IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis?

Sclerosing cholangitis (SC) is a heterogeneous disease entity. Different etiologies such as choledocholithiasis, biliary tumor, or pericholangitis can manifest as SC. Hepatic inflammatory pseudotumor (IP) is rarely associated with SC (sclerosing cholangitis associated with hepatic inflammatory pseudotumor; SC-hepatic IP), but sclerosing pancreatitis (SP) is not infrequently associated with bile duct lesions (sclerosing pancreatitis-associated sclerosing cholangitis; SP-SC). In this study, we compared the histologic changes of hepatic hilar and extrahepatic bile duct lesions of SC (7 cases), SC-hepatic IP (5 cases), SP-SC (5 cases), and typical primary sclerosing cholangitis (PSC) (5 cases). Histologically, all SP-SC cases showed extensive and dense fibrosis with marked lymphoplasmacytic infiltration, many eosinophils, and obliterative phlebitis. Four cases of SC showed bile duct lesions similar to those of SP-SC, whereas other three cases of SC showed milder lymphoplasmacytic infiltration, scant eosinophilic cell infiltration, and no obliterative phlebitis. All SC-hepatic IP cases showed bile duct lesions identical to those of SP-SC. Immunohistochemically, many IgG4-positive plasma cells were found in the bile duct lesions of all SP-SC cases, 4 SC cases with marked lymphoplasmacytic infiltration, and all SC-hepatic IP cases. By contrast, IgG4-positive plasma cells were scarce or hardly found in the remaining 3 SC cases and all PSC cases. In conclusion, 4 SC cases and all SC-hepatic IP cases showed bile duct lesions identical to those of SP-SC, suggesting that these three conditions may be a single disease entity. Their pathogenesis may be similar or closely related to that of SP, and in that respect they may represent an IgG4-related biliary disease. They may respond to steroid therapy as SP does.     

Cerebral tumor or pseudotumor?

Pseudotumoral lesions are uncommon but important to identity lesions. They can occur during inflammatory diseases (systemic diseases, vasculitis, demyelinating diseases), infectious, and vascular diseases. Also, in a patient with a treated tumor, pseudo-progression and radionecrosis must be differentiated from the tumoral development. Diagnosis can be difficult on an MRI scan, but some MRI aspects in conventional sequences, diffusion, perfusion and spectroscopy can suggest the pseudotumoral origin of a lesion. Imaging must be interpreted according to the context, the clinic and the biology. The presence of associated intracranial lesions can orientate towards a systemic or infectious disease. A T2 hyposignal lesion suggests granulomatosis or histiocytosis, especially if a meningeal or hypothalamic–pituitary involvement is associated. Non-tumoral lesions are generally not hyperperfused. In the absence of a definitive diagnosis, the evolution of these lesions, whether under treatment or spontaneous, is fundamental.     

Pleural solitary fibrous tumor with bullae: is it a microinvasive tumor?

We report a rare pleural solitary fibrous tumor with bullae. Chest computed tomography showed a nodular lesion with bullae adjacent to the left diaphragm. Thoracoscopic resection followed by a pathology study showed that the tumor was a solitary fibrous tumor beside the visceral pleura. We suggest that the bullae containing dilated bronchioles were caused by a checkvalve mechanism next to the microinvasive component in the solitary fibrous tumor.     

Right atrial solitary fibrous tumor - a new cardiac neoplasm?

Heart tumors are very rare neoplasms. Solitary fibrous tumors are mesenchymal neoplasms first described as pleura-based lesions. We are the first to describe the clinical symptoms, surgical technique for removal, histology, and exact location as well as medium-term follow-up of a solitary fibrous tumor in the heart.     

Is tartrate-resistant acid phosphatase 5b a potent bio-marker for late stage aseptic implant loosening?

Purpose

Aseptic loosening is a serious complication after total joint arthroplasty. Plain radiography, along with clinical signs of prosthesis loosening, is the technique of first choice to evaluate loosening of joint replacements. Nevertheless, radiographical signs of osteolysis may not be apparent until progressed stages of loosening. Thus the search for alternative diagnostic methods is of great importance. The purpose of the present study was to evaluate the potential diagnostic significance of TRAP 5b, Osteocalcin, CrossLaps and Bone ALP for aseptic loosening of total joint replacements.

Methods

Thirty-seven patients (25 women, 12 men, mean age 65 years, age range 54–76 years) treated with revision surgery due to clinically and radiologically confirmed late aseptic prosthesis loosening were examined prospectively. Serum levels of TRAP 5b, Osteocalcin, CrossLaps and Bone ALP were compared with a matched control group (n?=?39).

Results

We found a significant decrease in TRAP 5B level in patients with aseptic loosening. Bone ALP and Osteocalcin as markers of osteoblast activity, and CrossLaps as another resorption marker did not allow any prediction of bone remodeling in this late phase of loosening.

Conclusion

In the ?late“ phase of aseptic joint replacement loosening, no increase of TRAP 5b and therefore no increase of osteoclast number and activity was measurable. Thus, in this situation an anti-osteolytic therapy with a bisphosphonate or denosumab would not gain any further benefit.     

Is inflammatory joint disease in HIV-infected patients a form of spondyloarthropathy?

In Congo-Brazzaville, the seroprevalence of HIV infection is in the range 7-8%, and AIDS is the leading cause of aseptic arthritis (60% of cases). PATIENTS, MATERIAL AND METHODS: The ESSG and Amor's criteria for spondyloarthropathy were evaluated in 83 patients with HIV infection admitted for aseptic arthritis to the rheumatology department of the Brazzaville teaching hospital, over an 8-year period. RESULTS: All 83 patients were CDC stage IV; 66 (80%) had polyarthritis and 17 (20%) oligoarthritis. A single patient met ESSG and Amor's criteria, with six points; one patient had five points and 15 had three points. The joint involvement was asymmetrical and nonerosive. Nonsteroidal antiinflammatory therapy ensured resolution of the manifestations within 4-8 weeks. The most common sites of involvement were the knees (84%), ankles (59%), and great toes (23%) at the lower limbs and the wrists (41%), elbows (29%), and metacarpophalangeal and interphalangeal joints (25%) at the upper limbs. CONCLUSION: Inflammatory arthritis in HIV patients does not meet ESSG or Amor's criteria for spondyloarthropathy.     

Inflammatory myofibroblastic tumor of the pancreas: a case report of 2 pediatric cases—steroids or surgery?

Inflammatory pseudotumors also termed inflammatory myofibroblastic tumors (IMTs) are rare, benign, solid lesions of unclear etiology more usually found in the lung and very rarely in the pancreas. We report 2 cases and outline our management for each. The first case was treated surgically, whereas the second was treated with high-dose steroids. This represents the first reported case whereby steroid treatment has been successful in pancreatic IMT.     

Idiopathic sclerosing encapsulating peritonitis — Is a preoperative diagnosis possible? Report of three cases

Sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction that is usually diagnosed during a laparotomy; however, a preoperative diagnosis is possible. Sclerosing encapsulating peritonitis can be classified as primary or idiopathic and secondary types and only about 70 cases of idiopathic type have been reported since it was first described. It is characterized by a total or partial encasement of the small bowel by a thick fibrotic membrane. This report presents a series of three cases in which a diagnosis of idiopathic SEP was made preoperatively based on the clinical features and radiological findings, which were confirmed by a laparotomy and histopathology. All of the cases were successfully managed by the excision of the membrane. This report demonstrates that based on the clinical features and radiological investigations, in the absence of other plausible etiologies for intestinal obstruction, it is possible to suspect a preoperative diagnosis of SEP, thereby preventing a “surprise“ finding during a laparotomy and allowing for better management.     

Is a benign meningioma always an indolent tumor?

Primary cardiac sarcomas are very rare. We report one case of primary cardiac osteosarcoma arising from the left atrium of a 42-year-old woman and analyze its clinical and pathological features. Histopathologic examination revealed the tumor was composed of massive osteoid and cartilaginous differentiation embedded in spindle and polygonal cells with marked cytological and nuclear pleomorphism. The patient was alive without recurrence or metastasis at 20 months after surgery without further adjuvant chemotherapy or radiotherapy.     

Grade 2 chondrosarcoma: stage I or stage II tumor?

We analyzed chondrosarcomas of bone to assess whether Grade 2 tumors are more appropriately grouped with Grade 1 chondrosarcomas or Grade 3 and dedifferentiated chondrosarcomas (Musculoskeletal Tumor Society Stage I or Stage II). A retrospective chart review identified 109 patients who presented with nonmetastatic chondrosarcoma. Data were gathered on each tumor's histologic grade, extent (intracompartmental versus extracompartmental), resection margin status, and site (axial versus appendicular). The cohort included 60 males and 49 females with a mean age of 50 years. Forty tumors were located in the axial skeleton and the remainder were distributed throughout the appendicular skeleton. Fifty-three tumors were Grade 1, 40 tumors were Grade 2, eight tumors were Grade 3, and eight tumors were dedifferentiated. Statistical analysis established grade as the only significant variable for this group of patients; extracompartmental tumor spread also correlated with outcome, but not independent of the grade. Margin status and site were not statistically significant with respect to outcome. Chi-square analysis also established that Grade 2 tumors are grouped more appropriately with the Grade 1 chondrosarcomas as Musculoskeletal Tumor Society Stage I neoplasms. These data should be considered when developing the surgical plan, particularly for patients with Grade 2 appendicular chondrosarcomas.     

Haematuria; a late complication of TURP?

This study was designed to investigate the cause of haematuria for patients that had previously undergone a TURP. One hundred patients were identified in a walk-in haematuria clinic as having been treated by TURP and were prospectively investigated for the cause of bleeding. Prostate regrowth was the diagnosis in 63% of cases. A diagnosis of malignancy was made for 23.5% of these patients. We have confirmed the hypothesis that for patients who present with haematuria after a TURP, the prostate is the usual source of bleeding.Prostate Cancer and Prostatic Diseases (2001) 4, 178-179.     

Is a hybrid strategy a lower-risk alternative to stage 1 Norwood operation?

Background

For neonates with critical left ventricular outflow tract obstruction (LVOTO), hybrid procedures are an alternative to the Norwood stage 1 procedure. Despite perceived advantages, however, outcomes are not well defined. Therefore, we compared outcomes after stage 1 hybrid and Norwood procedures.

Is obesity an inflammatory disease?

BACKGROUND: Most obese individuals have elevated concentrations of interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-alpha), markers of inflammation closely associated with diabetes, hypertension, and stroke. HYPOTHESIS: Obesity is a low-grade inflammatory disease, and Roux-en-Y gastric bypass (RYGB) reduces biochemical markers of inflammation and modifies gene expression in hypothalamic food intake/energy-related nuclei and subcutaneous abdominal fat (SAF). METHODS: Obesity was induced in 24 3-week-old Sprague Dawley pups fed a high-energy diet (HED). Three groups (n = 8/group) were studied: RYGB, sham-operated pair-fed, and sham-operated ad libitum HED. Controls were nonobese rats fed chow (n = 6). Rats were killed 10 days after operation, and blood was collected to measure corticosterone and SAF and mesenteric fat to measure IL-6, TNF-alpha, and corticosterone. Total mRNA from arcuate nucleus and SAF purified for gene expression profiling. Data were analyzed with analysis of variance, Mann-Whitney test, and t test. RESULTS: Before operation, the body weight of the obese groups was 493 +/- 7 g and control = 394 +/- 12g. The 10-day postoperative weight was RYGB = 417 +/- 21 g, pair-fed = 436 +/- 14 g, and ad libitum HED = 484 +/- 15 g. Mesenteric and SAF weight decreased in RYGB. Mesenteric/SAF ratio of IL-6, TNF-alpha, corticosterone, and gene profiling showed decrease of inflammation after RYGB. CONCLUSIONS: Gastric bypass reduces biochemical markers of inflammation, suggesting that obesity is an inflammatory condition.     

Dendritic cell infiltration in a patient with seminomatous germ cell tumor of the testis: is there a relationship with infertility and tumor stage?

PURPOSE: Inflammatory cells, such as dendritic cells, are considered to trigger the antitumoral immune response against tumors, such as testicular cancer. Male infertility associated with cancer may be due to endocrine or immunological factors. We investigated possible associations of antigen expression with dendritic cells, histiocytic cells and seminoma stage as well as with impaired spermatogenesis. MATERIALS AND METHODS: From 1992 to 1999, 30 patients with seminoma underwent orchiectomy at our center, including 14 who underwent spermiography before orchiectomy. Streptavidin-biotin immunostaining was performed on paraffin -embedded tumor specimens using antibodies against protein S-100 for dendritic cells and CD68-KP1 antigen. RESULTS: Light infiltration by less than 20 dendritic cells and less than 103 CD68+ cells was associated with tumor size greater than 1.5 cm. in 75% and 80% of patients, respectively. Strong infiltration by greater than 20 dendritic cells and greater than 103 CD68+ cells was associated with negative lymph nodes in 86% of patients. Slight infiltration by dendritic cells was observed in 71% of patients with a sperm count of greater than 8.6 million per ml. and in 100% with more than 45% motile sperm (p not significant and 0.02, respectively). Necrospermia increased with dendritic and CD68+ cell infiltration. No association was noted among preoperative serum tumor marker levels, the sperm count and immunostaining. CONCLUSIONS: Sperm autoimmunity is a plausible mechanism of infertility in men with germ cell tumors. Dendritic cells may induce antitumor cell cytotoxic reactions, but may also be cytotoxic to sperm cells or lead to inhibited spermatogenesis. Further studies focusing on tumor rejection antigen and the cloning of specific cytotoxic T lymphocyte against gametes are required to confirm these finding.     

Orbital and extraorbital giant cell angiofibroma: a giant cell-rich variant of solitary fibrous tumor? Clinicopathologic and immunohistochemical analysis of a series in favor of a unifying concept

The clinicopathologic and immunohistochemical features of one orbital and nine extraorbital soft tissue lesions, the morphology of which overlaps with giant cell angiofibroma and solitary fibrous tumor, are presented. There were 3 male and 7 female patients. Age at diagnosis ranged from 18 to 81 years (median: 45 yrs). Development of a mass was the main presenting symptom. For two patients, the lesion had been evident for several years before excision. Extraorbital tumors were located in the head and neck area (3), back (3), retroperitoneum (1), hip (1), and vulva (1). Tumor size ranged from 1.3 cm to 11 cm (median: 4.5 cm). The lesions presented grossly as well-demarcated, unencapsulated soft tissue masses. Histologically, they were characterized by the presence of alternating cellular and sclerosing areas, keloidal collagen deposition, round- to staghorn-shaped, thick-walled vessels and multinucleated giant stromal cells often lining pseudovascular spaces. Cellular areas were composed of non-atypical spindle to round cells set in a variably collagenous background. Mitotic activity ranged from 1 to 3 mitoses/10 high-power fields. Immunohistochemical studies showed positive staining of the spindle/round cells and multinucleated stromal cells invariably for vimentin, CD34, CD99, and mostly for bcl-2 but negative for muscle specific actin, desmin, CD31, CD117 (c-kit), and inhibin. Occasionally, focal reactivity was observed for smooth muscle actin, S-100 protein, epithelial membrane antigen, and keratin. Treatment consisted of simple tumorectomy in eight patients and wide excision in two. Follow-up information for eight patients (range: 7-32 mos; median: 14 mos), including four with microscopically positive surgical margins, showed no recurrence. These lesions share the clinical, pathologic, and immunohistochemical features of giant cell angiofibroma and solitary fibrous tumor, supporting the view that these tumors are closely related. In addition, it shows that giant cell angiofibroma occurs equally in both sexes and has a wider distribution than initially thought, developing even more often in extraorbital locations than in the orbit.