A case of a 32-year-old female patient with a giant cell tumor originating in the middle part of the left 10th rib is presented. On X-rays and CT, the tumor caused a well-defined osteolysis with nonsclerotic borders. On MRI, it exhibited intermediate signal intensity on T1 sequences and central high signal and peripheral intermediate signal on T2 sequences. On contrast-enhanced MR images both central and peripheral-periosteal enhancement was noted. Thanks to its small size (2 × 1.3 cm), the lesion was easily resected en bloc with a part of the affected rib. The patient is free of recurrence for 3 years after the operation.
Central giant cell granulomas are non-neoplastic lesions of unknown etiology. They affect females more than males with the mandibular anterior region being the most common site of occurrence. Clinically central giant cell granulomas present as asymptomatic, expansile swellings causing deviation of associated teeth. Radiologically they usually presents as multilocular lesions causing expansion or perforation of cortical bone. Central giant cell granulomas are usually confused as other lesions both clinically and radiologically, and a definitive diagnosis can be made only histologically. We report here a rare case of central giant cell granuloma in association with congenitally missing tooth which was misdiagnosed to be an adenomatoid odontogenic tumor both clinically and radiologically. This case report also highlights yet another unique presentation of central giant cell granulomas that is in association with a congenitally missing maxillary lateral incisor.
Adenomatoid odontogenic tumor; central giant cell granuloma
The authors report the case of an 81-year-old male who presented with a 3-year-history of a bluish, nodular tumour located on the extensor side of his right forearm. Subjective symptoms included tenderness upon palpation and spontaneous haemorrhage. In order to exclude malignant neoplasms, for example, nodular melanoma, metastatic melanoma or angiosarcoma, the tumour was surgically removed and tissue submitted for microscopic examination. Histologically, the authors diagnosed this as giant vascular eccrine spiradenoma, a rare variant of eccrine spiradenoma, which can easily be mistaken for angiomatous lesions due to the haemorrhagic features and florid vascularisation. It is our aim to help clarify the diagnosis and differentiate giant vascular eccrine spiradenoma from other painful cutaneous tumours exhibiting a high degree of vascularisation, for example, angiosarcoma or venous thrombosis, as this case represents one of only seven found in published literature.
A case of osteoclast-type giant cell tumour of the pancreas is described and the features of eight other previously reported patients are reviewed. Characteristically, these neoplasms are large at presentation and show focal haemorrhage and necrosis, but seem slow to give rise to metastases. Histological examination reveals numerous osteoclast-like giant cells set in a sarcomatous stroma, the appearances being similar to those seen in giant cell tumours of bone. They are distinct from pleomorphic giant cell carcinomas of the pancreas and may have a slightly better prognosis after resection than ordinary adenocarcinomas. The histogenesis of these rare tumours is unknown.
Giant cell tumor (GCT) is an aggressive, but usually benign bone neoplasm most commonly arising in the metaphysis/epiphyses of long bones. While they are categorized as benign tumors, they can be locally aggressive and clinically have metastatic potential. The most common locations of this tumor include the distal femur, proximal tibia, and distal radius. We report a GCT arising in an atypical location and mimicking a breast mass.
This case was diagnosed at a large cancer center in Florida. Pertinent clinical findings were obtained from chart review and inter-departmental consultation. Radiologically, the initial impression included a deep-seated breast cancer with local chest wall invasion. Further evaluation revealed the mass to be an expansile rib lesion with extraosseous soft tissue invasion. Histological examination of the biopsy specimen showed bland multi-nucleated giant cells and mononuclear cells whose nuclei were morphologically similar. No necrosis, pleomorphism or mitotic activity was identified. No chondroid or osseous elements were present.
The histological features of bland mononuclear and multinucleated giant cells along with the lack of any additional mesenchymal elements led to the diagnosis of giant cell tumor. Resection of tumor was performed. The patient is disease free as of the last follow-up visit. This case is important as it shows where the physician must keep this diagnosis in mind whenever a deeply located breast mast is present.
Limited literature is available on the reconstruction of the distal radius using prosthetic replacement following resection of a bone tumour. We present the first reported case, in the English literature, of the use of an entirely metal endoprosthesis for the reconstruction of the distal radius. This case involves a 66-year-old male who was treated for giant cell tumour of the distal radius with surgical excision of the lesion and replacement of the defect using a predominantly titanium endoprosthesis. He was followed-up for 56 months following surgery and had a good functional outcome with no associated pain or complications. We propose that the use of a primarily titanium endoprosthesis for the reconstruction of a bone defect of the distal radius is a suitable alternative, providing good function of the forearm with satisfactory range of movement at the wrist and adequate pain relief.
Benign fibro—osseous lesions (BFOLS) of the jaws are a wide array of lesions that actually represent distinct phases of a single benign morphological process. These lesions share certain histopathological features which are in common with giant cell containing lesions, which include central giant cell granulomas (CGCGs). The association of BFOLS and CGCG has to be critically evaluated, pertaining to their clinical, radiologic and histologic features. Many pathologists diagnose these types of lesions, considering only one of the prominent features. Eventually, surgeons end up treating these lesions inadequately. This ambiguity may be because of very small number of cases have been reported in the literature, with uncertain clinical, radiologic and histologic features. We are reporting a case of fibrous dysplasia (FD) which was associated with a central giant cell granuloma (CGCG) and discussing the hypothetical pathogenesis of giant cells.
Fibrous dysplasia; Granuloma giant cell; Hybrid; Dimorphic; Stromal cells; Osteoclasts
A 42-year-old man with Erdheim-Chester disease (EC) is presented. This is the first case of this disease reported in Korea. The patient complained of knee pain and plain roentgenogram of the bilateral legs revealed diffusely increased density, coarsened trabecular pattern, and cortical thickening in the diaphysis, and metaphysis as well as epiphysis. Magnetic resonance imaging revealed that the lesions showed low signal intensity on T1-weighted images and heterogeneously low and high signal intensity on T2-weighted images. Histological examination of the biopsy specimen showed a xanthogranulomatous lesion consisting aggregations of foamy histiocytes and Touton-type giant cells. Immunohistochemical staining showed positive reaction to anti-S-100 and lysozyme in the cytoplasm of the giant cells.
Fibro-osseous lesions of the jaws can have certain histologic features in common with central giant cell granuloma (CGCG) including the presence of multinucleated giant cells. The clinical, radiologic and histologic features of these lesions should be carefully evaluated to distinguish between these conditions. Fibro-osseous lesions of the jaws are a heterogenous group of lesions characterized by the replacement of normal bone by fibrovascular tissue containing newly formed mineralized material. Central giant cell lesions are defined as an intraosseus lesion consisting of cellular fibrous tissue containing multiple foci of hemorrhage and aggregation of multinucleated giant cells. These lesions may sometimes lead to a confusion in their diagnosis as many pathologists report them taking into consideration one of the prominent histopathologic feature. These confusions may be because of the small number of cases reported in the literature with uncertain clinical, radiographic and histopathologic features of these lesions. So even surgeons may end up treating these lesions inadequately or patients may need to undergo multiple surgeries. We report such a case of Juvenile ossifying fibroma associated with CGCG and discuss the clinical, imaging, histologic, and treatment aspects of this hybrid lesion.
Central giant cell granuloma; fibro-osseous lesions; hybrid lesion
We report an atypical case of malignant round cell tumour in a 25-year-old man who presented with pain in the left hip of insidious onset and long duration of about 1½ years. Radiologically, the tumour was meta-epiphyseal in location with sparing of the diaphysis and showed a defined, narrow zone of transition. These appearances are contradictory to the well documented classical features of malignant round cell tumours, which usually present with a short history, are meta-diaphyseal in location, and appear ill-defined with a wide zone of transition due to infiltration of the adjacent bone.
The present article is a case report of a 16-year-old boy who presented with a benign bony tumour, which on histological analysis suggested giant cell reparative granuloma (GCRG), but was not corroborate by blood tests. The implications of this type of tumour and the correct diagnostic requirements were investigated. The correct identification of GCRG from other giant cell-containing tumours is important because the treatment modalities for these tumours significantly differ from one another. In most cases, histological findings are sufficient to identify the tumours. In most GCRG cases, curettage is usually a curative treatment option. However, due to high recurrence rates of GCRGs, close follow-up of these patients is warranted. Also, due to osteoclastic activity of the giant cells in GCRGs, the use of drugs such as calcitonin or bisphosphonates, which inhibit osteoclast differentiation and activation, may have an important influence on future treatments or in reducing the recurrence rate of these tumours.
Bony tumour; Giant cell granuloma; Phalanx
Primary hyperparathyroidism is a common endocrine disorder characterized by elevated parathyroid hormone levels, which cause continuous osteoclastic bone resorption. Giant cell tumor of bone is an expansile osteolytic tumor that contains numerous osteoclast-like giant cells. There are many similarities in the radiological and histological features of giant cell tumor of bone and brown tumor. This is a rare benign focal osteolytic process most commonly caused by hyperparathyroidism.
We report the unusual case of a 40-year-old Caucasian woman in which primary hyperparathyroidism was diagnosed after surgical ablation of a costal mass. The mass was suspected of being neoplastic and histopathology was compatible with a giant cell tumor of bone. On the basis of the biochemical results (including serum calcium, phosphorous and intact parathyroid hormone levels) primary hyperparathyroidism was suspected and a brown tumor secondary to refractory hyperparathyroidism was diagnosed.
Since giant cell tumor is a bone neoplasm that has major implications for the patient, the standard laboratory tests in patients with bone lesions are important for a correct diagnosis.
BACKGROUND: Giant-cell tumour of the distal radius is a rare neoplasm that affects the peri-articular metaphysial region of the bone. Curettage alone or with bone grafting has been reported to be associated with high incidence of local recurrence in these tumours. In the present series, we report the results of curettage only as the treatment for primary giant-cell tumour of the distal radius carried out at a single centre. PATIENTS AND RESULTS: A total of 287 patients with giant-cell tumour have been referred to us for treatment over the last 28 years; 24 of these were found to have lesion in the distal radius. One patient underwent endoprosthetic replacement of the distal radius. The remaining 23 patients underwent curettage of the primary neoplasm. Four out of the 23 (17%) patients developed local recurrence of disease, The mean time to local recurrence was 17 months (range, 9-27 months). Complications such as collapse of the articular cartilage are more common in patients with an extensive soft tissue component of the tumour. CONCLUSIONS: Curettage alone is adequate treatment for the majority of patients with giant-cell tumours of the distal radius but some form of stabilisation may be required in the presence of extensive bone destruction.
A classic case of central giant cell lesion (CGCL) is presented with emphasis on clinical, radiologic, and histologic features. The differential is discussed including peripheral giant cell granuloma, brown tumor of hyperparathyroidism, and giant cell tumor of bone. The molecular pathway of osteoclastogenesis is selectively reviewed and applied to suggest possible etiologies of the giant cell lesions. CGCL syndromes and treatment are also discussed.
Central giant cell lesion; Giant cell reparative granuloma; Osteoclast; Giant cell; RANK; RANKL; NFAT; NFATc1; SH3BP2; Peripheral giant cell granuloma; Giant cell tumor of bone; Brown tumor of hyperparathyroidism; Osteitis fibrosa cystica
Keratoacanthomas are fast-growing, solitary, cutaneous neoplasms that usually show spontaneous regression. The development of giant variants and aggressive behavior have been described. Clinically, a keratoacanthoma larger than 20 to 30mm is classified as a giant keratoacanthoma. A major challenge in dealing with these neoplasms is the difficulty of clinically and histologically differentiating them from squamous cell carcinoma. The authors report a practical approach using Mohs micrographic surgery for evaluation of large tumors. With this method, the lateral margins are evaluated and cleared prior to excision of the bulk of the tumor. The authors also describe alternative therapies for giant keratoacanthomas and present a case of a 61-year-old woman with a rapidly growing tumor on her left arm. Skin biopsy was consistent with a well-differentiated squamous cell carcinoma with focal features of a keratoacanthoma. The patient underwent Mohs micrographic surgery using the described approach, and no recurrence has been noted in four years. Surgical excision remains the treatment of choice for giant keratoacanthomas. Mohs micrographic surgery is a logical treatment option for giant keratoacanthomas. This case illustrates a useful approach that may prove valuable when treating large specimens during Mohs micrographic surgery.
Spindle cell oncocytoma of the adenohypophysis is a rare tumour recently reported by Roncaroli et al. in 2002. This tumour is considered a grade I tumour by the World Health Organization.
We describe what is, to the best of our knowledge, the 14th case of its kind in the literature. A 45-year-old African woman presented clinical and radiological findings related to a nonfunctioning pituitary adenoma. The diagnosis was made on the basis of histological and immunohistochemical findings.
The purpose of this work is to report a rare pituitary tumour and to describe its histological and immunohistochemical features, which were characterized by the expression of thyroid transcription factor 1 antigen by tumour cells. This fact could support the theory of a possible common origin of these tumours in pituicytomas. In fact, thyroid transcription factor 1 is considered to be a specific marker of pituicytes.
Myxofibrosarcoma is one of the most common sarcomas in elderly patients showing a slight male prevalence. The tumor is mainly located in lower and upper extremities and rarely in trunk, neck and feet. We present a case of a 84-year-old man referred to our tumour center with a giant and neglected high-grade tibial myxofibrosarcoma in the anteromedial side of tibial mid-diaphysis. Large size lesions in association with older age may jeopardise the maintenance of limb vitality, vascularity and stability.
Authors performed a complete tumour resection, followed by reconstruction of bone and soft tissue defects with cement, plate and a musculocutaneous gastrocnemius flap, in order to cover the underlying bone and promote uneventful healing and perfusion of the operated extremity.
At 2 years postoperatively, limb salvage, good functional outcome and no tumour recurrence were reported while the patient was able to effectively perform the majority of the daily activities.
The giant cell tumour of bone (GCT) is a locally aggressive intraosseous neoplasm of obscure biological behaviour. Although well defined in clinical, radiological and histological terms, detailed information on its biological development is still relatively incomplete. The tumoral tissue consists of three cell types – the neoplastic giant cell tumour stromal cells (GCTSC), representing the proliferative fraction, secondarily recruited mononuclear histiocytic cells (MNHC) and multinuclear giant cells (MNGC). These cellular components interact together with factors that have a role in regulating osteoclast function in normal bone tissue (e.g. RANK, RANKL, OPG, M-CSF). Recent publications suggest that the neoplastic stromal cells express differentiation features of mesenchymal stem cells. Further research of the pathogenesis of GCT as well as the complex interactions of its cellular populations may provide the knowledge necessary for developing approaches for a biological-based therapy of this neoplasm.
Objectives. The present study was conducted to categorise the morphological features on bone marrow aspirate cytology into common, uncommon, and atypical features in a nonendemic region which would be helpful in clinching an early and correct diagnosis especially in clinically unsuspected cases. Methods. The morphological features on bone marrow were categorized into common, uncommon, and atypical in cases of leishmaniasis from non endemic region. Results. Out of total 27 cases, 77.7% were residents of places at the height of 500 m or above and fever was the most common presentation followed by hepatosplenomegaly. Plasmacytosis, hemophagocytosis were the common cytological features while dysmyelopoiesis, presence of leishmania bodies in nonhistiocytic cells, and granuloma with necrosis were uncommon features. Aggregates of LD bodies in form of ring, floret, or strap shapes along with giant cells constitute the atypical morphological features. Conclusion. The knowledge of common, uncommon, and atypical features on bone marrow aspirate cytology is helpful in clinching an early and correct diagnosis of leishmaniasis especially in non endemic areas where clinical suspicion is low. These features will guide the pathologist for vigilant search of LD bodies in the marrow for definite diagnosis and thus will also be helpful in preventing unnecessary workups.
Giant cell tumours of the clivus are rare. These tumours present in the second and third decades of life and they are slightly more frequent in women than in men. We are presenting a case of a 20 years young patient who came with the complaints of headache, retro-orbital pain and recurrent transient bleeding from the nose since two and half months. MRI of the brain with contrast was done and its features were suggestive of a Giant cell tumour of the clivus. A transnasal endoscopic biopsy was done under general anaesthesia and the histopathology report suggested that the features were of a giant cell tumour. Excision of the mass was done by Transnasal endoscopy. Post operatively, the patient did not recover from the lateral rectus palsy which was there on the right side. The patient was discharged with an advice of a follow up and radiotherapy. Radiation therapy and chemotherapy may be effective as adjuvant treatments. Even though a recurrence usually occurs within 4 years of the initial treatment, these patients will need to be carefully followed for the remainder of their lives.
Clivus; Tumour; Osteogenic; MRI
Compression of the median nerve by a tumour in the elbow and forearm region is rare. We present a case of neuropathy of the median nerve secondary to compression by giant lipoma in the proximal forearm.
A 46-year-old man presented with a six month history of gradually worsening numbness and paresthesia on the palmar aspect of the left thumb and thenar eminence. Clinical examination reveals a hypoaesthesia in the median nerve area of the left index and thumb compared to the contralateral side. Electromyography showed prolonged sensory latency in the distribution of the median nerve corresponding to compression in the region of the pronator teres (pronator syndrome). Radiological investigations were initially reported as normal. Conservative treatment for one month did not result in any improvement. Surgical exploration was performed and a large intermuscular lipoma enveloped the median nerve was found. A complete excision of the tumour was performed. Postoperative revaluation the X-ray of the elbow was seen to demonstrate a well-circumscribed mass in the anterior aspect of the proximal forearm. At follow-up, 14 months after surgery, the patient noted complete return of the sensation and resolution of the paresthesia.
In case of atypical findings or non frequent localization of nerve compression, clinically interpreted as an idiopathic compression, it is recommended to make a pre-operative complementary Ultrasound or MRI study.
Giant cell rich osteosarcomas, histological variant of conventional osteosarcomas account for 3% of all cases of osteosarcomas and most of them are arise from the appendicular skeleton. Giant cell rich osteosarcomas have been rarely reported in the head and neck region. This is the first case report of giant cell rich variant of osteosarcoma in the maxilla. It is important to recognize this variant, as its prognosis is worse as compared to conventional osteosarcomas of the head and neck region. We here present a case of giant cell rich variant of osteosarcoma of the maxilla and along with its characteristic radiological and histopathological picture.
Giant cell variant of osteosarcoma; Maxilla; Worse prognosis
A 45 year old man presented with abdominal pain, loss of appetite, and significant weight loss over a period of about four weeks. Imaging of the abdomen showed a mass in the region of the head of the pancreas. In view of the size of the mass and the clinical picture, a Whipple’s procedure was performed. Histological evaluation of the pancreatic tumour showed an adenosquamous carcinoma (predominantly composed of squamous carcinoma), which was extensively infiltrative with perineural invasion and involvement of peripancreatic lymph nodes. Areas of pancreatic intraepithelial neoplasia grade III and merging of the squamous and adenocarcinoma components were evident. Unusual histological features that characterised this case included a pronounced acantholytic pattern within the squamous carcinoma component, and the presence of both osteoclastic and pleomorphic giant cells. Giant cells have not been documented previously in association with an adenosquamous carcinoma. Although an acantholytic pattern has been noted in squamous carcinomas in other sites, this is the first report of such a pattern in an adenosquamous carcinoma of the pancreas.
pancreas; adenosquamous carcinoma; giant cells; osteoclasts
We reviewed the literature to evaluate the demographic, clinical and histological profile of giant cell tumour of tendon sheath of the digits (GCTTSD). The overall recurrence rate and the factors affecting tumour recurrence were also assessed.
We searched for published articles regarding the GCTTSD in the English literature the last 30 years using the PubMed search engine. All retrieved papers were analysed and their reference lists were also screened if relevant. Clinical studies with less than five patients and follow-up less than 2 years were excluded from further evaluation. For each report, information was gathered related to trial characteristics and study population. Location and multicentricity of lesions, kind and severity of symptoms, type of applied treatment modality and histopathological features of the excised tumours were additionally recorded. A meta-analysis for estimating the pooled recurrence rate after surgical excision was also conducted. Statistical significance was assumed for p ≤0.05.
We found 21 studies with histological confirmation of GCTTS. However, only 10 studies including 605 patients were reviewed according to selection criteria (average follow-up 36.7 to 79 months). The male-to-female ratio was 1:1.47 (p < 0.005) and the mean age ranged from 32 to 51 years. Pain or sensory disturbances reported only in 15.7% and 4.57% of cases, respectively. A definite history of trauma recorded in 5% of lesions. The most frequent tumour location was the index finger (29.7%). In total, 14.8% of patients had tumour recurrence. Type I tumours (single lesions) were more frequently detected (78.7%) than type II tumours (two or more distinct tumours that were not joined together) (21.3%) but the latter were associated with a higher recurrence rate (p < 0.001). Study design also affected the possibility of recurrence as it was lower in prospective studies compared to retrospective studies (p = 0.003). Even though bone erosion was detected in 28.39%, recurrence was not more common in this group. In addition, recurrence was not significantly associated with a specific finger or phalanx.
Intrinsic biology of the tumour seems to play a more fundamental role in recurrence than tumour location or local invasiveness. More prospective well-designed studies including a large number of cases are necessary to identify tumours prone to recurrence and determine the proper treatment protocol for each individual patient.
Giant cell tumour; Tendon sheath; Digits; Hand
A 27-year-old man treated 2.5 years ago for synchronous multicentric giant cell tumor of bone located at the right proximal humerus and the right 5th finger presented now with complaints of pain in his right hip and wrist of two-month duration. Radiology and magnetic resonance revealed multicentric giant cell tumor lesions of the right proximal femur, the left ileum, the right distal radius, and the left distal tibia. The patient has an eighteen-year history of a healed osteosarcoma of the right tibia that was treated with chemotherapy, resection, and allograft reconstruction. A literature review establishes this as the first reported case of a patient with synchronous and metachronous multicentric giant cell tumor who also has a history of osteosarcoma.