|Year : 2020 | Volume
| Issue : 4 | Page : 329-331
Pagetic osteosarcoma detected in bone scintigraphy
Junita Rachel John, Julie Hephzibah, Regi Oommen, Nylla Shanthly
Department of Nuclear Medicine, Christian Medical College, Vellore, Tamil Nadu, India
|Date of Submission||02-Jun-2020|
|Date of Decision||27-Jun-2020|
|Date of Acceptance||03-Jul-2020|
|Date of Web Publication||19-Oct-2020|
Dr. Julie Hephzibah
Department of Nuclear Medicine, Christian Medical College, Vellore - 632 004, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Paget Disease (PD) is a disorder of the skeleton characterized by increased osteoclastic activity. Sarcomatous transformation, though rare, is a serious complication of PD and begets a poor prognosis. Bone scintigraphy is a useful tool in the early identification of fractures and osseous sarcomas. We report a case of PD presenting with sarcomatous transformation as the primary symptom which has rarely been described in the Indian population.
Keywords: Osteosarcoma, Paget's disease, pagetic sarcoma
|How to cite this article:|
John JR, Hephzibah J, Oommen R, Shanthly N. Pagetic osteosarcoma detected in bone scintigraphy. Curr Med Issues 2020;18:329-31
| Introduction|| |
Paget's disease (PD) of bone, also known as osteitis deformans, was first defined by Paget in 1877. PD arises mainly in the elderly and is described as the interruption of normal bone remodeling. Sarcomatous transformation, though rare, is a serious complication of PD of bones. Unlike primary osteosarcoma, prognosis in pagetic osteosarcoma is poor.
| Case Report|| |
A 58-year-old gentleman presented with a 5-month history of insidious onset of swelling over his right arm which was initially small but then rapidly progressed in size. Pain was localized, aggravated on lifting heavy objects, and relieved with medications. There was a history of rest pain and night pain. General physical and systemic examination was unremarkable. Local examination revealed a 20 cm × 15 cm swelling present over the mid-right arm, which was oval in shape extending anteriorly, medially, and laterally from 6 cm below the acromioclavicular joint to 4 cm above the cubital fossa. Margins of the swelling were ill defined, and there was diffuse tenderness present over it along with engorged veins. External rotation and internal rotation was restricted due to pain. His serum alkaline phosphatase (ALP) was 1980, and radiographs of the right shoulder and humerus showed a large ill-defined lobulated lesion with a wide zone of transition and extensive osteoid matrix arising from the proximal and midshaft of the humerus. A Tc99m MDP bone scan done showed polyostotic Paget's disease (PD) with irregular intense uptake of the midshaft of the right humerus [Figure 1]. Needle biopsy from the right humerus was suggestive of intermediate grade osteosarcoma. Computed tomography thorax done revealed few lung nodules suspicious of metastases. Salvage versus disarticulation of the shoulder was discussed with the patient. Considering the extent of the tumor, extracompartmental staging, encasement of neurovascular structures, probable lung metastases, and poor prognosis of Paget's sarcoma, it was decided to do a shoulder disarticulation. The surgical histopathology [Figure 2] report was confirmatory of the same. His ALP came down to 881 postsurgery, and he was initiated on adjuvant chemotherapy – six cycles of cisplatin and doxorubicin.
|Figure 1: Bone scintigraphy – Tc99mMDP bone scan done showed polyostotic Paget's disease involving skull, humerus, sternum, multiple ribs, femoris, and right proximal tibia with irregular and intense uptake in the mid-shaft of the right humerus (arrow insert).|
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|Figure 2: Histology – Lace-like and trabecular neoplastic osteoid seen amidst tumor cells.|
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| Discussion|| |
PD is a localized disorder of the skeleton characterized by increased osteoclastic activity. The prevalence in the Western population is 1%–2%, and the prevalence in India is unknown due to a lack of community data. A study reviewing all cases of pagetic sarcoma at the Royal Orthopaedic Hospital in Birmingham, UK, since 1975, suggested a declining incidence of sarcoma in PD of bone to about 0.3%. A retrospective review by Anjali et al. of 51 patients seen over 8 years found that their clinical features and pattern of presentation were quite similar to that reported from the West. The study showed polyostotic involvement in 90.2% of the patients, and the symptoms at presentation were bone pain in 65%, skeletal deformities in 37%, and pathological fractures in 20%; 10% were incidentally diagnosed during the evaluation of elevated serum ALP. None of the study patients had high output cardiac failure or osteosarcoma of the bones though the mean follow-up period was only 43 months.
Pagetic sarcomas may present with worsening pain, lytic lesion on X-ray, fracture, a new mass, or rising ALP. The most common bones involved in pagetic osteosarcoma include pelvic bones, femur, humerus, tibia, and skull. Interestingly, despite being frequently involved by PD, osteosarcoma is uncommon in the pagetic spine. The number of sites affected by nonmalignant PD in a skeleton is correlated with markers of both bone resorption and formation. Although osteosarcoma (81%) is the most common malignant transformation associated with PD, the other neoplastic forms include fibrosarcoma (14%), chondrosarcoma (3%), malignant fibrous histiocytoma (1%), malignant giant cell tumor, and lymphosarcoma (0.5%).
Our patient presented with pain and visible swelling over the right arm with an elevated ALP level. An X-ray [Figure 3] of the right humerus showed a large ill-defined lobulated lesion with a wide zone of transition and extensive osteoid matrix seen arising from the proximal and midshaft of the humerus. Areas of cortical destruction are particularly useful in the detection of malignant degeneration. A high index of suspicion must be maintained when evaluating radiographs of patients with PD, especially of those presenting with pain or a palpable mass.
|Figure 3: Plain radiograph – A large ill-defined lobulated lesion with a wide zone of transition and extensive osteoid matrix arising from the proximal and midshaft of the humerus.|
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Bone scans are generally of value in detecting complications of PD such as fracture or sarcoma. On bone scintigraphy, sarcomatous degeneration may appear as a “cold” area of absent uptake, representing the decreased accumulation of the tracer at the site of neoplasm and necrosis. Occasionally, a cold area within high uptake is present.
| Conclusion|| |
Sarcomatous transformation in PD, a known complication, presenting as the first symptom has rarely been described in the Indian population. A combination of tests – biochemical, scintigraphy, radiological, and pathology – aids in the prompt diagnosis in the current era. Bone scintigraphy is a useful tool to identify sarcomatous transformation in PD.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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