10. Pathology and Oncology MCQs

DISCUSSION: The findings are most consistent with a rupture of the anterior tibial tendon. The damaged area of tendon should be resected, followed by tendon reconstruction or tenodesis. The histology is not consistent with giant cell tumor of the tendon sheath, gout, or synovial sarcoma. Fibromatosis is characterized by a large number of spindle cells within the collagen background. REFERENCES: Otte S, Klinger HM, Loreaz F, Haerer T: Operative treatment in case of closed rupture of the anterior tibial tendon. Arch Orthop Traum Surg 2002;122:188-190. Kausch T, Rutt J: Subcutaneous rupture of the tibialis anterior tendon: Review of the literature and case report. Arch Orthop Traum Surg 1998;117:290-293.

This radiolucent lesion with a “fallen leaf sign” is typical for a unicameral bone cyst (UBC). The most appropriate treatment is to allow the fracture to heal with clinical and radiographic observation. Curettage and bone grafting is not the best initial management for UBC. Wide resection is not indicated for UBC. The proximal humerus is the most common site for UBC. While staging studies consisting of MRI, bone scan, and CT of the chest are appropriate for lesions suspected of being malignant, the classical appearance of this UBC is such that this work-up is not necessary initially. Following fracture healing, aspiration and injection of the cyst may be indicated.

The radiographs reveal a geographic lesion of the proximal femur with the classic “ground glass” appearance noted in fibrous dysplasia. This intramedullary lesion is modestly expansile, demonstrates some minimal cortical thinning, and has no aggressive features. Chondroblastoma, giant cell tumor, and osteoblastoma are more lytic in appearance, and the location is not typical for giant cell tumor or chondroblastoma. While enchondroma may be considered, the uniform ground glass appearance, lack of punctuate mineralization, and distinct margination of the lesion make that diagnosis less likely.

The classic Ponseti technique is associated with a low recurrence rate when followed precisely. The most common reason for recurrence is noncompliance with postsurgical bracing. The initial treatment for recurrence after Ponseti casting is recasting.

Resection of a previously excised soft-tissue sarcoma is recommended. Reoperation is recommended after removal of malignant tumors previously believed to be benign, as approximately one half of the patients will have residual tumor in the re-excised specimen. Observation is not indicated in most patients because local recurrence is likely. Radiation therapy alone may result in long-term local control but is felt to be less effective than reoperation. Chemotherapy and radiation therapy alone are not recommended. Wide excision is the most important factor for local disease control. Radiation therapy after surgical re-excision may also decrease the risk of local recurrence.

The most common destructive lesions in a patient older than age 40 years are, in decreasing incidence, metastatic carcinoma, multiple myeloma, lymphoma, and chondrosarcoma. Osteosarcoma is found primarily in younger patients.

After plain radiographs of the affected area have been obtained, magnetic resonance imaging modality is the best imaging modality for detecting and characterizing the lesion, regarding definition of normal muscle, fascial boundaries, and the tumor mass. Although MR imaging is not specific in determining whether lesions are benign or malignant, it can be useful in evaluationother characteristics, such as size, pattern of growth, integrity of natural boundaries, and homogeneity.Intravenous contrast agents are not necessary to evaluate neurovascular structures. Both the T1 & T2 weighted images are essential to detect and characterize soft tissue lesions.Most Tumors have long T1 and T2 relaxation times, therefore, in most instances signal intensity alone is of limited benefit. Exceptions are lipoma, hematoma, intra-lesional hemorrhage.Hemorrhage may occur in some soft tissue lesions, especially sarcomas, leading to the low T1 and high T2 intensity sound on MR. Miller, indicates that Water, CSF, acute hemorrhage and soft tissue tumors appear dark on T1 and light on T2.

This patient has a lytic lesion filling the proximal humerus with a history of renal cell carcinoma. In a patient with a history of localized cancer with a bone lesion, staging with CT scan of the chest, abdomen, and pelvis is indicated to define the extent of disease. For a patient with a solitary lesion and no history of metastatic disease, biopsy is indicated to exclude a new primary tumor before surgical treatment is undertaken. Patients with solitary lesions have longer survival, but the likelihood of true cure is low even with en bloc resection. There is no known prognostic significance of extraosseous extension or gene amplification in renal carcinoma.

DISCUSSION: Fibromatosis is a benign but aggressive fibrous lesion that principally arises from the connective tissue of muscle and the overlying fascia. The peak incidence is between the ages of 25 and 35 years. Most patients have a deep-seated, firm, poorly circumscribed mass that has grown insidiously and causes little or no pain. MRI is helpful in diagnosing the lesion and in assessing the extent of disease prior to surgical intervention. Histologically, the lesion is poorly circumscribed and infiltrates the surrounding tissue. The lesion appears bland with uniform spindle cells separated by abundant collagen, with little or no cell-to-cell contact. Despite its bland microscopic appearance, the tumor frequently behaves in an aggressive manner. These lesions do not metastasize but have a high incidence of recurrence. Treatment options consist of surgical resection, radiation therapy, chemotherapeutic protocols, hormone modulation, and/or anti-inflammatory medications.

DISCUSSION: The history, examination findings, and studies are consistent with an osteoid osteoma. The child has had a 2-year history of pain that even wakes her from sleep, so observation and anti-inflammatory drugs is not a preferred treatment. Bracing will not help with the discomfort because the pain is not mechanical in nature. The osteoid osteoma is close to the spinal cord so radiofrequency ablation is not preferred. Surgical removal and biopsy is the treatment of choice.

DISCUSSION: The findings are typical for an osteochondroma. It is found as an outgrowth of bone and cartilage from those bones that arise from enchondral ossification. It may be flat, verrucous, or with a long stalk and cauliflower-like cap. Osteochondromas can become symptomatic secondary to irritation of the adjacent musculature. They cease to proliferate when epiphyseal growth ceases. REFERENCE: Schmade GA, Conrad EV III, Raskind WH: The natural history of hereditary multiple exostoses. J Bone Joint Surg Am 1994;76:986-992.

Giant cell tumors are common around the knee, distal radius, sacrum and hands. They are not typically found in the diaphysis of bone. Giant cell tumors are one of the more common tumors in young adults 22-40 years of age. There is much argument as to whether giant cell tumors arise in the metaphysis or the epiphysis, but most authors agree that giant cell tumors are very uncommon in the diaphysis. The most common sites of giant cell tumors in bone are the distal radius, sacrum, distal femur, proximal tibia and proximal humerus.

DISCUSSION: A chordoma is a malignant neoplasm originating from remnants of the notochord. It is usually localized to the midline with 50% at the sacrococcygeal area, 35% at the skull base, and 15% at the mobile portion of the spine. Large vacuolated cells (physaliferous cells) are a characteristic of the tumor. REFERENCES: Mindell ER: Chordoma. J Bone Joint Surg Am 1981;63:501-505. Samson IR, Springfield DS, Suit HD, Mankin HJ: Operative treatment of sacrococcygeal chordoma: A review of twenty-one cases. J Bone Joint Surg Am 1993;75:1476-1484.

DISCUSSION: The radiograph and pathology are consistent with adamantinoma. While the mechanism underlying adamantinoma has not been identified, it is believed to be closely related to osteofibrous dysplasia, which may represent a precursor. The other diagnoses are not known to give rise to adamantinoma. REFERENCE: Springfield DS, Rosenberg AE, Mankin HJ, et al: Relationship between osteofibrous dysplasia and adamantinoma. Clin Orthop 1994;309:234-244.

DISCUSSION: Synovial sarcoma should always be considered in the differential diagnosis of a foot mass; however, the histopathology shows a typical example of PVNS, with hemosiderin, giant cells, and synovium. Synovial chondromatosis would have metaplastic cartilage in the synovium. The radiograph shows subtle erosion of the lateral cortex of the cuboid, and the MRI scan shows a soft-tissue mass. REFERENCES: Ghert MA, Scully SP, Harrelson JM: Pigmented villonodular synovitis of the foot and ankle: A review of six cases. Foot Ankle Int 1999;20:326-330. Jones BC, Sundaram M, Kransdorf MJ: Synovial sarcoma: MR imaging findings in 34 patients. Am J Roentgenol 1993;161:827-830. Sartoris DJ, Resnick D: Magnetic resonance imaging of pediatric foot and ankle disorders. J Foot Surg 1990;29:489-494. Scully SP, Temple HT, Harrelson JM: Synovial sarcoma of the foot and ankle. Clin Orthop 1999;364:220-226.

DISCUSSION: Radiation-associated sarcomas typically occur at least 5 years following radiation therapy, in the radiation therapy field, and with different histology than the original disease. The radiograph shows a lytic destructive lesion of the medial clavicle. The radiographic differential could include any of the above etiologies. The CT and MRI scans show this same reaction with extension into the adjacent soft tissue and periosteal reaction. These findings eliminate a degenerative process or radiation-induced osteonecrosis but do not distinguish between a neoplastic and infectious process. A PET scan showed marked uptake in the distal clavicle, which is more consistent with a malignant neoplastic process than a reactive process, like that of an infection. These findings, combined with the cellular atypia and bone formation on the biopsy specimen, confirm the diagnosis of radiation-associated sarcoma. In this older patient, radiation-associated sarcoma appears in an unusual location secondary to her previous radiation treatment in that region, which can occur 3 to 50 years after previous radiation therapy. REFERENCES: Shaheen M, Deheshi BM, Riad S, et al: Prognosis of radiation-induced bone sarcoma is similar to primary osteosarcoma. Clin Orthop Relat Res 2006;450:76-81. Tabone MD, Terrier P, Pacquement H, et al: Outcome of radiation-related osteosarcoma after treatment of childhood and adolescent cancer: A study of 23 cases. J Clin Oncol 1999;17:2789-2795.

DISCUSSION: The patient has a presumed metastatic bone tumor. The approach to evaluating a patient with a bone tumor of unknown primary origin is to obtain laboratory studies that include a CBC, an erythrocyte sedimentation rate, a serum protein electrophoresis, a calcium level, a urinalysis, and a prostate-specific antigen. In addition, a bone scan, a radiograph of the chest, and CT scans of the chest and abdomen should be obtained. These evaluations can identify the primary site in 85% of patients. GI studies rarely are of diagnostic value. Prophylactic stabilization is contraindicated until a diagnosis is confirmed by histology. REFERENCES: Frassica FJ, Frassica DA, McCarthy EF, Riley LH III: Metastatic bone disease: Evaluation, clinicopathologic features, biopsy, fracture risk, nonsurgical treatment, and supportive management. Instr Course Lect 2000;49:453-459. Rougraff BT, Kneisl JS, Simon MA: Skeletal metastases of unknown origin: A prospective study of a diagnostic strategy. J Bone Joint Surg Am 1993;75:1276-1281.

DISCUSSION: In 1940, Batson described a valveless plexus of veins that extend from the dural venous sinuses of the skull to the sacrum. This system permits retrograde blood flow and enables tumor cells to enter vertebral bodies at multiple levels. Increased intra-abdominal pressure will enhance this retrograde blood flow. REFERENCE: Batson OV: The function of the vertebral veins and their role in the spread of metastases. Ann Surg 1940;112:138-149.

The peak incidence of osteosarcoma occurs in the second decade, followed by the third decade. Up to 75% of all cases of osteosarcoma occur in patients between 10 and 25 years. It rarely occurs after age 30. Affected women tend to be younger than affected men. Osteosarcoma associated with Paget’s disease or radiation-induced osteosarcoma occurs in an older population.