Tumoral calcinosis in the upper cervical spine causing progressive radiculomyelopathy--case report

A 54-year-old woman with chronic renal failure presented with tumoral calcinosis manifesting as progressive radiculomyelopathy. Magnetic resonance imaging revealed a spinal epidural mass in the C-2 to C-4 levels. The clinical and radiological findings suggested malignant tumor. Resection of the lesion was performed with total C-2 laminectomy and C-3 and C-4 laminoplasty. The symptoms totally disappeared after surgery. The histological diagnosis was tumoral calcinosis. Tumoral calcinosis is a rare tumoral calcium pyrophosphate dihydrate crystal deposition disease which presents as periarticular soft tissue calcification. Tumoral calcinosis should be considered in patients with a mass lesion involving the upper cervical spine and associated with metabolic abnormalities. Surgical excision is the treatment of choice, because this is completely curative without known recurrence.     

Progressive myelopathy due to idiopathic intraspinal tumoral calcinosis of the cervical spine. Case report

Tumoral calcinosis is a rare disorder that most often occurs in periarticular regions of the extremities. Here, the authors report on an extremely rare case of idiopathic intraspinal tumoral calcinosis of the cervical spine. This 54-year-old man presented with a 2-week history of progressive cervical myelopathy. Results of magnetic resonance imaging and computed tomography myelography of the cervical spine revealed an intraspinal calcified mass lesion posterior to the spinal cord at the C3-4 level, resulting in marked spinal cord compression. Spinal cord decompression and en bloc resection of the mass lesion were performed via a C-2 laminoplasty and C3-4 laminectomy. The mass was localized in the dura mater. Histologically, the lesion consisted of numerous nodules with amorphous calcified materials and a florid proliferation of multinucleated giant cells; that is, its histological characteristics were identical to those of tumoral calcinosis. The symptoms disappeared completely after surgery. In all previously reported cases of cervical tumoral calcinosis, the lesion was located in the paraspinal soft tissue, with bone and facet joint involvement. The present case is the first reported instance of cervical tumoral calcinosis localized only in the spinal canal.     

Tumoral calcinosis of the spine: a study of 21 cases

STUDY DESIGN: A retrospective study was conducted to investigate 21 patients found during spinal surgery to have paraspinal masses of dystrophic calcification (tumoral calcinosis). OBJECTIVES: To determine the magnetic resonance imaging characteristics of this disorder, and to document the associated spinal pathology. SUMMARY OF BACKGROUND DATA: Tumoral calcinosis usually is associated with hereditary disorders of calcium metabolism or renal dialysis. It also occurs in degenerated tissues in the absence of systemic disorders. Characteristically, calcific masses in the appendicular skeleton are visible on plain radiographs. Tumoral calcinosis has only rarely been reported in the spine. Documented patients have had an obvious calcific mass, and almost always the disorder has existed in other locations as well. Careful histologic study of specimens removed during spinal surgery suggests that tumoral calcinosis is common in the spine and usually is secondary to preexisting pathology. METHODS: This study involved 21 patients with lesions of tumoral calcinosis identified by histopathologic analysis of specimens removed during spinal surgery. The magnetic resonance images and the plain radiographs of the patients were reviewed and correlated with their clinical histories. RESULTS: In all the patients, the lesion of tumoral calcinosis was associated with a mass lesion seen on magnetic resonance imaging. Calcific masses were not apparent on plain films. In no case was the mass diagnosed before surgery as tumoral calcinosis. The magnetic resonance imaging changes were variously misinterpreted as neoplasms, infections, extruded disc material, or cysts. The observed features of tumoral calcinosis were those of an extradural mass showing a heterogeneous mixed-signal lesion that was identical on T1- and T2-weighted images. Characteristically, gadolinium did not enhance the lesions. CONCLUSIONS: Awareness of tumoral calcinosis of the spine may prevent unwarranted diagnoses of a more serious lesion in patients with characteristic magnetic resonance imaging changes. Also, this awareness may prevent pathologists from interpreting lesional tissue as nondiagnostic when other diagnoses are suspected clinically. This process may be a manifestation of degenerative spinal disease that has become so dominant that the underlying processes are obscured.     

La calcinose tumorale chez un patient hémodialysé chronique : intérêt de l’imagerie hybride tomographie d’émission monophotonique/tomodensitométrie

IntroductionTumoral calcinosis is a rare benign disease, defined by the presence of calcified deposits in periarticular tissues. It can be hereditary or secondary at chronic renal failure at the stage of dialysis. This work illustrates the contribution of single-photon emission computed tomography (SPECT/CT) in the diagnosis and management of tumoral calcinoses in a chronic hemodialysis patient, based on a clinical case.Case reportA 62-year-old patient, chronic hemodialysis since 24 years, presented a mechanical pain shoulders, knees and hips with limitation of joint mobility. The clinical exam found a mass of soft tissue in the buttocks. The radiological exam showed the presence of periarticular calcifications with no bone involvement. The SPECT/CT revealed a multifocal tumoral calcinosis affecting shoulders, elbows, wrists, hips and knees, associated with alveolar and abdominal calcinosis.Discussion and conclusionTumoral calcinosis is a distinct clinicopathological entity characterised by periarticular soft tissue calcium deposits. The SPECT/CT is important in the diagnosis, the assessment of extension and monitoring of tumoral calcinosis after treatment.     

Deposition of calcium pyrophosphate dihydrate crystals in the hand

A rare case with deposition of calcium pyrophosphate dihydrate crystals around the proximal portion of the first and second metacarpal bones is reported. The second metacarpal had a cystic lesion, and the cortex of the first metacarpal had irregular osteolytic change. There were degenerative changes in the first carpometacarpal joint, trapeziotrapezoid articulation, and second carpometacarpal joint. The patient had recurrent acute inflammatory attacks at the affected site. Initially the patient was thought to have tumoral calcinosis, or a calcifying soft-tissue tumor, with the possibility of a malignant tumor because of angiographic evidence of tumor stain and hypervascularity. Surgical biopsy with partial curettage of the calcified mass resulted in early recurrence of deposition of the crystals. Total excision would seem to be necessary to avoid recurrence.     

Tumoral calcinosis of the foot.

Tumoral calcinosis is characterized by periarticular deposition of calcium phosphate, usually in the setting of normocalcemia and hyperphosphatemia. The term tumoral calcinosis can be used to describe lesions with periarticular deposition of calcium phosphate resulting either from a primary disorder or a secondary disorder, such as renal failure with associated secondary hyperparathyroidism. Treatment entails phosphate deprivation, control of any primary disease processes such as secondary hyperparathyroidism, and resection for recalcitrant symptoms. We present a case of tumoral calcinosis involving the foot which required resection and ultimately hyperparathyroidectomy.     

Tumoral calcinosis of the shoulder

Tumoral calcinosis is a rare clinical and histopathologic syndrome that causes the formation of calcium salt crystal deposits in periarticular soft tissue. This soft tissue calcification typically behaves as a large-sized tumor. However, the mechanism is unknown for the massive periarticular calcification caused by tumoral calcinosis. In addition, tumor calcinosis occurs infrequently in patients who have been on long-term hemodialysis and have end-stage renal disease (ESRD). This report describes a 40-year-old male uremia patient on long-term hemodialysis. This patient developed a huge tumor mass and pain over the left shoulder and was diagnosed with tumoral calcinosis. Although common treatments include management of metabolic levels such as calcium and phosphate, in some cases, surgical intervention may be needed. In this patient case, complete surgical excision was done successfully with an excellent outcome at the 2-year follow-up.     

Foramen magnum syndrome secondary to calcium pyrophosphate crystal deposition in the transverse ligament of the atlas.

STUDY DESIGN: This report illustrates two rare cases of foramen magnum syndrome caused by a retro-odontoid mass in which calcium pyrophosphate dihydrate crystals were found. OBJECTIVES: To analyze the preoperative studies and the diagnostic criteria and to discuss the surgical treatment. The present cases will be compared with previous ones described in the literature. SUMMARY OF BACKGROUND DATA: Deposition of calcium pyrophosphate dihydrate crystals occurs into the fibrous and hyaline cartilage of the joints and intervertebral discs of the spine. Half of the patients known to have a chondrocalcinosis had asymptomatic calcification in the odontoid region. Ten patients were published in the literature as having a spinal cord syndrome secondary to calcium pyrophosphate dihydrate deposition in the odontoid region. METHODS: In both cases the preoperative studies were analyzed, and the retro-odontoid mass was resected and histologically examined. Both had an anterior transoral approach and have been followed for 1 year. RESULTS: According to the preoperative radiographs the diagnosis was suspected and confirmed histologically. Transoral approach was done with no need in either case for a posterior stabilization. CONCLUSIONS: Compression of the spinal cord by calcium pyrophosphate dihydrate deposition may occur. The preoperative diagnosis may be highly suspected after radiographic study and histologically confirmed. Transoral resection is the treatment of choice. Posterior stabilization should be considered only in cases of craniovertebral instability.     

Cervical radiculomyelopathy caused by deposition of calcium pyrophosphate dihydrate crystals in the ligamenta flava. Case report

A case of cervical radiculomyelopathy caused by multiple calcified nodules in the ligamenta flava is presented. Roentgenological examination of the cervical spine showed radiopaque nodular lesions, 7 x 7 x 5 mm in size, located in the paramedian portion of the posterior spinal canal. The nodules were removed surgically and they were confirmed to be calcifications of ligamenta flava. Microscopic examination of the nodules with the polarized light revealed extensive deposition of crystals. By x-ray diffraction study, the crystal was determined as calcium pyrophosphate dihydrate (CPPD: Ca2P2O7 . 2H2O). Although CPPD deposition in the cartilage has been known as pseudo-gout syndrome, deposition in the ligament has been reported only in a few cases. This is the first case with radiopaque calcified nodules in the ligamenta flava causing spinal cord compression, the composition of which proved to be CPPD.     

Tumoral calcinosis in infants: a report of three cases and review of the literature

Tumoral calcinosis is the deposition of calcium crystals and salts in the periarticular soft tissues. It commonly is seen as a complication of renal dialysis or in patients with a hereditary disposition. Occasionally, it is seen as an isolated condition with no known cause. Tumoral calcinosis usually affects adults and rarely, children. This disorder is extremely uncommon in infants. Only 13 cases have been reported. In this study three additional infants with tumoral calcinosis are reported, bringing the total to 16 cases. These 16 cases provide the opportunity to compare tumoral calcinosis in infants (younger than 18 months) with the disease in older patients. Although histologic and radiologic features in infants and adults are identical, some clinical features differ. In almost all infants, the disease develops without any predisposing factors, such as a family history. Surgical removal of lesions in infants is curative. By contrast, lesions in adults tend to recur after surgery.     

Symptomatic retro-odontoid pseudotumor causing calcium pyrophosphate dihydrate deposition combined with multilevel cervical spondylotic myelopathy

IntroductionSymptomatic retro-odontoid pseudotumor (ROP) caused by cervical compression and myelopathy is rare. Pathological diagnosis is recommended for differential diagnosis including the following: inflammatory disease, primary bone tumor, metastatic disease and calcium pyrophosphate dihydrate deposition (CPPD) also known as “crowned dens syndrome”. The authors report a rare case of ROP caused by CPPD deposition combined with multilevel cervical spondylotic myelopathy (MCSM) which was treated by tumor resectioning using a transoral approach combined with posterior decompression and fusion.Case presentationA 66-year-old male presented with progressive neck pain and spastic gait with no history of trauma. Radiographic imaging revealed degenerative change involving the atlanto-axial and atlanto-occipital joints with calcified enhancing soft tissue around the odontoid process causing cord compression and cervical instability at the C1-C2 level combined with MCSM and spinal cord compression at C3 to C7. Microscopic assisted transoral tumor resection combined with posterior decompression and fusion was performed at the occiput to T2. The pathology report describes a rhomboid-shaped crystal caused by calcium pyrophosphate dihydrate deposition (CPPD) disease. At the 6-month follow-up following the operation, the patient's neck pain and spastic gait were improved compared to the preoperative examination.DiscussionCervical compression and myelopathy from ROP causing CPPD combined with MCSM is rare. Pathology diagnosis and surgical management are highly recommended.ConclusionIn this case, a combined surgical approach: tumor resection using a transoral approach and a posterior approach for decompression and fusion at occiput to T2 was an effective option for this condition.     

Tumoral calcinosis of the lumbar meninges: case report

OBJECTIVE AND IMPORTANCE: Tumoral calcinosis is a rare disorder of unknown origin. Tumoral calcinosis involving the spine is extremely rare. This is the first case of tumoral calcinosis localized in the dura mater of the lumbar spine. CLINICAL PRESENTATION: This 55-year-old male patient presented with tumoral calcinosis of the lumbar meninges. T1-weighted, sagittal magnetic resonance imaging scans of the lumbar spine revealed a round mass of slightly increased intensity with high-intensity margins, located posterior to the cauda equina at the L4-L5 level. The cauda equina was severely compressed anteriorly. T2-weighted scans revealed that the mass was composed of a high-intensity area with low-intensity margins. T1-weighted magnetic resonance imaging scans obtained after intravenous gadolinium administration revealed some enhancement at the margins. INTERVENTION: The mass lesion was totally resected. Three years after surgery, no recurrence was observed in follow-up magnetic resonance imaging scans. CONCLUSION: Although tumoral calcinosis is a rare cause of mass lesions of the lumbar spine, it should be considered in differential diagnoses. If the lesion can be totally resected, the prognosis should be good, similar to that for general tumoral calcinosis.     

Tophaceous pseudogout of the thoracic spine

Calcium pyrophosphate dihydrate deposition disease (CPDD, tophaceous pseudogout) is a rare crystal arthropathy characterized by pyrophosphate crystal deposition in joints, synovitis and chondrocalcinosis on imaging. We present the case of a 72-year-old man with 6 months of left chest pain; magnetic resonance imaging revealed a T9/T10 herniated disc. Intraoperatively, the material was sent for pathological analysis revealing pseudogout. Axial calcium pyrophosphate crystal deposition is rare but reported in the literature and found at the craniocervical junction and skull. Spinal calcium pyrophosphate crystal deposition is rare in the thoracic spine. It is often asymptompatic and can involve the disc or ligaments. This case demonstrates a unique presentation of CPDD.     

Tumoral calcinosis: what is the treatment? Report of two cases of different types and review of the literature

Tumoral calcinosis (TC) is a rare problem that can be idiopathic (primary) or secondary to other diseases, particularly end-stage renal disease. Although these 2 types of TC do not differ in their radiologic and histopathologic presentations, their treatment modalities may be changed on the basis of etiology. Surgical resection of the calcified mass is the main treatment for the primary type, but should be avoided in hemodialysis-related types, which are instead often treated with parathyroidectomy. In this article, we report on 2 cases of TC (different types). We also describe several aspects of diagnosis and different therapeutic approaches through a review of the literature.     

Uremic tumoral calcinosis causing atlantoaxial subluxation and spinal cord compression in a patient on continuous ambulatory peritoneal dialysis

Uremic tumoral calcinosis (UTC) is a form of metastatic tissue calcification unique to dialysis patients, manifesting with amorphous and cystic masses containing calcium phosphate deposits in periarticular soft tissue. An involvement of the cervical spine with bone destruction is extremely rare in UTC. We describe a 44-year-old uremic female on long-term continuous ambulatory peritoneal dialysis who developed UTC in the peri-odontoid region with consequent atlantoaxial subluxation and spinal cord compression, featuring severe neck soreness, headache, and hypertension. Surgical removal of the destructive cervical spine lesion, showing typical tumoral calcinosis on histology, completely resolved the clinical symptoms. To date, the patient maintains uneventful postoperative course with tight control of serum phosphorus, calcium, and secondary hyperparathyroidism by medical treatment. We also review other reported unusual cases of UTC involving the cervical spine and discuss the differential diagnosis of destructive spinal lesions in uremic patients, such as UTC, dialysis-related amyloidosis, and brown tumors.     

Spinal cord compression due to tumoral idiopatic calcinosis

STUDY DESIGN: A case of thoracic spinal cord compression due to tumoral calcinosis (TC) is reported. SETTING: Galiza, Spain. CASE REPORT: A 59-year-old man was admitted to our hospital with a 2-month history of gradual leg weakness and sensory deficit. The neurological examination revealed paraparesis with T12 sensory level. Magnetic resonance imaging (MRI) showed an extradural right posterolateral mass at T11-T12 level, resulting in a marked spinal cord compression. He underwent T11-T12 laminectomy and mass excision. Histological examination finally led to the diagnosis of TC. CONCLUSION: TC is an uncommon cause of mass lesions of the spine. Since there is no typical spine TC MRI appearance, the final diagnosis is done by histological studies. TC should be considered in differential diagnosis of spinal cord compression and constitutes a treatable cause of paraparesis.     

Calcium pyrophosphate dihydrate deposition disease causing thoracic cord compression: case report

OBJECTIVE AND IMPORTANCE: Calcium pyrophosphate dihydrate (CPPD) deposition disease is being increasingly recognized. Spinal involvement in CPPD deposition disease is rare. When involved, the cervical and lumbar regions are commonly affected. We report a rare case of CPPD deposition disease that caused thoracic cord compression. CLINICAL PRESENTATION: A 45-year-old woman presented with clinical features suggestive of thoracic cord compression. Radiographic findings were consistent with calcification of the ligamenta flava in the lower thoracic levels causing cord compression. Calcification of the ligamentum flavum is commonly attributed to CPPD deposition disease. Evaluation for conditions that might be associated with CPPD deposition disease proved to be negative. INTERVENTION: Laminectomy with removal of the calcified ligamenta flava was performed. Histopathological examination of the excised ligaments revealed evidence of CPPD crystals. Postoperatively, the patient's spasticity decreased and sensations improved, with no significant improvement in motor power. CONCLUSION: Calcification of the ligamenta flava due to CPPD deposition disease is a rare cause of thoracic cord compression. CPPD deposition disease should be entertained in the differential diagnosis of thoracic cord compression.     

Thoracic myelopathy caused by calcified ligamentum flavum

Calcification of the ligamentum flavum is a rare manifestation of the calcium pyrophosphate dihydrate deposition disease (CPPD). In CPPD deposition disease, spinal involvement is rare. Until now, thoracic spine CPPD causing thoracic cord compression has been reported in only sporadic cases. We report a new case of thoracic calcification of the ligamentum flavum. In our case, similar to the other reported cases, an affected middle-aged woman despite the clinical and MRI signs of myelopathy had an unexpected important and rapid improvement of the neurological picture. This condition should be considered in differential diagnosis of thoracic cord compression to offer the patient an early and useful surgical treatment.     

Calcium pyrophosphate dihydrate crystal deposition disease in the cervical ligamentum flavum

The authors describe three cases of cervical radiculomyelopathy caused by calcium pyrophosphate dihydrate crystal deposition disease (CPPDcdd). Radiological investigations revealed nodular calcifications, 5 to 7 mm in diameter, in the cervical ligamentum flavum compressing the spinal cord. Light microscopic, scanning electron microscopic, and x-ray diffraction studies were performed on all three surgical specimens obtained by laminectomy. In two of the cases x-ray microanalysis and transmission electron microscope studies were also performed. This study defined the presence of two patterns of crystal deposition in the ligamentum flavum. One is a nodular deposit, in which hydroxyapatite crystals are seen in the central part of the nodules, with calcium pyrophosphate dihydrate (CPPD) being distributed thinly around them. The other pattern is a linear deposit seen in multiple ligaments and composed of pure CPPD, which causes minimal thickening of the ligaments. A transitional pattern between the two types was also observed. This study revealed details of the nodular deposition of crystals in the ligamentum flavum and demonstrates that CPPDcdd and so-called "calcification of the ligamentum flavum" are the same disease: namely, CPPDcdd. Hydroxyapatite is assumed to have been transformed from CPPD.     

Foraminal deposition of calcium pyrophosphate dihydrate crystals in the thoracic spine: possible relationship with disc herniation and implications for surgical planning. Report of two cases

The authors report two cases of nodular calcium pyrophosphate dihydrate (CPPD) crystal deposition close to the thoracic neural foramen, which caused chronic radiculopathy. Preoperatively, the lesions were interpreted as calcified disc herniations. Both patients underwent surgery in which an extended transfacet pedicle-sparing approach was used. Incision of the posterior longitudinal ligament released soft degenerated material. In both cases, histological examination showed abundant degenerative debris along with CPPD crystals. Spinal CPPD deposition is a comparatively rare disease that almost invariably involves the posterior aspect of the spinal canal, typically the ligamentum flavum. The exceptional foraminal location of the lesions reported here, combined with the surgical findings, indicated that the CPPD crystals were deposited on a laterally herniated disc fragment. A distinctive feature in both cases was the soft consistency of the resected tissue. The consistency of the disc material and the location of the lesion in the axial plane (that is, median compared with lateral) are key factors in determining the optimal surgical approach to thoracic disc herniations. In describing consistency, terms such as "calcified" and "hard" have been used interchangeably in the literature. In the cases reported here, what appeared on computerized tomography and magnetic resonance imaging studies to be densely calcified lesions were shown intraoperatively to be soft herniations. The authors' experience underscores that not all densely calcified herniated discs are hard. Although detection of this discrepancy would have left surgical planning for the lateral disc herniations unchanged, it could have altered planning for centrally or centrolaterally located disc herniations.