Long-term evaluation of a novel surgical approach to the pseudotumour of the ilium in haemophilia: exeresis and transposition of the omentum in the residual cavity.

Pseudotumour of the ilium is a rare but severe complication in haemophiliacs. Excision is often complicated by infections, fistulation and extensive pelvic bone destruction. In 1978, the first author carried out excision of the pseudotumour with transposition of the omentum in the dead cavity to avoid recurrence. This type of surgery has been carried out in three additional patients. The long follow-up of these four patients suggests that this procedure is feasible and curative; local bleeding, infection and fistulation did not recur and the patients remained ambulant with the aid of appropriate devices.     

Excision of pseudotumour in a patient with haemophilia A and inhibitor managed with recombinant factor VIIa

We describe a patient with haemophilia A and factor VIII inhibitor who underwent surgical excision of a large pseudotumour in the left femoral region. Haemostasis was successfully maintained with bolus doses of recombinant factor VIIa at 2-h intervals and anti-fibrinolytic therapy, and the pseudotumour was removed. Subsequently, the dose interval was gradually prolonged to 8 h for a total of 18 days. Although a spontaneous haemorrhage was observed on postoperative day 8, haemostasis was achieved by reducing the dosage interval. No adverse event occurred during the course of treatment.     

Inflammatory pseudotumour of the lung

Inflammatory pseudotumours of the lung are extremely rare. Their pathogenesis is controversial, their diagnosis is often difficult and their clinical behaviour may be unpredictable – ranging from benign to locally invasive, to metastatic in spite of an apparently ‘benign’ histology. A patient who presented with multiple recurrent lesions in the contralateral lung almost two years after the resection of a large primary tumour of the left upper lobe is reported.     

Recombinant factor VIIa in the management of a pseudotumour in acquired haemophilia

Bleeding in nonhaemophilic patients with high-titre factor VIII autoantibodies is often severe, life-threatening and refractory to treatment with factor VIII concentrates. In this report, we describe an elderly woman who required surgical excision of a large haemophilic pseudotumour adjacent to the left gluteal muscle. The Bethesda titre of 11 U precluded treatment with human factor VIII, and the patient had an anaphylactic reaction to porcine factor VIII. However, haemostasis was successfully achieved with recombinant factor VIIa and the pseudotumour was removed. The patient was treated with repeated cycles of cyclophosphamide, vincristine, and prednisone. The Bethesda titre eventually declined to 0.7 U and the factor VIII rose to 20%. During an 18-month follow-up period there has been no recurrence of bleeding or of the pseudotumour.     

Massive retroperitoneal pseudotumour in a patient with type 3 von Willebrand disease

Formation of destructive haemorrhagic pseudocysts or pseudotumours thought to arise from unresolved, encapsulated haematomas is a well-recognized, rare complication of severe haemophilia A or B, and has been reported in a single patient with von Willebrand disease (vWD). We report a 41-year-old patient with type 3 vWD who underwent incomplete resection of a large retroperitoneal pseudocyst in 1995 and presented with a recurrent, extensive right abdominal and flank mass and signs and symptoms of large bowel obstruction. He required emergency partial colectomy for bowel ischaemia and removal of his right kidney, which was hydronephrotic due to prolonged ureteral obstruction by the pseudocyst. Following repeat partial resection of the pseudotumour, he developed persistent bleeding into the operative site despite aggressive administration of von Willebrand factor (vWF)-rich factor VIII concentrates, resulting in retroperitoneal haematomas and abscesses, which resolved after 13 months of percutaneous drainage, extended supplementation of vWF and antibiotic therapy.     

Therapeutic embolization and surgical excision of haemophilic pseudotumour

Haemophilic pseudotumour is a rare complication of haemophilia. Few cases of iliac haemophilic pseudotumour have been reported in the literature. These tumours can act as a focus for infection and cause cutaneous fistulas. When they present perforations and infections of endogenous origin their course is usually fatal. Suitable treatment has been investigated on numerous occasions, most of the literature agreeing that the only curative treatment is surgical resection. We present a case of haemophilic pseudotumour of the iliac and caecum with cutaneous fistulas, with a septic process of endogenous origin. It was treated with surgical resection after performing arterial embolization to reduce the vascularization of the pseudotumour, thereby reducing its size and the risk of bleeding complications during surgery.     

Successful excision of a pseudotumour in a congenitally factor V deficient patient

We report a patient with congenital homozygous factor V deficiency in whom a large pseudotumour in the right upper leg was successfully surgically excised under continuous substitution with fresh-frozen plasma.     

Clinical and histological coexistence of inflammatory pseudotumour of the lymph nodes and rheumatoid arthritis

Inflammatory pseudotumour (IPT) of the lymph nodes is an uncommon, self-limiting, non-neoplastic proliferation of spindle cells, associated with a polymorphous inflammatory cell infiltrate embedded in a collagen-rich stroma and a variable degree of fibrosis, arising in the nodal parenchyma. Its clinical picture is characterised by site-specific signs and the presence, in most cases, of constitutional symptoms. The pathogenesis of IPT is unknown, but it has been interpreted as an aberrant reactive condition of the nodal connective framework, possibly related to viral infections or chronic inflammatory conditions. Its prognosis is usually favourable. We here report the simultaneous onset of seronegative rheumatoid arthritis (RA) and nodal IPT in a 31-year-old woman. Notably, in the nodal biopsy the coexistence of rheumatoid nodules, as well as histological and immunohistochemical features of IPT, was observed. To our knowledge, such an association has not been previously reported and the hypothesis that IPT could represent an unusual epiphenomenon of an RA-related chronic inflammatory response is suggested.Abbreviations RA Rheumatoid arthritis - IPT Inflammatory pseudotumour - IMT Inflammatory myofibroblastic tumours - ALK Anaplastic lymphoma kinase - MCP Metacarpophalangeal - PIP Proximal interphalangeal - ESR Erythrocyte sedimentation rate - EBV Epstein-Barr virus - 6-MP 6-methylprednisolone - MTX Methotrexate     

Haemophilic pseudotumour of the paranasal sinuses: management with radiotherapy and factor replacement therapy

A case of pseudotumour of the paranasal sinuses occurring in a patient with haemophilia A is reported. There was a favourable response to combined treatment with radiation therapy and factor VIII replacement.     

经皮肝Ⅰ期胆道造瘘联合胆道镜治疗胰十二指肠切除术后继发胆管结石

目的探讨经皮肝I期胆道造瘘(PTOBF)联合胆道镜治疗胰十二指肠切除术后继发胆管结石的安全性和有效性,探讨胰十二指肠切除术后胆肠吻合口狭窄的原因及处理对策。方法回顾性分析2017年10月至2021年5月于山东省第二人民医院接受PTOBF联合术中硬质胆道镜、术后电子胆道镜治疗的12例胰十二指肠切除术后继发胆管结石患者的临床资料。结果所有患者均成功行PTOBF,经胆道镜取净肝胆管结石,术中发现胆肠吻合口狭窄8例(线结性狭窄3例),经胆道气囊扩张及胆肠吻合口线结拆除,7例近期缓解(87.5%),1例再次胆肠吻合手术。结论PTOBF联合胆道镜治疗胰十二指肠切除术后继发胆管结石安全有效,取净率高,可缓解胆肠吻合口狭窄。     

The surgical treatment of a haeophilic pseudotumour in an extremity: a report of three cases with pathological fractures

A haeophilic pseudotumour is a rare complication of Haemophilia occurring in 1–2% of patients with a factor VIII or IX deficiency. This report presents three surgical cases of pseudotumours involved in a pathological fracture in the extremities. All cases showed a favourable post-operative course. If the preoperative management is appropriately designed, a limb salvage operation for a pathological fracture due to a pseudotumour could be carried out successfully. Before choosing amputation of a limb, the surgeon should consider the possibility of limb salvage.     

Regional pancreatectomy and transverse colectomy with mesenteric vascular reconstruction for inflammatory pseudotumour of the head of pancreas and mesenteric root

BackgroundInflammatory myofibroblastic pseudotumour is a rare pancreatic lesion.Case OutlineA 32-year-old woman with such a tumour was treated by a radical operation comprising proximal pancreatic-duodenectomy (Whipple Procedure) and transverse colectomy with resection and reconstruction of the superior mesenteric artery and vein. She remains well 6 years later.DiscussionThe importance of aggressive surgical clearance rather than chemotherapy is highlighted in the management of patients with these unusual tumours.     

经皮肝Ⅰ期胆道造瘘联合胆道镜治疗胰十二指肠切除术后继发胆管结石

目的探讨经皮肝I期胆道造瘘(PTOBF)联合胆道镜治疗胰十二指肠切除术后继发胆管结石的安全性和有效性,探讨胰十二指肠切除术后胆肠吻合口狭窄的原因及处理对策。方法回顾性分析2017年10月至2021年5月于山东省第二人民医院接受PTOBF联合术中硬质胆道镜、术后电子胆道镜治疗的12例胰十二指肠切除术后继发胆管结石患者的临床资料。结果所有患者均成功行PTOBF,经胆道镜取净肝胆管结石,术中发现胆肠吻合口狭窄8例(线结性狭窄3例),经胆道气囊扩张及胆肠吻合口线结拆除,7例近期缓解(87.5%),1例再次胆肠吻合手术。结论PTOBF联合胆道镜治疗胰十二指肠切除术后继发胆管结石安全有效,取净率高,可缓解胆肠吻合口狭窄。     

All-trans-retinoic acid (ATRA) responsive skin relapses of acute promyelocytic leukaemia followed by ATRA-induced pseudotumour cerebri

A 30-year-old woman with acute promyelocytic leukaemia (APL) went into complete remission following idarubicin and cytarabine chemotherapy; 18 months later she developed repeated skin relapse, with no bone marrow involvement. DNA and RNA analysis of skin lesions revealed the presence of the PML/RARα hybrid gene, which was not detected at the same time in bone marrow. The skin relapses were successfully treated by all- trans -retinoic acid (ATRA) as single agent over 2 years. However, prolonged administration of ATRA caused pseudotumour cerebri, which disappeared upon drug withdrawal. The absence of the hybrid gene in the bone marrow by RT-PCR analysis led to the patient being autografted.     

All-trans-retinoic acid (ATRA) responsive skin relapses of acute promyelocytic leukaemia followed by ATRA-induced pseudotumour cerebri

A 30-year-old woman with acute promyelocytic leukaemia (APL) went into complete remission following idarubicin and cytarabine chemotherapy; 18 months later she developed repeated skin relapse, with no bone marrow involvement. DNA and RNA analysis of skin lesions revealed the presence of the PML/RARα hybrid gene, which was not detected at the same time in bone marrow. The skin relapses were successfully treated by all-trans-retinoic acid (ATRA) as single agent over 2 years. However, prolonged administration of ATRA caused pseudotumour cerebri, which disappeared upon drug withdrawal. The absence of the hybrid gene in the bone marrow by RT-PCR analysis led to the patient being autografted.     

Inflammatory pseudotumour of the liver: a diagnostic dilemma.

Inflammatory pseudotumour is a rare, focal, benign inflammatory lesion of the liver parenchyma. It is largely a self-limiting entity and has favorable prognosis; it is thus important to preoperatively distinguish this lesion from malignancy, which it closely imitates. Inflammatory pseudotumour may present variously. We present the case of a 54-year old gentleman who presented with a three-month history of low-grade intermittent fever. Ultrasonography and computed tomography revealed a mass in the left lobe of the liver and the erythrocyte sedimentation rate was raised with coincident hypergammaglobulinaemia. A diagnostic laparotomy with left lateral hepatectomy was performed and histopathological evaluation of the specimen along with special staining and tissue culture revealed an inflammatory pseudotumour. On the second day post-operative the fever subsided and following an uneventful five days the patient was discharged and remains well at one-year follow up with no recurrence or relapse.     

Successful surgical treatment of haemophilic pseudotumour, filling the defect with hydroxyapatite

A preliminary report is made on the use of hydroxyapatite for surgical procedure of pseudotumour, a rare but serious complication of the bleeding diathesis in patients with inherited bleeding disorders. Surgical or percutaneous treatment and refilling with fibrin sealant is shown to be a successful therapy in a 19-year-old male with severe haemophilia B. The pseudotumour, in the upper pad of the left leg, was filled with hydroxyapatite after surgery. We suggest that the use of hydroxyapatite is a new and useful option in the surgical treatment of haemophilic pseudotumour.