A succesfully managed inflammatory pseudotumour of liver without surgery: report of a case

The inflammatory pseudotumor (IPT) of the liver is an extremely rare focal lesion of the parenchyma with unknown aetiology. It has the appearance of a malignant tumor but has a benign histology and clinical course. Herein, we report a case of IPT in a 55 year-old woman. She had complained of right upper quadrant pain and subfebrile fever. Imaging studies revealed a heterogeneous, solitary mass in right lobe of liver which was 90 x 81 mm in size. Histopathological examination of ultrasonography-guided true-cut biopsy demonstrated IPT. After the initiation of the antibiotic and nonsteroidal anti-inflammatory drugs (NSAID), the size of hepatic pseudotumor decreased (20 x 20 mm) and then completely disappeared. To rule out a malignancy and to reach the diagnosis of IPT, biopsy from the mass is mandatory. After the diagnosis, IPT gave an excellent response to short course of antibiotic and NSAID therapy. To prevent unnecessary resective liver surgery, IPT should be kept in mind in the differential diagnosis of liver mass which can be successfully managed medically.     

Inflammatory pseudotumor of the liver and spleen diagnosed by percutaneous needle biopsy

An inflammatory pseudotumor (IPT) is a relatively rare lesion characterized by chronic infiltration of inflammatory cells and areas of fibrosis. IPTs are difficult to diagnose because of the absence of specific symptoms or of characteristic hematological or radiological findings. In this study, a case of a woman aged over 70 years was reported, who presented with a general malaise lasting more than two months. A computed tomography scan demonstrated a diffusely spread lesion of the liver with a portal vein occlusion and a splenic lesion surrounded by a soft density layer. Since the percutaneous liver biopsy showed findings that suggested an IPT, although the radiological findings did not exclude the possibility of a malignancy, we performed a percutaneous spleen biopsy to enable a more definitive diagnosis. The microscopic findings from the spleen specimen lead us to a diagnosis of IPT involving the liver and spleen. Subsequent steroid pulse therapy was effective, and rapid resolution of the disease was observed.     

Concomitant inflammatory pseudotumor of the liver and spleen.

We report the case of a 53-year-old man with inflammatory pseudotumor (IPT) of the liver and spleen. This concomitant association has rarely been reported. The patient presented with a hypoechoic mass in the liver and a clinical picture of recurrent sepsis; hematochemical exams and imaging data were nonspecific. Antibiotic therapy improved the clinical course, but did not resolve it definitively. After 50 days of therapy, as the hepatic mass decreased a similar lesion appeared in the spleen. The final diagnosis was made on splenectomy and an intra-operative biopsy of the residual liver lesion. The diagnostic problems encountered in this very rare association of IPT of the liver and spleen were similar to those for isolated IPT in the respective single organ sites. After 15 months of follow-up, the patient is in good health and no recurrence of symptoms or masses has been observed.     

Concomitant inflammatory pseudotumor of the liver and spleen

Abstract: We report the case of a 53‐year‐old man with inflammatory pseudotumor (IPT) of the liver and spleen. This concomitant association has rarely been reported. The patient presented with a hypoechoic mass in the liver and a clinical picture of recurrent sepsis; hematochemical exams and imaging data were nonspecific. Antibiotic therapy improved the clinical course, but did not resolve it definitively. After 50 days of therapy, as the hepatic mass decreased a similar lesion appeared in the spleen. The final diagnosis was made on splenectomy and an intra‐operative biopsy of the residual liver lesion. The diagnostic problems encountered in this very rare association of IPT of the liver and spleen were similar to those for isolated IPT in the respective single organ sites. After 15 months of follow‐up, the patient is in good health and no recurrence of symptoms or masses has been observed.     

A case of hemangioma accompanied by inflammatory pseudotumor of the spleen

Both hemangioma and inflammatory pseudotumor (IPT) of the spleen are rare benign mass lesions. Moreover, a splenic hemangioma accompanied by IPT is extremely rare. A 61-year-old woman who suffered from liver cirrhosis had a splenic cavernous hemangioma surrounded by granuloma. The literature on IPT of the spleen has described several possibilities of its causes; however, it is still unknown. This case was accompanied by portal hypertension due to liver cirrhosis, which may cause microrupture of hemangioma resulting in an IPT.     

Inflammatory pseudotumor of the liver complicated with recurrent gouty arthritis.

Inflammatory pseudotumor (IPT) of the liver is a rare benign lesion of unknown etiology and is often accompanied by fever. Unexplained persistent fever unresponsive to antibiotics developed in a 70-year-old man suffering from intractable recurrent gouty arthritis. 67Ga-scintigraphy disclosed intense focal uptake in the upper abdomen. The lesion in the left lobe of the liver was an ill-defined hypodensity mass on computed tomographic scan and was enhanced on dynamic magnetic resonance imaging. The tumor was surgically removed and a diagnosis of IPT was made. Fever and arthritis resolved completely after surgery. Possible interaction between IPT of the liver and gouty arthritis was suggested.     

Inflammatory pseudotumor following hematopoietic stem cell transplantation: a new case and review of the literature

Inflammatory pseudotumor (IPT) is a rare tumor that occurs in various organs and tissues. The clinical picture varies from the more frequent benign lesions to the rare malignant tumors with distant metastases. IPT associated with hematopoietic stem cell transplantation (HSCT) is rarely reported. In this article, we review the reports of IPT after HSCT and describe the first case of bladder IPT. We also review the possible factors involved in the pathogenesis. IPT might be rare but it is a potentially serious complication of HSCT. It should be considered in patients with otherwise unexplained inflammatory symptoms or signs or with any mass lesion in the post-HSCT period. A knowledge of this entity and insistence on a definitive biopsy of mass lesions in the post-HSCT period can avoid unnecessary treatment such as radical surgery, chemotherapy or radiotherapy.     

Inflammatory pseudotumor of the liver with primary sclerosing cholangitis

Inflammatory pseudotumor (IPT) of the liver is a rare benign variant of hepatic masses, and its exact etiology has not been elucidated. We report a case of IPT associated with primary sclerosing cholangitis (PSC). The patient was a 50-year-old man admitted to our hospital because of jaundice. Abdominal ultrasonography (US) and computed tomography showed multiple dilations of the intrahepatic bile ducts and multiple masses in the liver. On magnetic resonance imaging, the masses were slightly hypointense on T1-weighted images and slightly hyperintense on T2-weighted images. On T1-weighted images after the bolus infusion of Gd chelate, the masses had no contrast enhancement, and they were hypointense in the arterial phase and portal venous phase. However, they were slightly enhanced and became almost isointense relative to the surrounding normal liver parenchyma in the delayed phase. Endoscopic retrograde cholangiography demonstrated multiple irregular strictures and dilations of the intrahepatic bile ducts. Angiography demonstrated no abnormal findings, but, interestingly, subsequent dynamic CO₂-enhanced US showed a strongly hyperechoic string, indicating that an artery had penetrated through the hypoechoic mass. A US-guided percutaneous needle biopsy revealed that the lesions were morphologically comparable to IPT. After cholangiography and microscopic analysis of the tumor, the final diagnosis was determined to be IPT of the liver with PSC. A number of previous reports have suggested a possible relationship between IPT and PSC, based on pathological findings. This report confirmed, based on clinical findings, that PSC is one of the causes of hepatic IPT. Received: May 12, 1999 / Accepted: October 22, 1999     

Inflammatory pseudotumor of the liver associated with Crohn's disease

Inflammatory pseudotumor (IPT) of the liver is a rare, benign lesion that is characterized by proliferating fibrous tissue infiltrated by inflammatory cells. The exact etiology of IPT remains unclear. Although the association of IPT with systemic inflammatory disorders has been established, a specific link with Crohn's disease is rare. We report two cases of IPT associated with Crohn's disease. Both patients were elderly males who presented with abdominal pain and jaundice. At the time of presentation, both had active Crohn's disease that was undiagnosed. Computed tomography demonstrated a liver mass, and endoscopic retrograde cholangiography revealed biliary strictures in both patients. Given their symptomatic jaundice and concern for cholangiocarcinoma, they underwent abdominal surgical exploration and pathology results revealed IPT. They were subsequently diagnosed with Crohn's disease. Following treatment of Crohn's disease, there was significant improvement in their overall clinical status. An underlying diagnosis of inflammatory bowel disease should be considered in patients with IPT.     

Chest wall metastasis from unknown primary site of hepatocellular carcinoma

Previous reports of a solitary metastatic hepatocellular carcinoma have been rare. Because this tumor has a different treatment modality and prognosis, an accurate differential diagnosis is essential. Here we report a rare case of a solitary chest wall metastasis from unknown primary site of hepatocellular carcinoma. It involves a 51-year-old man who was admitted to our hospital because of a palpable left upper chest wall mass. The mass was resected and pathologic examination confirmed a diagnosis of metastatic hepatocellular carcinoma. Despite our investigation, no evidence was found that indicated the primary origin of the hepatocellular carcinoma. Four months later, the patient was admitted again because of spinal cord compression at the third and fourth thoracic vertebrae. Emergent decompressive laminectomy was performed and microscopic features revealed the same pathology as the initial chest wall mass resected 4 months earlier. After one year, a follow-up abdominal computed tomography (CT) still revealed no evidence of primary hepatocellular carcinoma.     

Splenic inflammatory pseudotumor mimicking angiosarcoma

Splenic tumors are rare. Differentiation of the tumors before operation is of great value regarding the outcome. A case of a 32-year-old man with a splenic inflammatory pseudotumor （IPT） mimicking splenic angiosarcoma is described. The tumor was highly suspected of being splenic angiosarcoma based on radiological findings preoperatively. However, after splenectomy, histopathological examinations revealed splenic IPT. Splenic IPT and angiosarcoma are rare and often pose diagnostic difficulties because the clinical and radiological findings are obscure. Due to large differences in prognosis, we briefly reviewed the clinical, radiological, and pathological features of both of the tumors.     

Spontaneous regression of an inflammatory pseudotumor of the liver

Inflammatory pseudotumors (IPT) have been described for virtually every site in the body but is rare in the liver. The clinical presentation of hepatic IPT is unspecific, patients complain of fever, malaise, weight loss and often of symptoms related to a mass effect. Routine imaging procedures are not sufficient to make the diagnosis and a biopsy is necessary to differentiate IPT from neoplasms. The course of the disease is unpredictable and in most of the reported cases the patients underwent resection or were medically treated. We report a patient with a histologically proven IPT of the liver that regressed spontaneously over several months without any specific treatment.     

A case of lymphomatoid gastropathy: a self-limited pseudomalignant natural killer (NK)-cell proliferative disease mimicking NK/T-cell lymphomas

Natural killer (NK)/T-cell lymphomas exhibit aggressive tumor behavior and have a poor prognosis. Recently, self-limited pseudomalignant NK-cell proliferative disorders of the stomach mimicking NK/T-cell lymphomas have been recognized. We report a rare case of lymphomatoid gastropathy in a 71-year-old female. The patient underwent esophagogastroduodenoscopy (EGD) because of slight epigastric discomfort which revealed a 10-mm, reddish, flat elevation with erosion on the posterior wall of the lower gastric body. Histological examination of biopsy specimens showed atypical NK/T cell infiltration with cytoplasmic CD3+, CD4?, CD5?, CD7+, CD8?, CD16?, CD20?, CD56+, CD68?, CD117?, MPO?, TIA1+, and granzyme B+. Epstein?Barr virus-encoded RNA in situ hybridization was negative. Three months later, repeated endoscopic examination surprisingly revealed spontaneous regression of the lesion without any treatment. It is important that endoscopists consider this rare entity in the differential diagnosis, and excessive treatment should be avoided.     

First Report of TTSuV1 in Domestic Swiss Pigs

Serum prevalence of Torque teno sus viruses (TTSuV1 and k2; family Anelloviridae) is known to be high in the porcine population worldwide but pathogenesis and associated pathomorphological lesions remain to be elucidated. In this study, quantitative real-time PCR for detection of TTSuV1 was performed in 101 porcine samples of brain tissue, with animals showing inflammatory lesions or no histological changes. Additionally, a pathomorphological and immunohistochemical characterization of possible lesions was carried out. Selected cases were screened by TTSuV1 in situ hybridization. Furthermore, TTSuV1 quantitative real-time PCR in splenic and pulmonary tissue and in situ hybridization (ISH) in spleen, lungs, mesenteric lymph node, heart, kidney, and liver were performed in 22 animals. TTSuV1 was detected by PCR not only in spleen and lung but also in brain tissue (71.3%); however, in general, spleen and lung tissue displayed lower Ct values than the brain. Positive TTSuV1 results were frequently associated with the morphological diagnosis of non-suppurative encephalitis. Single TTSuV1-positive lymphocytes were detected by ISH in the brain but also in lungs, spleen, mesenteric lymph node and in two cases of non-suppurative myocarditis. A pathogenetic role of a TTSuV1 infection as a co-factor for non-suppurative encephalitides cannot be ruled out.     

A case of Ewing's sarcoma of the right femur associated with pulmonary metastases detected by fluorodeoxyglucose positron emission tomography

A 32-year-old man was referred to our hospital because of a nodular shadow on his chest x-ray film. Chest computed tomography scan revealed multiple nodules in both lungs. Video-assisted thoracoscopic surgery was performed to diagnose the tumor. Microscopic examination of the resected specimen showed tumor connecting of small round cells. Fluorodeoxyglucose positron emission tomography demonstrated strongly increased uptake in the right femur. Subsequently, magnetic resonance imaging showed a well defined mass, approximately 5cm in diameter, in the right lateral femur with isointensity on T1-weighted images and high intensity on T2-weighted images. A needle biopsy of the tumor of the right femur was performed. Pathological examination revealed the same findings of the pulmonary lesions. Immunohistochemical staining was positive for CD99 in the tumor cells. Ewing's sarcoma gene rearrangement was found using fluorescence in situ hybridization on frozen-section specimens. Based on these findings, a diagnosis of Ewing's sarcoma was made. The patient died 13 months after the initial detection of the disease despite intensive chemotherapy. In cases of multiple pulmonary nodular lesions in a young patient, metastatic tumors arising from bone soft tissue should be included in the differential diagnosis.     

Inflammatory Myofibroblastic Tumor of the Liver: A Diagnostic Challenge

Inflammatory myofibroblastic tumor (IMT) is an uncommon myofibroblastic neoplasm that was formerly included within the broad category of inflammatory pseudotumor (IPT). IMT is rarely encountered in the liver. Similar to IMT of other organs, the interchangeable use of the terms IMT and IPT in liver has made the analysis of these lesions difficult. In this review, clinical and pathological features of IMT of the liver are reviewed and the differential diagnosis of IMT is discussed, with emphasis on IPT and the other entities included in this large category. IMT can mimic malignant tumors. There are no known unique diagnostic clinical, laboratory, or radiological features. The definitive diagnosis of IMT depends on careful pathological examination. The histopathological evaluation of hepatic IMT reveals that, the myxoid/vascular pattern is the most frequently observed, followed by, in decreasing frequency, fibrous histiocytoma-like pattern and hypocellular fibrous pattern. In IMT of the liver, anaplastic lymphoma kinase (ALK) expression reliably predicts the presence of an ALK gene rearrangement. The diagnosis of hepatic IMT depends on the dominant histopathological pattern, and the management of the disease is still controversial. IMT of the liver is a distinctive neoplasm of intermediate biological potential, and should be distinguished from the variety of lesions that are included under the broad category of IPT. Therefore, to avoid confusion regarding the true incidence and behavior of hepatic IMT, the term IPT should not be used interchangeably with IMT. The rarity of IMT in liver should not minimize its consideration in the differential diagnosis of liver tumors, especially in patients with tumor markers in normal range.     

Inflammatory pseudotumor of the liver: report of eight cases, including three unusual cases, and a literature review

BACKGROUND: Inflammatory pseudotumor (IPT) of the liver is a rare but increasingly recognized tumor-like lesion. This condition is becoming an important differential diagnosis in patients with hepatic space-occupying lesions. This study reports the clinical features of eight cases of IPT of the liver, including the first report of three cases with an unusual associated disease or clinical course. METHODS: The study sample included cases of IPT diagnosed based on the histopathology in our institution from 1995 to 2004. Clinical events were reported and compared with reports in the literature. RESULTS: The age of the patients ranged from 28 to 78 years. The most common symptoms were abdominal pain, fever and bodyweight loss. Three patients were diagnosed incidentally. The initial clinical diagnoses were hepatocellular carcinoma in three cases, liver abscess in two cases, metastasis in one case and uncertain nature in two cases. Two cases were associated with preceding endophthalmitis and cholangiocarcinoma, respectively. Two patients had spontaneous lesion regression, but one of these had possible recurrence 10 months later. The other two patients experienced resolution of their lesions following antibiotic treatment. Four patients underwent surgical resection with subsequent recovery. CONCLUSIONS: The clinical and imaging features of IPT in this small series were found to sometimes mimic those of malignant liver tumors or abscess. Surgical resection was performed in half of the cases. However, IPT of the liver could resolve spontaneously or following antibiotics treatment.     

Rectal inflammatory myofibroblastic tumor: Case report and literature review

Rationale:Rectal inflammatory myofibroblastic tumor (IMT) is an extremely rare mesenchymal tumor characterized by a mixture of spindle-shaped myofibroblasts or fibroblasts and inflammatory infiltration of lymphocytes and plasma cells. To date, only 8 cases of rectal IMT have been reported. Herein, we report an additional case of rectal IMT in a 28-year-old woman.Patient concerns:A 28-year-old woman presented with abdominal pain and hematochezia.Diagnoses:Colonoscopy showed a 3.0-cm subepithelial tumor with central ulceration, covered by white exudate in the rectum. Rectal magnetic resonance imaging revealed a 4.0 × 3.0-cm-sized well-defined subepithelial tumor in the right wall of the rectum, with suspicious right perirectal fat infiltration.Interventions:Laparoscopic anterior resection was performed. Microscopic examination of the surgical specimen revealed bland-looking spindle cells intermingled with lymphoplasma cells. Immunohistochemistry and fluorescence in situ hybridization showed anaplastic lymphoma kinase positivity and anaplastic lymphoma kinase positivity rearrangement. Rectal IMT was confirmed based on histological, immunohistochemical, and fluorescence in situ hybridization findings. The patient was doing well without evidence of tumor recurrence 1 year after the surgery.Lessons:Rectal IMT, despite its rarity, should be considered in the differential diagnosis of rectal cancer. Second, an accurate histopathologic diagnosis and complete surgical resection can be the most important approaches to offer a chance for the cure of rectal IMT.     

Clinical Features,Image Findings,and Prognosis of Inflammatory Pseudotumor of the Liver: A Multicenter Experience of 45 Cases

Background/Aims

Inflammatory pseudotumor (IPT) of the liver is a rare disease characterized by chronic infiltration of inflammatory cells. However, the clinical characteristics and outcomes of IPT remain uncertain.

Methods

Clinical features, image findings, and outcomes of 55 patients with histologically proven IPT were evaluated.

Results

They consisted of 26 men and 19 women with median age of 65 years. Serum carcinoembryonal antigen and carbohydrate antigen 19-9 levels were normal in 42 patients (93.3%). Enhanced CT scans indicated poorly defined peripheral enhancement (82.5%) at the arterial phase and poorly defined hyperattenuating lesions with internal hypoattenuating areas at the equilibrium phase (77.0%). Gadolinium-enhancement MRI revealed poorly defined peripheral rim-like enhancement (77.8%). Ten patients underwent surgical resection and 35 were treated conservatively with or without antibiotics. No recurrence was noted after surgical resection during follow-up (1 to 48 months). In all patients who received conservative treatment, complete resolution or size reduction was noted during follow-up (1 to 192 months).

Conclusions

CT and MRI provide clues to the diagnosis of IPT in patients with liver masses and normal tumor markers. However, due to the lack of pathognomonic findings, the clinician''s suspicion and histological diagnosis are necessary to make an accurate diagnosis of IPT.     

Current status of primary malignant melanoma of the esophagus: clinical features, pathology, management and prognosis

Primary malignant melanoma of the esophagus (PMME) is a rare disease with an extremely poor prognosis. Up to 2011, approximately 300 cases had been reported worldwide. The average age of onset is 60.5 years old, with a prevalence of males (2:1). A typical finding of PMME is a lobular or polyploid, well-circumscribed and pigmented tumor, partly covered with normal mucosa. PMME represents various colors depending on its melanin quantity and commonly coexists with intramural metastases, melanocytosis or melanoma in situ. The tumor is located from the middle to lower thoracic esophagus. The accuracy of diagnosis from biopsy is approximately 80%, because many cases are misdiagnosed as a poorly differentiated carcinoma because of the absence of melanin granules. A definite diagnosis was made by immunohistochemical examination with positive results of S100 protein, HMB45 and neuron-specific enolase. PMME has a highly metastatic potential, and the incidence of distant metastasis at the initial diagnosis is around 40–80%. A metastatic tumor from cutaneous malignant melanoma is another pigmented esophageal tumor to be considered when making the differential diagnosis for PMME. Junctional activity with melanotic cells in the adjacent epithelium and the presence of in situ melanoma and/or a satellite tumor without a previous history of cutaneous melanoma are definitive. Most of the reported patients were treated with radical esophagectomy, which is believed to be an effective approach for localized PMME. Five-year survival rates have been achieved in 37% recently, while adjuvant therapy has not been proven to increase overall survival but plays a palliative role.