Asymptomatic Paget’s bone disease in ethnic Thais: a series of four case reports and a review of the literature

Paget's bone disease is quite common in some parts of Europe and countries inhabited by European emigrants, but it is rare in Asia. There have been only 13 reported cases in Southeast Asia, including one reported case from Thailand. Half of the previously reported cases had bone symptoms and the other half were asymptomatic, but were incidentally discovered when patients were being investigated for other medical problems. Here are reported cases of four asymptomatic patients who presented elevation of serum alkaline phosphatase during routine annual medical checkups. All patients were of Chinese descent and all cases were proven by biopsy. Based on this experience, we are of the opinion that a substantial number of unrecognized cases of Paget's disease exist among ethnic Thais. We feel that they would be revealed if clinicians were alerted of its presence and if they included it as a possible diagnosis together with metastasis and osteoporosis when examining bone lesions or when results for elevated serum alkaline phosphatase are detected during routine checkups. We also anticipate that a higher prevalence of this disease may occur in future Thai generations due to the addition of offspring from Asian-European intermarriages to offspring of Chinese descent in the ethnic Thai population.     

Cementless total hip arthroplasty in Paget’s disease of bone: a retrospective review

Paget’s disease of bone (PDB) is a localised chronic osteopathy leading to bone deformities, bone hypervascularity, structural weakness and altered joint biomechanics. The pelvis and upper femur are frequently involved, resulting in disabling hip disease, and total hip arthroplasty (THA) may be required. We performed a retrospective study on the management and the outcome of 39 uncemented hydroxyapatite fully-coated THA in patients with PDB of the hip. The follow-up averaged 79.4 months (range 24–194). Functional scores improved significantly and, using the Harris hip score, 84% of patients had an excellent clinical outcome at the latest follow-up. Despite one case of an uncemented acetabular component with probable loosening, no implant revision had been required at our latest follow-up. Signs of implant loosening were found to be significantly more frequent in patients with active disease. For this reason, we advocate the use of pre-operative medication with bisphosphonates to reduce disease activity. Another benefit of this treatment is the significant decrease of intra-operative blood loss. Provided the control of disease activity in the pre-operative period with bisphosphonates is achieved, good outcome of cementless THAs can be expected. Bisphosphonates reduced the risk of implant loosening and excessive intra-operative blood loss.     

Treatment of giant cell tumor of bone: surgery or radiotherapy?]

The results of treatment for giant cell tumor of bone in 41 patients subdivided into 4 groups have been assessed. In group treated with radical surgery (amputations, extensive excision) 100% patients were cured. In group treated with curettage alone success rate was 33.3% and in radiotherapy group--72.3%. Curettage and radiotherapy combined rendered 95% success rate and this mode of treatment should be recommended.     

Stabilisation of acute femoral fractures in Paget’s disease

Eleven cases of acute femoral fractures in patients with Paget’s disease are presented following stabilisation with the solid AO femoral nail. In three cases with severe deformity of the femur, reaming was required to enable implant insertion. No corticotomies were required. The median operation time was 55 (35–65) min. There was no operative mortality and no patient developed signs of acute respiratory distress syndrome/fat embolism. Skeletal stability was achieved in all cases. The reconstruction proximal locking option (spiral blade) was utilised in ten of the eleven cases. In all cases a 9 mm nail was inserted. One patient (pagetic sarcoma) died 23 months after surgery. Ten of the eleven fractures healed uneventfully. The mean time to union was 32 (26–42) weeks.

---

Résumé  Nous présentons onze cas de fracture du fémur associée à la maladie de Paget que nous avons traité avec un enclouage solide verrouillé (SFN) de AO (Synthes). Dans trois cas la déformation du fémur était si sévère qu’il fallu faire un alésage du canal médullaire pour permettre l’introduction du clou. En aucun cas il a était nécessaire de faire une ostéotomie. Le temps médian opératoire était 55 minutes (35–65). La mortalité opératoire était nulle avec pas un seul cas de syndrome d’insuffisance réspiratoire ou d’embolie graisseuse pendant ou aprés l’opération. La stabilité de l’ostéosynthèse a été obtenue dans tous les cas. Dans 10 cas sur 11, l’option de verrouillage avec la lame spirale a été utilisé. Dans chaque cas un clou de 9 mm de diametre a été utilisé. Un malade (avec sarcome associéà la maladie de Paget) est mort 23 mois aprés la chirurgie. La consolidation de la fracture a étéétablie dans 10 cas sur 11 sans aucun problème. Le temps moyen d’union était 32 (26–42) semaines.

---

---

Accepted: 5 September 1999     

Perianal Paget’s disease

Perianal Paget’s disease is a rare intraepithelial adenocarcinoma of the anal margin. Because of its rarity, delay in diagnosis is common and requires a high index of suspicion. Although its etiology has never been definitively determined, it now appears that 2 types of perianal Paget’s disease exist. Primary perianal Paget’s disease likely represents intra-epithelial neoplasm from an apocrine source, whereas secondary disease may represent “pagetoid” spread from an anorectal malignancy. Diagnosis of the 2 types of disease can be established from distinct immunohistochemical stain patterns. Wide local excision remains the treatment of choice for primary disease, with or without local invasion. Although local recurrence is high, re-excision can still be curative. Treatment of secondary disease is less clear, though aggressive therapy appears indicated for curative intent. Although adjuvant therapy is desirable, experience is still very limited.     

Zoledronic acid-induced transient hepatotoxicity in a patient effectively treated for Paget��s disease of bone

Bisphosphonate (BP)-induced hepatotoxicity is very rare. There are only a few reports of liver injury after BP treatment, including aledronate and risedronate in postmenopausal osteoporosis patients. We describe hereby the case of a patient with Paget’s disease of bone accompanied by nonalcoholic fatty liver disease (NAFLD) who developed transient hepatotoxicity after zoledronic acid (ZOL) treatment. NAFLD had been diagnosed 1 year before presentation, based on liver ultrasonography (US). One day after infusion, serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), and gamma-glutamyltransferase (GGT) were increased by 8.1, 6.7, and 6.7 times, respectively, compared with pretreatment values. Serum bilirubin remained normal. US revealed hepatic mild homogenous brightness without focal lesion of the liver or biliary ducts. Subsequent biochemical and serologic investigation did not reveal a specific liver or systematic disease. The patient remained asymptomatic, and ALT, AST, and GGT were normalized 7 days post-treatment. Although the mechanism by which ZOL may cause liver damage is elusive, physicians should be aware of this possible adverse effect and ZOL cautiously administered in NAFLD patients.     

Perianal Paget’s disease: case report and review of the literature

A peri-anal skin lesion, often eczema-like and with symptoms of pruritus, that does not resolve after classical local therapy should be biopsied. We present a case of peri-anal extramammary Paget’s disease (EMDP) and associated anal adenocarcinoma. Reviewing the literature, more than 30% of patients with EMDP present a second primary tumour in their past, present or future history. In Europe, the risk of developing a new primary tumour in patients with this condition is increased compared with the standard population. In cases of peri-anal Paget’s disease (PPD), specific histochemical markers allow us to differentiate between a primary and a secondary form, the secondary one is strongly associated with colorectal and anal tumours. We provide information about the most commonly suggested therapy for PPD with or without associated malignancy and about the recommended follow-up.     

Efficacy, tolerability, and safety of risedronate in Japanese patients with Paget’s disease of bone

This study evaluated the clinical efficacy of treatment with oral risedronate (17.5 mg once daily) for 8 weeks in 11 Japanese patients with Paget’s disease of bone (PDB). Risedronate suppressed the excessive bone turnover associated with PDB and improved several biochemical markers, including serum alkaline phosphatase (ALP), serum bone-specific ALP (BALP), urinary deoxypyridinoline (DPD), and urinary cross-linked N-telopeptide of type 1 collagen (NTX). These markers began to decrease within about 2 weeks after the initiation of treatment in most patients, and the response persisted for up to 40 weeks after the cessation of treatment. Risedronate reduced pain by week 24 in most patients. According to quantitative bone scintigraphy, the lesion with the highest radioisotope (RI) uptake showed a decrease of uptake from 12.7 ± 6.8 to 6.0 ± 2.3 (mean ± SD) in week 24, although each lesion of patients with polyostotic disease had a different scintigraphic response. Overall, risedronate at a dose of 17.5 mg once daily was well tolerated by patients with PDB, even though the dosage was seven times higher than that approved for the treatment of osteoporosis in Japan (2.5 mg once daily). In conclusion, treatment with high-dose risedronate for 8 weeks resulted in clinically significant and sustained improvement of biochemical markers of bone turnover for 48 weeks in patients with PDB, and this improvement was associated with a decrease of RI uptake by Paget’s bone lesions and with reduced pain.     

Double anal canal cancers associated with a long history of perianal Paget’s disease: report of a case

Extramammary Paget's disease (EPD) is rare. We report a case of double anal canal cancers in a patient with a long history of perianal Paget's disease. The patient, a 68-year-old Japanese woman, refused surgery initially and was treated with electron beam therapy, which achieved remission. However, 6 years later, Paget's disease was found to be progressing again and double anal canal tumors were also detected in the proctos and external skin area. We performed abdominoperineal resection (Miles' operation) and lymph node dissection for the Paget's disease with double anal canal tumors. Immunohistochemical staining revealed cytokeratin (CK)-20 expression in the adenocarcinomas and Paget's disease lesion, but not CK-7 or gross cystic disease fluid protein-15 expression. The lesion was joined to the carcinoma by a stalk. The immunohistochemistry results suggested secondary EPD, although it was originally considered to be Paget's carcinoma (primary EPD) based on the clinical history.     

Castleman’s disease in the posterior mediastinum: Report of a case

(Received for publication on June 4, 1999; accepted on Nov. 11, 1999)     

Clinical research of holmium laser therapy in extramammary Paget’s disease

This study aims to investigate the safety and efficiency of the holmium laser therapy in extramammary Paget’s disease. The clinical data of 61 patients was collected since 2002 to 2012, confirmed as non-subcutaneous invasive extramammary Paget’s disease by biopsy and underwent surgery. All patients were divided into two groups. Group A included 30 patients who underwent the holmium laser therapy. Group B included 31 patients who underwent the traditional surgical therapy. The clinical data of all patients included preoperative, intraoperative, and postoperative management and follow-up records. Compared with the traditional operation group, the holmium laser group had a shorter operation time and was easier to perform. There were no significant differences between the two groups in cases of intraoperative and postoperative complications, the recurrence-free survival, and the disease-specific survival. But the holmium laser group had a longer recovery time than the traditional operation group in large and deep nidus. Multiple-factor analysis of prognostic parameters of 61 patients confirmed that any of these two methods chosen was not a prognostic parameter for recurrence-free survival. The holmium laser therapy might prove to be a preferable alternative to the traditional operative therapy of extramammary Paget’s disease. However, the holmium laser therapy did not demonstrate to have an obvious advantage over traditional operative therapy in the recurrence-free survival and the disease-specific survival.