the presence of a large dyshomogeneously hypervascular neoformation within the left hepatic lobe associated with the presence of an anatomic variant in which the left hepatic artery originated from the right gastric artery. Selective catheterisation of the common hepatic artery did not reveal any opacity within the neoformation Fig. .The patient therefore underwent left hepatic lobectomy Fig.  and focaThe anatomopathological examination showed a mass of 11.5 cms with a smooth, regular external surface and well-defined margins; the walls were of a yellowish colour and in the centre there was a wide area of necrotic, haemorrhagic tissue extending almost as far as Glisson's capsule. Microscopic examination showed the presence of mature, vacuolated hepatocytes; no portal or biliary structures were present, which confirmed the diagnosis of hepatic adenoma. The smaller nodule in the VI segment presented exactly the same histopathological features.Following surgery, the patient stopped taking OCs and ultrasound follow-up examination at six months did not reveal the presence of any further focal lesions.HCA is a primary benign tumour of hepatocellular origin rarely seen before the introduction of OCs in the 1960s . More ofSeveral diagnostic procedures, such as US, CT and MR, can indicate the presence of an HCA, but this diagnosis must be confirmed by the histopathological examination.Most HCAs are usually first detected at US. The US HCA may appear as a hyperechoic lesion or else as a hypo- or anechoic solid mass, well-circumscribed and rarely capsulated. A mixed appearance is typical of voluminous and dyshomogeneous masses presenting haemorrhage or necrosis; calcifications are rarely present.Colour Doppler US may provide some clues for distinguishing HCA from FNH, since the former shows a continuous venous flow in the central portion and either a pulsatile or continuous peripheral flow ('basket pattern'). These findings are absent in FNH, in which colour Doppler US may show a typical spoke-wheel arterial pattern of vessels .On pre-contrast CT scans, HCA has a varied and non-specific appearance, possibly with hypoattenuating areas due to the presence of fat, previous haemorrhage or necrosis, whereas recent haemorrhage or large amounts of glycogen are observed as hyperdense areas. On contrast CT, HCA often shows substantial enhancement during the arterial phase, decreasing during the portal phase and gradually becoming iso- or hypodense in the liver on delayed scans. In some cases, there may be a thin, continuous hyperdense rim due to the presence of a peripheral capsule .HCAs frequently show heterogeneous hypointensity both on un-enhanced T1-weighted and T2-weighted images due to the presence of fat, haemorrhage, or necrosis . SometimThe various imaging techniques, which are extremely important for the diagnosis of HCA, are particularly useful in all those cases where pain is among the symptoms. The lesion should be kept under strict observation and any rapid increase in volume with endotumoral bleeding linked to a reduction of haemochrome parameters should suggest an immediate surgical approach in order to avoid complications which might be brought about by possible rupture of the tumour.In the case observed by us, the US and the CT scan using a contrast medium and triphasic techniques were essential for reaching a diagnosis. The ultrasound and radiographic features of the lesion, correlated to a medical history of the long-term use of Ocs, made it possible right from the beginning of the diagnostic work-up to suspect the presence of an HCA. Nevertheless, we considered that this diagnostic hypothesis should be confirmed by means of a biopsy, since, although the risk of malignant transformation of an HCA is fairly low, it may occur, and would be an important indication for a surgical approach. In any case, this treatment is not particularly invasive, is well-tolerated and does not generally involve a high rate of complications.During hospitalisation, an MR examination performed because the patient was becoming progressively more and more anaemic showed a further increase in the size of the lesion and probable endotumoral bleeding. Since this fact indicated a surgical approach due to the high risk of endoperitoneal rupture of the lesion, we immediately performed selective angiography of the liver in order to evaluate vascularisation of the organ and of the tumour. This examination revealed the presence of an anatomic variant in which the left hepatic artery originated from the right gastric artery, and showed that the neoplasia was vascularised by the left hepatic artery.The therapeutic approach to HCA is still not clear. For asymptomatic patients, conservative treatment requires stringent follow-up with ultrasonography of the liver and only in the case of further growth is surgical treatment indicated. In a recent review regarding the indications for a surgical approach towards benign hepatic neoplasias, it has The role of elective surgical resection for HCA is still controversial and mainly depends upon the risk of complications, the uncertain diagnosis and the presence of symptoms related to tumour size and site, particularly with regard to the risk of rupture and resulting haemorrhage ,9. ElectIn the case observed by us, the persistent pain caused by the tumoral growth and the resulting distension of Glisson's capsule, together with progressive anaemia brought about by endotumoral bleeding, led us to suspect the possible rupture of the tumour with consequent haemoperitoneum; immediate surgery was thus considered the treatment of choice.In conclusion, HCA is a rare benign tumour of the liver, generally involving a history of a prolonged use of OCs. Accurate diagnostic imaging almost always provides a correct differential diagnosis from other benign tumours of the liver. Although there is still some doubt regarding the therapeutic approach to asymptomatic patients, surgery is probably indicated in large-size HCAs with or without abdominal symptoms in order to avoid certain complications such as haemorrhage or rupture of the tumour.The author(s) declare that they have no competing interests.LS, CC, and GG performed the operation, assessed the didactic importance of the clinical case and therefore acquired the data; TVB and FC were responsible for the imaging; SLP and OC. TVB and FC were involved in drafting the manuscript and its revision; MAL gave the final approval of the version to be published.All authors read and approved the final manuscript.


Primary leptomeningeal astrocytomas are rare intracranial tumors. These tumors are believed to originate from cellular nests which migrate by means of aberration, ultimately settling in the leptomeningeal structure. They may occur in both solitary and diffuse forms. The literature reports only fifteen cases of solitary primary intracranial leptomeningeal astrocytomas.The authors report the case of a seventy-eight year-old woman with a polycystic variant of a solitary primary intracranial leptomeningeal astrocytoma. The first neurological signs were seizures and aphasia. CT and MRI scans demonstrated a fronto-parietal polycystic tumor adherent to the sub arachnoid space. A left fronto-temporo-parietal craniotomy revealed a tight coalescence between the tumor and the arachnoid layer which appeared to wrap the mass entirely. Removal of the deeper solid part of the tumor resulted difficult due to the presence of both a high vascularity and a tight adherence between the tumor and the ventricular wall.A new case of a solitary primitive intracranial leptomeningeal astrocytoma of a rare polycystic variant is reported. Clinical, surgical, pathologic and therapeutic aspects of this tumor are discussed. Moore was the first in 1954 to describe a diffuse form of a primary leptomeningeal astrocytoma (PLA) [Gliomas very rarely arise from the meninges and, to date, their histogenesis remains controversial. The diffuse forms normally affect the entire leptomeninges, giving a gliomatosis appearance and a macroscopic similarity to meningitis , while tA seventy-eight year-old woman complained of a two-week history of partial seizures in right arm with diffusion to the entire right side. The seizures had been preceded by a right-sided dysesthesia and dysphasia. At admission, the patient was in G.C.S. 14. Speech ability and cranial nerve functions were normal. No gait disturbances were present. Limb strength and sensory examination were normal. Deep and superficial reflexes were bilaterally normal and symmetrical.A brain CT scan revealed a large, round mass (seven cm. in diameter) in the left fronto-parietal area causing a shift of the midline structures. A thickening of the meninges was detected around the polycystic mass. The left parietal inner bone appeared thin. Figure .A brain MR highlighted the polycystic shape of this mass Figure .Cerebrospinal fluid (CSF) was studied by means of an intracranial drainage for ICP measurement. Analysis of CSF showed an increased protein level and a reduced glucose level. Cytological examination showed no malignant cells. Gram's and Ziehl-Neelsen stains were negative.After implantation of a cardiac pace-maker necessary because of hypokinetic arrhythmia, a left fronto-temporo-parietal craniotomy was performed. The meninges adhered tightly to a grey, extra-axial, polycystic neo-formation. Once the meninges were opened, it was possible to penetrate into a large cystic cavity composed of smaller cysts ranging in size between one and three centimetres. A few cysts were communicating whereas others were isolated. The cysts contained a dense yellowish liquid. Furthermore, the cyst walls were quite thin and their inner wall showed a dense, veil-like, white tissue connected with a solid part of the tumor. The core of the tumor consisted almost entirely of a dense, intra-cystic liquid which was suctioned. Removal of the deeper solid part of the tumor resulted difficult owing to the presence of both a high vascularity and a tight adherence between the tumor and the ventricular wall.The immediate postoperative course was good even though, on the fourth day after surgery, despite carbamazepine, the patient suffered a new episode of dysphasia which spontaneously resolved.Anatomical specimens appeared rust-coloured and were composed of many cellular areas. Microscopic examination revealed leptomeningeal thickening with a diffused, well-differentiated and fairly glial cellular proliferation in the subarachnoid space Figure . NuclearA primary leptomeningeal astrocytoma (W.H.O. grade II) was diagnosed.The patient was discharged from hospital ten days after surgery in good clinical conditions. At the two-year follow-up, the neurological exam was normal and a total body CT scan revealed no distal metastases.PLA is considered a neoplastic transformation of glial heterotopias ,4 represThe cellular nests may contain oligodendrocytes, ependymal cells ,18, as wSLAs are believed to have a non-heterotopic origin and are considered a leptomeningeal spread of intra-axial gliomas. These forms occur in 20–25% of patients suffering from extensive cerebral gliomas .Cooper and Kernohan first dictated the main criteria on which to base the diagnosis of PLA: no apparent attachment of extramedullary meningeal tumor to the neural tissue, no evidence of primary neoplasia within the neuraxis and the existence of distinct leptomeningeal encapsulation around the tumor .It is noteworthy that PLAs may have two well established anatomoclinical forms: nodular form, first described by Bailey and Dietrich as "a solitary or focal leptomeningeal gliomatosis, defined by limited tumor masses in cranial or spinal leptomeninges," ,24 and aThe literature reports only fifteen cases of intracranial solitary PLAs which are summarized in Table Clinical presentation of PLAs includes a variety of neurological symptoms but headache, seizures and increased intracranial pressure are the most common observed clinical signs. In many cases the MRI and CSF studies are unable to clarify the diagnosis of these tumors. Tumor biopsy often becomes imperative to confirm the diagnosis.The lack of symptom specificity as well as the variability of the clinical onset makes the diagnosis of these tumors difficult. This is why most of the described cases were diagnosed post-mortem.The reported case represents a new case of solitary intracranial SLA and, in particular, an uncommon polycystic variant of it. The main symptoms were seizure and aphasia. The CT scan and MR scan supported the diagnosis of an extra-axial tumor. The microsurgical dissection demonstrated a strong coalescence between the tumor and the arachnoid layers, which enveloped the polycystic mass entirely. The histological studies confirmed a strong coalescence between the tumor-cell proliferation and the arachnoid layers.Due to the aforementioned lack of specificity concerning clinical, radiological and CSF analysis, the pre-mortem diagnosis of PLAs still remains a challenge for clinicians, who often have difficulty in differentiating these lesions from infectious or autoimmune pathologies. The literature reports almost 30% of cases with no clinical established diagnosis.Among infections, chronic tuberculous meningitis is the most difficult differential diagnosis from leptomeningeal tumors. Indeed, even in tuberculous meningitis the clinical onset consists of subacute headache, drowsiness, confusion and meningism. CSF examination typically shows increased proteins, decreased glucose concentration and pleocytosis .Unfortunately, when CSF stains for tuberculous are negative, differential diagnosis between tuberculous or malignant meningitis and leptomeningeal tumors is based solely on the responsiveness to antituberculous chemotherapy. Indeed, these patients are treated with steroids and anti-tuberculous therapy until clinical and radiological improvement has been achieved. The authors are in agreement with Rogers and Wechsler who believe that a search for malignancy is mandatory in those patients who had failed to respond to the anti-infectious and anti-inflammatory treatment although iterative CSF examination has demonstrated no neoplastic cells ,28.Other intracranial glial tumors may have a cystic pattern. Among these, pleomorphic xanthoastrocytoma (PXA) is one of the most frequently observed in supratentorial spaces, especially in elderly patients. Indeed, in a series of 20 patients reported by Rostomily all the tumors were supratentorial and 5 were cystic . In a reFrom a macroscopic point of view, although PXAs are usually superficial in location, they are an intra-axial neoplasm only partly occupying the leptomeninges and with a natural tendency to involve perivascular Virchow-Robin spaces. On the contrary, the diagnosis of a PLA requires "no evidence of primary neoplasia within the neuraxis and the existence of distinct leptomeningeal encapsulation around the tumor" .Since the first description by Kepes in 1979, the diagnosis of PXAs should comply with a few criteria such as: 1) highly pleomorphic tumor cells, 2) presence of lipid-laden, foamy, or "xanthomatous" tumor cells, 3) absence of necrosis, 4) rare or no mitoses, 5) GFAP positivity in some tumor cells, and 6) rich pericellular or stromal reticulin staining . Even thFrom a therapeutic point of the view, the literature debates chemo and/or radiotherapy protocols for PLA.To the authors' knowledge, only six cases of these rare tumors were treated with adjuvant chemo-radiotherapy ,33-37. AIn the reported case the authors decided against adjuvant therapies after radical surgery considering the lack of tested protocols of chemo-radiotherapy in PLAs.ConclusionsThe present case is an interesting new case of a PLA considering both the patient's good outcome observed at the two-year follow-up as well as the fact that no chemo and/or radiotherapy was undergone. The patient's positive outcome suggests that polycystic variants of a PLA might be associated with a better prognosis compared to other PLAs.The author(s) declare that they have no competing interests.ADT: conceived the case report, participated in the surgery as first operator and drafted the manuscript. GO: participated in the surgery and in clinical management of the patient.    CDT: participated in the clinical management of the patient and in the literature review.SL: participated in the clinical management of the patient and in the literature review.AC: examined the histological specimens and participated in the literature review.PC: proofread the manuscript.All the authors have read and approved the final manuscript.


Pituitary tumours may present with a variety of neurological and endocrinological signs and symptoms. It is very rare however for them to present with sudden onset painful diplopia. The current literature and possible mechanisms for this are discussed.We describe a case of a pituitary mass which presented with sudden onset painful diplopia with an associated restricted pattern on Lees Chart testing. This led to an initial working diagnosis of orbital myositis.Awareness of different modes of presentation of pituitary lesions is important so that appropriate imaging may be requested and delay in diagnosis prevented. It is rare for a pituitary tumour to present with painful diplopia due to a partial third nerve palsy. We present such a case in which the initial lack of pupil involvement or ptosis combined with the restricted painful ocular movements, led to an initial suspected diagnosis of orbital myositis. This is a previously undocumented mode of presentation of a large non-secretory pituitary mass. The history is unusual in its acute, painful onset and examination findings were in keeping with a restricted ocular motility defect.A 47 year old female who was otherwise healthy presented with a sudden onset vertical diplopia associated with right sided headache and right eye pain. On examination she had a right hypotropia measuring 20^ for distance. Eye movements were limited in dextroelevation with associated discomfort. Ocular motility testing with a Lees chart showed a restrictive profile figure . There wThyroid function tests and inflammatory markers were within normal limits . An initial diagnosis of orbital myositis was suspected due to the marked painful limitation in elevation of the right eye. MRI of the orbits revealed no muscle or tendon abnormality but an incidental pituitary lesion was identified. Further intracranial imaging with contrast delineated a cystic pituitary mass compressing the chiasm which showed ring enhancement of the tumour, measuring 1.72 cm × 1.28 cm × 1.29 cm .The following week the patient developed anisocoria, slight right sided ptosis and her visual acuities fell to 6/12 right eye and 6/9 in the left eye. Although initially a macroadenoma or craniopharyngioma was suspected, further histological analysis revealed a benign squamous epithelium lined lesion which was consistent with an epidermoid cyst, dermoid cyst or a teratoma. There were no signs of infarction or haemorrhage. The lesion was removed by transphenoidal resection, leading to a gradual resolution of symptoms.There have been reports of recurrent or intermittent third nerve palsy as well as ptosis as a presenting feature of pituitary tumours -4. HowevIn our case there was an initial sudden onset of vertical diplopia which may have been the result of compromise to the vascular supply of the third nerve. Pupil involvement eventually followed. On radiological analysis the tumour appeared to abut the optic chiasm however visual fields remained full. Although this presentation is unusual, a pituitary mass should be included in the differential diagnosis in cases of isolated painful diplopia.The author(s) declare that they have no competing interests.SB – The lead author involved in carrying out the literature search, study design and writing the case report.KM assisted with writing the paper, supervising and designing the study.AK supervised the management of the case and participated in its design and writing the case report.All authors have been involved in approving the final manuscript.The pre-publication history for this paper can be accessed here:


Gardner's syndrome can occasionally be complicated with intra-abdominal desmoid tumor. These tumors usually remain asymptomatic but can exhibit symptoms due to intestinal, vascular and ureteral compression and obstruction.A rare case of a 41-year-old male patient with Gardner's syndrome complicated with intra-abdominal desmoid tumor, which first presented as acute abdomen, is presented.Extra-abdominal manifestations of Gardner's syndrome along with a palpable abdominal mass would raise suspicion for the presence of a desmoid tumor in the majority of cases. In life-threatening cases, surgical treatment should be considered as a palliative approach, though the extent of excision remains debatable GardnerHereditary desmoid disease has also been found to be caused, at least in some cases, by mutation in the APC gene , whereasDesmoid tumors are benign and are characterized by fibroblastic proliferation of fascial and musculoaponeurotic origin. They usually remain asymptomatic for a long time before symptoms of intestinal, vascular and ureteral compression and obstruction evolve. They may cause obstructive jaundice or bowel obstruction and fistulization. A rare manifestation involves neural paralysis  due to compression ,8. The rA 41-year-old male was admitted to the Emergency Department complaining for acute sharp abdominal pain followed by abdominal distention, nausea, vomiting and bloody stools. The patient was a heavy smoker (70 cig/24 h) and reported mild abdominal distention during the last month and an episode of anal bleeding 4 years ago, which had been attributed to hemorrhoids. His mother died after surgical operation for multiple polyposis coli.The physical examination revealed abdominal distention, hypoactive bowel sounds, and a large palpable intra-abdominal mass with peritoneal signs. A palpable osteomatosis of the right mandible, multiple sebaceous cysts and subcutaneous fibromas of the trunk and head were observed. The absence of intestinal sounds, the distention and the abdominal contraction suggested acute abdomen. The blood analysis suggested leukocytosis (WBC 18110/μL out of which 94% were neutrophils) and anemia (Hct 29.3% and Hb 7.7 g/dl).Abdominal ultrasound revealed a large mass with necrotic lesions. Triplex study of the mass indicated decreased internal circulation with measurable velocity. An abdominal CT scan that performed after oral gastrographin administration and IV contrast medium infusion, revealed a large intra-abdominal neoplastic mass consisting of tissues of variable density. The mass was extended from the pancreas to the pelvic entrance figure . The tumThe patient was surgically treated. After entering the abdominal cavity a large solid neoplasmatic mass which invaded parts of the small and large intestine was recognized. The abdominal tumor was resected, along with the involved parts of the small intestine and the right colon Figure . The excPostoperative ophthalmologic examination of the fundus, revealed the characteristic black spots of congenital hypertrophy of the retinal pigment epithelium typical of Gardner syndrome. Full length colonoscopy was scheduled after patient's discharge. Unfortunately the patient died three months later from acute myocardial infarction. We failed to identify clinically any only obvious risk factors for the subsequent AMI except smoking.The histological examination of the specimen revealed desmoid tumor. Further cytogenetic studies along with APC mutation analysis confirmed the diagnosis. Mutation was identified in exon 9 and codon 1444.th postoperative day.Extra-abdominal manifestations of Gardner's syndrome along with a palpable abdominal mass would raise suspicion for the presence of a desmoid tumor in the majority of cases. Computed tomography would help to establish the diagnosis -12. The Gardner's syndrome consists of extensive adenomatous polyps of the colon, sebaceous cysts, osteomatosis of the skull and the mandible, and cutaneous and subcutaneous fibromas. In some cases this entity is complicated with other malignancies to include periampullary neoplasms and intra-abdominal desmoid tumor. Some authors described a rare co-existence with small bowel adenomas, carcinomas of the thyroid and adrenal gland and hepatoblastomas. The incidence of adrenal adenomas in patients with Gardner syndrome is yet unidentified, as relevant literature is inadequate ,13.Intra-abdominal desmoid tumors remain asymptomatic for long periods, and the initial symptoms are usually due to intestinal, vascular or ureteric compression and obstruction, while in rare cases neural paralysis or fistula may be the first sign . Once deThe unique feature of our case is that the prevailing symptom of the tumor was the acute abdomen. Although the diagnosis of Gardner's syndrome was evident due to the presence of various clinical signs suggestive of the disease, the etiology of acute abdomen in our patient remained unclear. Intestinal perforation might be an explanation, but the surgical procedure provided no proof for this speculation. As no similar reports in the literature were identified, this case report is of special interest.CT scan contributes not only in the diagnosis of the lesion but also in the definition of the extension in the adjacent visceral structures. A possible disadvantage of the CT scan is the inability to distinguish a desmoid tumor from a soft tissue sarcoma. The differential diagnosis of the tumor characteristics can be supported by MRI, although this study cannot assess the margin of the lesion. Recent data suggest that preoperative performance of both CT and MRI may be helpful for accurate diagnosis as well as assessment of the disease extent ,10.Though they are not cancer and do not metastasize, desmoids can cause significant morbidity and occasionally death through local/regional invasion of critical structures . NeverthIn general, surgery is not indicated in patients with intraabdominal desmoid tumors and should be reserved for life threatening complications since surgery  is associated with high recurrence rates (over 65%) and may involve major small bowel resection with its consequences. In these patients medical treatment , chemotherapy  and radiotherapy or combination of them can result tumor remission .In life-threatening cases, surgical treatment should be considered as a palliative approach, though the extent of excision remains debatable . Some auThe kind and extent of resection are determined by the location of the tumor and the adjacent structures that are involved. In our case we tried to achieve an adequate resection margin, thus we excised the part of the small and large intestine that was involved in the lesion. As a suspicious lesion in the right adrenal was identified in the CT scan, colectomy, lumpectomy and enterectomy was combined with right adrenalectomy; this decision was supported by the fact that abdominal desmoid tumors might co-exist with adrenal carcinoma. This combined procedure leads to excision of the lesion in adequate macroscopic and microscopic free of disease margins.The role of adjuvant radiotherapy and chemotherapy in the treatment of desmoid tumors still remains unclear. Some authors believe that radiotherapy contributes in the local control of the disease . Recent Gardner's syndrome should be suspected in patients with osteomatosis, multiple sebaceous cysts and cutaneous and subcutaneous fibromas. Although the appropriate treatment of intra-abdominal tumors remains unclear we believe that wide resection in adequate margin offer the best alternative for local control of the tumor.The author(s) declare that they have no competing interests.HA: Had made substantial contributions to conception and the patient's treatment. FD: Collected the data and wrote the manuscript. PV: Corrected the manuscript. FG: Collected the data and contributed to the manuscript writing. RS: Corrected and approved the manuscript and participate to the patient's treatment. SP: Contributed to the conception, corrected and approved the manuscript.All authors read and approved the final manuscript.


Abdominal cocoon is a rare cause of intestinal obstruction. The abdominal cocoon is probably a developmental abnormality, largely asymptomatic, and is found incidentally at laparotomy or autopsy. Pre-operative diagnosis cannot be often made correctly. This rare entity of intestinal obstruction has been described in the whole literature as a thick fibrotic sac covering the small bowel partially or completely. The etiology of abdominal cocoon is unknown and most often it is found in adolescent girls from tropical or subtropical countries. Complete recovery is generally expected after the removal of the membrane surgically. This paper reports a male patient who has had intestinal obstruction symptoms and has per-operatively been diagnosed as abdominal cocoon. The abdominal cocoon was first described and named in 1978 by Foo et al -5. A MedA 30-year-old male patient was admitted to the Emergency Department of our hospital with a 24-hour history of abdominal pain which had developed suddenly and initially been colicky in nature, with intermittent vomiting and abdominal distension. There was no history of peritonitis, abdominal surgery or tuberculosis. There had been two similar episodes in one year. But, because of the spontaneous regression of the symptoms, he was not examined by any physician in those episodes. Because of the asthma and hypertension, he had used calcium canal blockers and teophiline for two years.General physical examination revealed an anxious looking and mild dehydration. His pulse was 110/minute, temperature 37.8°C, blood pressure 130/70 mmHg. There was no cyanosis or jaundice. No abnormalities of the chest or cardiovascular system were found. Local examination of the abdomen revealed a distended abdomen, hypoactive bowel sounds, mild tenderness and rigidity in the whole abdomen. A tender lump was palpated in the umbilical region which moved from side to side with mild guarding. There was no hepatomegaly or splenomegaly and rectal examination was unremarkable.Routine laboratory workup revealed a total leukocyte count of 13300 cells/ml, hemoglobin of 17.9 g %, and normal serum chemistry and normal urine analysis. PA X-ray of the chest was normal but plain standing X-ray of the abdomen showed multiple air-fluid levels with no free gas under the diaphragm. An ultrasound examination of the abdomen showed normal liver, gallbladder, pancreas and kidneys. But there was a small amount of fluid in the pelvis and a heterogen, vascularized mass lesion was seen in the periumblical region which had 96 × 75 mm dimensions.Under a provisional clinical diagnosis of mechanical small bowel obstruction, emergency laparotomy was performed through a midline incision. After opening the peritoneum, especially the proximal section of the small bowel was observed to be dilated, its mesentery was edematous and the whole small bowel was covered by a dense whitish and approximately 2 mm thick membrane which gave the appearance of a cocoon . This membrane was freely mobile freed from the parietal peritoneum. There was a straw-colored fluid around 200 ml in the pelvis. The membrane enveloping the small bowel was incised carefully and separated from the intestinal serosa by sharp and blunt dissections and the whole small bowel was freed and followed from treitz to ileocaecal junction. Partial omentectomy and appendectomy were performed. The whole freed small bowel was viable and the colon was normal; thus no further surgical procedure was deemed necessary. The peeled-off membrane was studied histopathologically and microscopic examination revealed a chronic non-specific inflammatory reaction and mature fibrous tissue.The patient had an uneventful post-operative period and was discharged from the hospital on the seventh post-operative day. He has been regularly attending our follow-up clinics for the last one year and has remained free of bowel symptoms throughout this period.The etiologies of intestinal obstruction differ very little from one part of the globe to another. Adhesion formation causing intestinal obstruction is a very important and frequently observed pathology. The case reported by us in this paper is peculiar and is presented with unusual types of adhesions.et al. described a curious condition by 10 cases of adolescent females with acute or subacute small bowel obstruction symptoms named as "abdominal cocoon" [In 1978 Foo  cocoon" . The con cocoon" . Most caThe characteristic finding is that of the encasement of the whole or part of the small bowel by a thick shiny membrane, aptly simulating a cocoon. The loops of the small bowel have been stuck together by filmy soft adhesions separated easily by blunt or sharp dissections from the cocoon .Surgery remains the cornerstone in the management of abdominal cocoon. Careful dissection and excision of the thick sac with the release of the small intestine leads to complete recovery. Resection of the bowel is indicated only if it is nonviable . An inciTo explain the etiology and the formation of the membrane of this condition a number of hypotheses have been proposed. These include retrograde menstruation with a superimposed viral infection , retrogrIn literature some authors define these cases by different diagnoses such as 'encapsulating sclerosing peritonitis', 'abdominal cocoon', 'peritoneal encapsulation' or they use all these definitions for one patient. The abdominal cocoon should not be confused with "encapsulating sclerosing peritonitis", which occurs in association with the use of the beta blocker practolol, cirrhosis of the liver, chronic peritoneal dialysis, carcinoid syndrome, familial Mediterranean fever and asbestos exposure ,11,12. Ath week of gestation ['Peritoneal encapsulation' is a related condition of abdominal cocoon but it differs in that small or large bowel is found behind an accessory, but otherwise normal, peritoneal membrane . This coestation ,15.The prognosis of abdominal cocoon after surgery seems excellent and no recurrence has been described. Only one patient in literature who had presented with long-standing symptoms and weight loss died post-operatively after subclavian vein thrombosis due to intravenous hyperalimentation .The table includes clinical summaries of the cases which were diagnosed as abdominal cocoon in English literature up to now. (*: No information about these data could be found by Medline research).Click here for file