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Chondroma

Chondroma (Morphologic Abnormality)

Chondroma is a small, benign tumor of hyaline cartilage, and can arise either within the bone medullary cavity or on the bone surface. It most commonly develops in the short bones of hands and feet. Chondroma has an asymptomatic course in the majority of patients, but pain and pathologic fractures may occur. In most cases, it is diagnosed incidentally. Treatment includes surgical excision and carries a good prognosis.


Presentation

The majority of chondromas are discovered as incidental findings, because of their indolent and asymptomatic nature. In some cases, symptoms such as local pain and swelling may be present, while pathologic fractures may be observed as a result of local skeletal damage by the tumor. Most commonly, tumors are diagnosed in the hands and feet, while the femur, humerus, clavicle, ribs, the pelvic region, and spine are sites where this tumor also could be diagnosed. In patients with Maffucci syndrome other abnormalities should also be considered, since it is associated with increased risk of developing other tumors as well, including ovarian and brain tumors.

Galactorrhea
  • A 40-year-old Japanese woman with amenorrhea and galactorrhea for the last 2 years was referred to our department of neurosurgery for the evaluation of high prolactin levels and a tumor in the sella turcica.[ncbi.nlm.nih.gov]
Finger Pain
  • When enchondroma involves short tubular bones of the hands, it can result in bulbous swelling of the fingers. Pain is often not present. A complaint of pain should raise the suspicion of microfracture or a malignant transformation.[boneandspine.com]
Hunting
  • In this case presentation we describe a 10 year old boy who had an MRI for facial paralysis due to Ramsey Hunt Syndrome, which incidentally revealed a parapharyngeal mass.[ncbi.nlm.nih.gov]
Swelling of the Finger
  • When enchondroma involves short tubular bones of the hands, it can result in bulbous swelling of the fingers. Pain is often not present. A complaint of pain should raise the suspicion of microfracture or a malignant transformation.[boneandspine.com]
Exertional Dyspnea
  • Full Text We report the case of a 65-year-old man with 2-month history of exertional dyspnea. Chest CT showed a mass in the left upper bronchus.[archbronconeumol.org]
Malocclusion
  • We report a case of chondroma of the mandibular condyle that presented as temporomandibular pain with malocclusion, and which relapsed 5 years after the initial treatment.[ncbi.nlm.nih.gov]
Nail Deformity
  • We report a subungual extraskeletal chondroma with a convex nail deformity originating from the index finger in a 39-year-old man. The tumor was excised and its histopathology showed obvious nuclear pleomorphism.[ncbi.nlm.nih.gov]
  • Ishii T, Ikeda M, Oka Y: Subungual extraskeletal chondroma with finger nail deformity: case report. J Hand Surg Am 2010; 35: 296–299. Cartilaginous and osseous soft tissue tumors; in Goldblum JR, Folpe AL, Weiss SW (eds): Soft Tissue Tumors, ed 6.[karger.com]
Subcutaneous Nodule
  • A 54-year-old woman presented with a 2-year history of a slow-growing subcutaneous nodule of the medial right lower eyelid adjacent to the inferior canaliculus. The patient reported right-sided epiphora suggesting lacrimal outflow obstruction.[ncbi.nlm.nih.gov]
Subcutaneous Mass
  • We present an unusual case of periosteal chondroma of the middle phalanx in the right ring finger that presented as a subcutaneous mass in a 21-year-old man. It was treated by marginal excision, with no postoperative local recurrence.[ncbi.nlm.nih.gov]
Skin Ulcer
  • There was no skin ulceration. No history of pain, systemic disease, drug administration, local trauma, irradiation, or chronic inflammation was recorded. Clinical diagnosis of multiple sebaceous cysts was made.[ijpmonline.org]
Anterior Knee Pain
  • A 16-year-old boy presented with a patellar mass and anterior knee pain without any trauma.[ncbi.nlm.nih.gov]
Neck Mass
  • After relevant examinations, subtotal thyroidectomy and excisional biopsy of the neck mass were performed under general anesthesia.[ncbi.nlm.nih.gov]
Radiculopathy
  • We report a patient who presented with left lumbar radiculopathy, and a peripherally-enhancing dumbbell shaped soft tissue mass involving both the intraspinal and extraspinal compartments on MRI.[ncbi.nlm.nih.gov]
  • Keywords: Dural-based chondroma, lumbar mass, radiculopathy, spinal chondroma How to cite this article: Pace J, Lozen AM, Wang MC, Cochran EJ. Extradural chondroma presenting as lumbar mass with compressive neuropathy .[jcvjs.com]
  • Cetinkal A, et al. (2008) Lumbar spinal chondroma presenting with radiculopathy case report. Turk Neurosurg 18: 397-399. 3. Kim DH, et al. (2013) Lumbar spinal chondroma presenting with acute sciatica. Korean J Spine 10: 252-254. 4.[scifedpublishers.com]
Tingling
  • The patient had a mild tingling sensation in the dorsum of the first web space associated with mild difficulty in upward stretching of his left big toe. However, there was no history of trauma.[ncbi.nlm.nih.gov]
Tremor
  • A 1-year post-operative MRI showed no residual tumor or recurrence, but cure of the essential tremor was not achieved. Discussion Intracranial chondromas are most common on the skull base.[scielo.br]
Urinary Retention
  • CT scans showed an extensive tumour filling the entire minor pelvic cavity and proximal femur with impression and infiltration of the femoral blood vessels, and displacement of both ureters, colon and urinary bladder causing urinary retention.[ncbi.nlm.nih.gov]

Workup

The diagnosis of chondroma is usually made incidentally. Several imaging techniques could be used. Plain X-rays may reveal ring-form calcified areas within the bone that not change over time. Any changes in shape, size, and lucency of the surrounding bone, same as decreased calcifications, should raise suspicion of the presence of a malignant tumor, primarily chondrosarcoma [10] [11]. Periosteal chondromas may show erosion of the cortex, punctate calcifications, and subchondral sclerosis [12].

Other imaging techniques, including CT and MRI, can be performed if X-ray findings are inconclusive. CT scan can show subtle calcification changes, which is important for differentiation of benign and malignant tumors. MRI is the imaging technique of choice for intramedullary chondromas. These tumors show low signal intensity on T1-weighed images, but a high intensity on T2-weighed images.

A definite diagnosis can be obtained by biopsy, but it is not indicated in all patients. Asymptomatic chondromas are usually observed and followed up with imaging techniques, but in patients who experience pain, the biopsy is indicated. In patients with severe pain and pathologic fractures in whom surgery is indicated promptly, the biopsy may be performed during surgical excision.

Brain Edema
  • Imaging usually showed a well-demarcated extramedullary tumor, centrally located, without surrounding brain edema, partially calcified (73.3%) and with minimal vascularity, often accompanied by erosion and destruction of surrounding bone (56.7%).[ncbi.nlm.nih.gov]

Treatment

Because chondromas have an asymptomatic and benign course in most cases, treatment may be deferred, and only observation of the tumor may be indicated. If patients do not experience pain, nor have any issues with a function of the affected part of the body, surgical treatment is not necessary. However, in patients who complain of pain, and those who develop fractures because of the present tumor, surgical excision is the main form of treatment.

Simple curettage without postcurettage void augmentation is proven to be safe and effective [13]. This procedure is readily performed for chondromas that develop in the hands, but if the tumor develops in the spine, scapula, or in the pelvic region, local resection is indicated, which also shows good results. Bone grafting or cement injections as augmentation therapy are indicated for sizable tumors which eroded larger parts of the bone [14]. In the case of synovial chondroma, synovectomy of the affected joint may be indicated to reduce the burden of the tumor.

Recurrence rates after surgery are minimal, while full recovery was observed within months after treatment [15]. These findings indicate that patients that are surgically treated have little or no sequelae after removal of the tumor. However, if recurrences do occur, malignant transformation of the tumor should always be suspected.

Prognosis

The prognosis for all types of chondromas are good, they have very low rates of recurrence after surgical excision, and their malignant potential is minimal. Pathologic fractures can be fully recovered, and minimal of no loss in function is observed in the majority of cases within few months after removal. However, patients with multiple chondromas, such as those with Maffucci syndrome, have an increased risk of malignant transformation of tumors, as well as complications after surgical treatment, which is why appropriate therapy must be initiated early on.

Etiology

It is still not known why chondromas develop, but several genetic anomalies have been detected. Supernumerary ring chromosomes derived from chromosome 12 have been established in soft tissue chondromas [3], as well as an aberration of chromosomes 6 and 11 [4]. Additionally, other genetic changes have been detected, including 12q13, and their implications are under research [5]. These facts point to a genetic background for the development of this tumor, while other factors, such as environmental or infectious, have not been determined.

Epidemiology

Chondroma is the most common tumor of the hand, and it is established that enchondromas comprise almost 50% of all hand tumors [6]. Enchondromas are much more common than periosteal and synovial chondromas. However, because of the asymptomatic course and common diagnosis as an incidental finding, the actual rates of this tumor are not fully known. Gender predilection has not been established, as this tumor is evenly distributed in men and women. Also, no ethnic predilection has been found.

This tumor is most commonly diagnosed in patients between 20 and 50 years of age, but chondromas have been reported both in children and elderly, which implies that this tumor may be diagnosed at any age.

Sex distribution
Age distribution

Pathophysiology

Chondromas that develop within the medullary cavity originate from actively proliferating cartilaginous tissue of growth plates. Certain groups of chondrocytes, for unknown reasons, don't undergo apoptosis, change the original location and are the source of enchondroma formation [7].

Periosteal chondromas are slowly growing tumors, and develop adjacent to the bone cortex, and within the periosteal connective tissue [8]. As a result of constant pressure by the tumor to the bone, cortical erosion and periosteal reaction occur, which can produce intense pain and damage to the bony structures [9].

Genetic abnormalities have been increasingly proposed as a key event in the pathogenesis of this tumor.

Prevention

Prevention of chondromas is not currently possible, but regular follow-ups, as well as proper treatment when indicated, provide good outcomes for patients. However, if multiple chondromas are diagnosed, or if the tumor recurs after surgery, a malignant transformation should be suspected and investigated.

Summary

Chondroma is a benign tumor of hyaline cartilage, with two distinct forms: enchondroma (when the tumor arises within the bone medullary cavity) and juxtacortical or periosteal chondroma (arising on the bone surface) [1]. This tumor may also arise from tendon synovial sheaths (synovial chondroma), but it is a rare occurrence. In most cases, chondromas are small (up to 3 cm), solitary lesions, but multiple enchondromas may appear in Ollier disease. In Maffucci syndrome, multiple enchondromas are associated with soft tissue angiomas. Chondroma is not an aggressive tumor, and rarely progresses into a malignant form, but patients with Ollier disease and Maffucci syndrome are at increased risk for this event. The cause and pathogenesis of this tumor are not known, and it is most commonly diagnosed in middle-aged adults between ages 20-50. However, patients of any age may develop this benign tumor, while gender and ethnic predilection have not been established. This tumor has a good prognosis, as it poses little or no malignant potential except when multiple chondromas are present. It does not have aggressive properties, and with successful surgical treatment, recurrences are very rare.

Because chondroma is often an incidental finding and has an asymptomatic course in most patients, the exact prevalence rates are unknown. It is established that chondroma most often develops in the short tubular bones of the hands and feet, while it may also appear in the proximal humerus and other long bones. When symptoms are present, they include local pain, swelling, and development of pathologic fractures. The diagnosis is made by imaging techniques, and X-ray may reveal typical "ring calcifications" that occur as a result of endochondral ossification and calcification of the matrix [2]. CT and MRI are superior to X-ray and may aid in establishing the diagnosis if X-ray findings are inconclusive. A biopsy could be performed to confirm the diagnosis, and microscopic findings include the presence of hyaline cartilage and scattered chondrocytes in the lacunae without mitotic activity.

For smaller tumors that are discovered incidentally and are asymptomatic, simple observation and follow-up are sufficient, as such tumors do not pose any threat to the patient. But if manifestations such as pain and development of fractures occur, surgical excision through curettage is recommended. In the majority of patients, full recovery is achieved, with little or no restriction of motion, and recurrences of the tumor are very rare.

Patient Information

Chondroma is a benign tumor that occurs either on the surface or inside the bone, or it may rarely develop in the joint. This tumor is often diagnosed incidentally, meaning that it rarely causes symptoms, and is detected during check-ups for other reasons. Chondroma is usually solitary, and most commonly arises in the short bones of the hands and feet, but may appear in any bone of the body. It does not possess malignant potential, except in rare cases where multiple chondromas develop simultaneously when there is an increased risk for a transformation of this benign tumor into malignant. The exact reason why this tumor develops is not known, but faults in the chromosomal structures have been observed and imply a genetic background in the development of this tumor. Chondroma equally develops across genders and is most commonly diagnosed in adults between 20-50 years, but patients of various ages have been reported. Chondroma is asymptomatic in the majority of patients, but local pain, swelling, and the development of pathologic fractures (in an absence of trauma) are reported symptoms. The diagnosis of this tumor is made by imaging studies, such as X-rays, CT scan or MRI, while a definite diagnosis can be made by performing a biopsy. This tumor has a good prognosis, and asymptomatic patients do not require treatment but are instead regularly being followed-up by imaging studies. However, patients who experience pain and develop fractures should be treated by surgery. Surgical excision of the tumor shows very good results, and recurrence rates are very low.

References

Article

  1. Kumar V, Abbas K, Fausto N, Mitchell N. Robbins Basic Pathology 9th edition. Philadelphia: Saunders Elsevier; 2012.
  2. Folpe AL, Inwards CY. Foundations in Diagnostic Pathology. Saunders Elsevier; 2010.
  3. Shadan FF, Mascarello JT, Newbury RO, et al. Supernumerary ring chromosomes derived from the long arm of chromosome 12 as the primary cytogenetic anomaly in a rare soft tissue chondroma. Cancer Genet Cytogenet. 2000;118(2):144-147.
  4. Dal Cin P, Qi H, Sciot R, et al. Involvement of chromosomes 6 and 11 in a soft tissue chondroma. Cancer Genet Cytogenet. 1997;93(2):177-178.
  5. Tallini G, Dorfman H, Brys P, et al. Correlation between clinicopathological features and karyotype in 100 cartilaginous and chordoid tumours. A report from the Chromosomes and Morphology (CHAMP) Collaborative Study Group. J Pathol. 2002;196(2):194-203.
  6. Simon MJ, Pogoda P, Hövelborn F, et al. Incidence, histopathologic analysis and distribution of tumours of the hand. BMC Musculoskelet Disord. 2014;15:182.
  7. Milgram JW. The origins of osteochondromas and enchondromas. A histopathologic study. Clin Orthop Relat Res. 1983;(174):264-284.
  8. Al-Qudah AS, Abu-Ali HM, Al-Hussaini MA, Massad IM. Periosteal chondroma of the clavicle: case report and review of the literature. Int J Surg. 2009;7(2):140-141.
  9. Lewis MM, Kenan S, Yabut SM, et al. Periosteal chondroma. A report of ten cases and review of the literature. Clin Orthop Relat Res. 1990;256:185-192.
  10. Geirnaerdt MJ, Hermans J, Bloem JL, et al. Usefulness of radiography in differentiating enchondroma from central grade 1 chondrosarcoma. AJR Am J Roentgenol. 1997;169(4):1097-1104.
  11. Nojima T, Unni KK, McLeod RA, et al. Periosteal chondroma and periosteal chondrosarcoma. Am J Surg Pathol. 1985;9(9):666-677.
  12. Bauer TW, Dorfman HD, Latham JT Jr. Periosteal chondroma. A clinicopathologic study of 23 cases. Am J Surg Pathol. 1982;6(7):631-637.
  13. Bachoura A, Rice IS, Lubahn AR, et al. The surgical management of hand enchondroma without postcurettage void augmentation: authors' experience and a systematic review. Hand (N Y). 2015;10(3):461-471.
  14. Tang C, Chan M, Fok M, Fung B. Current Management of Hand Enchodroma: A Review. Hand Surgery. 2015;20(1):191–195.
  15. Sassoon AA, Fitz-Gibbon PD, Harmsen WS, et al. Enchondromas of the hand: factors affecting recurrence, healing, motion, and malignant transformation. J Hand Surg Am. 2012;37(6):1229-1234.

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Last updated: 2019-07-11 22:41