527509
research-article2014
CPJXXX10.1177/0009922814527509Clinical PediatricsChauhan et al
Resident Rounds
Was Adam Really a Man or Was Eve Missing a 12th Rib
Clinical Pediatrics 2014, Vol. 53(5) 501–503 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922814527509 cpj.sagepub.com
Aman Chauhan, MD1, Perry Dubin2, Vincent Adolph, MD3, and Raj Warrier, MD3 Patient Report A 15-year-old Caucasian female was evaluated by the primary care physician for hypertension. Chest X-ray showed an absence of the 12th rib on the left side (Figure 1). A computed tomography scan performed at an outside facility reported a cystic lesion in her spleen, a lytic lesion of her 12th rib, and a possible para vertebral mass (Figure 2). She had no specific complaints or subjective symptoms and denied headache, fever, flushing, night sweats, palpitation, cough, diarrhea loss of appetite, or weight loss. Review of systems was otherwise negative. Differential diagnosis to explain the hypertension and the mass included paraganglioma or extra-adrenal pheomochromocytoma with hypertension, osteogenic/Ewing’s sarcoma, or a functional neuroendocrine tumor. She was transferred to the pediatric oncology service. Her past medical history and review of systems were noncontributory. Family history was significant for father in his 30s with hypertension, hypercholesterolemia, and diabetes. Her paternal grandfather had pancreatic cancer at 52 years of age. Her mother and 3 siblings had no significant medical history. Social history showed an active role in sports and good school performance. Vital signs on exam were as follows: temperature 97.5°F, pulse 78 beats/min, blood pressure 145/78 mm Hg, weight 69 kg, and height 158 cm. Physical exam showed a mildly obese, alert, and active girl in no acute distress. Systemic exam was within normal limits. Pertinent laboratory values included normal erythrocyte sedimentation rate 6 (normal = 0-20 mm/h), alkaline phosphatase 136 (normal = 74-390 U/L), C-reactive proteiv 400 (normal = 68-110), low high-density lipoprotein 34 (normal = 40-75 mg/dL), and a borderline high serum cholesterol 177 (normal = 120-199 mg/dL). She had an elevated thyroid-stimulating hormone 6.48 (normal = 0.4-5.0 uIU/mL), but normal Free T4 0.84 (normal = 0.71-1.51 ng/dL). Review of the computed tomography scan showed an expansile lytic lesion of the left 12th rib. Magnetic
Figure 1. Plain chest X-ray showing what looks an absent 12th rib but a possible mass on more careful exam.
resonance imaging at our facility confirmed an expansile rib lesion with signal characteristic of a telangiectatic osteosarcoma, atypical aneurysmal bone cyst, or an osteoblastoma. The bone lesion showed mass effect on the surrounding soft tissues including the diaphragm and paraspinous musculature.
Diagnosis Aneurysmal bone cyst. Pediatric surgery was consulted and the mass was totally excised with resection of the left 12th rib. The pathology report on the lesion 1
Louisiana State University Health Sciences Center, New Orleans, LA, USA 2 Tulane University, New Orleans, LA, USA 3 Ochsner Clinic, New Orleans, LA, USA Corresponding Author: Aman Chauhan, Louisiana State University Health Sciences Center, 1542 Tulane Ave, Suite 441, New Orleans, LA 70112, USA. Email: [email protected]
Downloaded from cpj.sagepub.com at UNIV OF CO HEALTH SCIENCE CTR on December 13, 2014
502
Clinical Pediatrics 53(5)
Figure 2. Computed tomography scan showing expansile rib lesion in various planes.
demonstrated multiple varying sized vascular spaces and fibrous proliferative stroma without hypercellularity, indicating an aneurysmal bone cyst.
Discussion Aneurysmal bone cysts (ABCs) are uncommon benign tumors of bone known to grow rapidly.1 First described in the literature by Jaffe and Lichtenstein in 1942,2 their incidence is greatest in the first 2 decades of life and have a greater preponderance for the female sex.1,3 The ABC is of great significance to the pediatrician because of its young age of onset. It commonly occurs before the age of 20 years, found by Mankin et al in a study of 150 patients to have a mean age of onset of 18 years with a standard deviation of 12 years.3 The most common sites of occurrence are the long bones of the “femur, tibia, humerus, spine and pelvis.”3 Usually, the tumors do not cross the epiphyses and remain in the metaphysis and diaphysis.3 Case reports of crossing an open or originating in an epiphyses have been documented.4 ABCs can also be found in the small bones of the hands and feet, vertebrae, ribs, and maxilla.1,3 The patient here presented with a 12th rib ABC, which in a study by Mankin et al was found in only 2 of the 150 case reports.3 The greatest incidence in this study was found in the tibia and femur at 37/150 and 26/150, respectively.3 In a similar study by Vergel De
Dios et al, 8/238 cases were localized to the ribs, and the greatest concentration of ABCs in this study was also localized to the long bones of the legs.5 The clinical presentation of an ABC most often involves pain, swelling, or both localized to the site of the lesion. Pathologic fracture is another possible presenting symptom. The location of the ABC can also lead to corresponding symptoms, such as a progressive paralysis if located in one of the corresponding vertebral bodies, or proptosis if located around the orbit.1 The location of the ABC also holds the potential for angular or growth plate deformity. Cases, much like the one reported here, are also commonly found incidentally on radiographs taken for other reasons. Diagnosis of an ABC requires the use of radiographic techniques. The classic presentation for an ABC is “as a fusiform expansile ‘blow-out’ lesion located eccentrically relative to the long axis of the bone.”1 There are 4 phases of radiographic presentation corresponding to the ABC’s natural history: Initial phase—small, lytic lesion without evidence of lifting off of the periosteum; phase 2, rapidly enlarging demonstrating a characteristic blowout appearance; phase 3, growth slows or stops after some sort of intervention; and phase 4, healing with progressive ossification and calcification of the cavity.1
Definitive diagnosis is established through the use of open biopsy, often performed at the time of surgery for
Downloaded from cpj.sagepub.com at UNIV OF CO HEALTH SCIENCE CTR on December 13, 2014
503
Chauhan et al resection of the ABC. Fine needle aspiration is often misleading due to the potential inadequacy of the sample, sampling errors, and risk of bleeding.1,4 Histologically, the sample shows cavernous slits that may or may not contain blood.1 Atypical or malignant cells may indicate a telangiectatic osteosarcoma.4 Treatment always involves some form of surgical excision. “Curettage and bone grafting and/or cementation” to replace the defect are often employed. If the ABC is deemed aggressive or recurrent, wide excision and radiotherapy are further techniques used to control the lesion.1 Resection is generally adequate, but it is not uncommon to see recurrence. Mankin et al saw a 30% recurrence in their study and showed that anatomic site of the ABC is the greatest risk factor for recurrence.1,3 The patient’s hypertension was evaluated by Nephrology and diagnosed as early onset primary hypertension, and she was started on appropriate therapy. She has no evidence of recurrence of the ABC 2 years after the resection. Although uncommon, aneurysmal bone cyst should be considered in a pediatric patient with an osteolytic lesion and may be found incidentally without associated systemic signs.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Mendenhall WM, Zlotecki RA, Gibbs CP, Reith JD, Scarborough MT, Mendenhall NP. Aneurismal bone cyst. Am J Clin Oncol. 2006;29:311-315. 2. Jaffe HL, Lichtenstein L. Solitary unicameral bone cyst with emphasis on the roentgen picture, the pathologic appearance and the pathogenesis. Arch Surg. 1942;44:1004-1025. 3. Mankin HJ, Hornicek FJ, Ortiz-Cruz E, Villafuerte J, Gebhardt MC. Aneurismal bone cyst: a review of 150 patients. J Clin Oncol. 2005;23:6756-6762. 4. Subramaniam CS, Mathias PF. Aneurysmal bone cyst. J Bone Joint Surg Br. 1962;44:93-101. 5. Vergel De Dios AM, Bond JR, Shives TC, McLeod RA, Unni KK. Aneurysmal bone cyst. A clinicopathologic study of 238 cases. Cancer. 1992;69:2921-2931.
Downloaded from cpj.sagepub.com at UNIV OF CO HEALTH SCIENCE CTR on December 13, 2014
research-article2014
CPJXXX10.1177/0009922814527509Clinical PediatricsChauhan et al
Resident Rounds
Was Adam Really a Man or Was Eve Missing a 12th Rib
Clinical Pediatrics 2014, Vol. 53(5) 501–503 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922814527509 cpj.sagepub.com
Aman Chauhan, MD1, Perry Dubin2, Vincent Adolph, MD3, and Raj Warrier, MD3 Patient Report A 15-year-old Caucasian female was evaluated by the primary care physician for hypertension. Chest X-ray showed an absence of the 12th rib on the left side (Figure 1). A computed tomography scan performed at an outside facility reported a cystic lesion in her spleen, a lytic lesion of her 12th rib, and a possible para vertebral mass (Figure 2). She had no specific complaints or subjective symptoms and denied headache, fever, flushing, night sweats, palpitation, cough, diarrhea loss of appetite, or weight loss. Review of systems was otherwise negative. Differential diagnosis to explain the hypertension and the mass included paraganglioma or extra-adrenal pheomochromocytoma with hypertension, osteogenic/Ewing’s sarcoma, or a functional neuroendocrine tumor. She was transferred to the pediatric oncology service. Her past medical history and review of systems were noncontributory. Family history was significant for father in his 30s with hypertension, hypercholesterolemia, and diabetes. Her paternal grandfather had pancreatic cancer at 52 years of age. Her mother and 3 siblings had no significant medical history. Social history showed an active role in sports and good school performance. Vital signs on exam were as follows: temperature 97.5°F, pulse 78 beats/min, blood pressure 145/78 mm Hg, weight 69 kg, and height 158 cm. Physical exam showed a mildly obese, alert, and active girl in no acute distress. Systemic exam was within normal limits. Pertinent laboratory values included normal erythrocyte sedimentation rate 6 (normal = 0-20 mm/h), alkaline phosphatase 136 (normal = 74-390 U/L), C-reactive proteiv 400 (normal = 68-110), low high-density lipoprotein 34 (normal = 40-75 mg/dL), and a borderline high serum cholesterol 177 (normal = 120-199 mg/dL). She had an elevated thyroid-stimulating hormone 6.48 (normal = 0.4-5.0 uIU/mL), but normal Free T4 0.84 (normal = 0.71-1.51 ng/dL). Review of the computed tomography scan showed an expansile lytic lesion of the left 12th rib. Magnetic
Figure 1. Plain chest X-ray showing what looks an absent 12th rib but a possible mass on more careful exam.
resonance imaging at our facility confirmed an expansile rib lesion with signal characteristic of a telangiectatic osteosarcoma, atypical aneurysmal bone cyst, or an osteoblastoma. The bone lesion showed mass effect on the surrounding soft tissues including the diaphragm and paraspinous musculature.
Diagnosis Aneurysmal bone cyst. Pediatric surgery was consulted and the mass was totally excised with resection of the left 12th rib. The pathology report on the lesion 1
Louisiana State University Health Sciences Center, New Orleans, LA, USA 2 Tulane University, New Orleans, LA, USA 3 Ochsner Clinic, New Orleans, LA, USA Corresponding Author: Aman Chauhan, Louisiana State University Health Sciences Center, 1542 Tulane Ave, Suite 441, New Orleans, LA 70112, USA. Email: [email protected]
Downloaded from cpj.sagepub.com at UNIV OF CO HEALTH SCIENCE CTR on December 13, 2014
502
Clinical Pediatrics 53(5)
Figure 2. Computed tomography scan showing expansile rib lesion in various planes.
demonstrated multiple varying sized vascular spaces and fibrous proliferative stroma without hypercellularity, indicating an aneurysmal bone cyst.
Discussion Aneurysmal bone cysts (ABCs) are uncommon benign tumors of bone known to grow rapidly.1 First described in the literature by Jaffe and Lichtenstein in 1942,2 their incidence is greatest in the first 2 decades of life and have a greater preponderance for the female sex.1,3 The ABC is of great significance to the pediatrician because of its young age of onset. It commonly occurs before the age of 20 years, found by Mankin et al in a study of 150 patients to have a mean age of onset of 18 years with a standard deviation of 12 years.3 The most common sites of occurrence are the long bones of the “femur, tibia, humerus, spine and pelvis.”3 Usually, the tumors do not cross the epiphyses and remain in the metaphysis and diaphysis.3 Case reports of crossing an open or originating in an epiphyses have been documented.4 ABCs can also be found in the small bones of the hands and feet, vertebrae, ribs, and maxilla.1,3 The patient here presented with a 12th rib ABC, which in a study by Mankin et al was found in only 2 of the 150 case reports.3 The greatest incidence in this study was found in the tibia and femur at 37/150 and 26/150, respectively.3 In a similar study by Vergel De
Dios et al, 8/238 cases were localized to the ribs, and the greatest concentration of ABCs in this study was also localized to the long bones of the legs.5 The clinical presentation of an ABC most often involves pain, swelling, or both localized to the site of the lesion. Pathologic fracture is another possible presenting symptom. The location of the ABC can also lead to corresponding symptoms, such as a progressive paralysis if located in one of the corresponding vertebral bodies, or proptosis if located around the orbit.1 The location of the ABC also holds the potential for angular or growth plate deformity. Cases, much like the one reported here, are also commonly found incidentally on radiographs taken for other reasons. Diagnosis of an ABC requires the use of radiographic techniques. The classic presentation for an ABC is “as a fusiform expansile ‘blow-out’ lesion located eccentrically relative to the long axis of the bone.”1 There are 4 phases of radiographic presentation corresponding to the ABC’s natural history: Initial phase—small, lytic lesion without evidence of lifting off of the periosteum; phase 2, rapidly enlarging demonstrating a characteristic blowout appearance; phase 3, growth slows or stops after some sort of intervention; and phase 4, healing with progressive ossification and calcification of the cavity.1
Definitive diagnosis is established through the use of open biopsy, often performed at the time of surgery for
Downloaded from cpj.sagepub.com at UNIV OF CO HEALTH SCIENCE CTR on December 13, 2014
503
Chauhan et al resection of the ABC. Fine needle aspiration is often misleading due to the potential inadequacy of the sample, sampling errors, and risk of bleeding.1,4 Histologically, the sample shows cavernous slits that may or may not contain blood.1 Atypical or malignant cells may indicate a telangiectatic osteosarcoma.4 Treatment always involves some form of surgical excision. “Curettage and bone grafting and/or cementation” to replace the defect are often employed. If the ABC is deemed aggressive or recurrent, wide excision and radiotherapy are further techniques used to control the lesion.1 Resection is generally adequate, but it is not uncommon to see recurrence. Mankin et al saw a 30% recurrence in their study and showed that anatomic site of the ABC is the greatest risk factor for recurrence.1,3 The patient’s hypertension was evaluated by Nephrology and diagnosed as early onset primary hypertension, and she was started on appropriate therapy. She has no evidence of recurrence of the ABC 2 years after the resection. Although uncommon, aneurysmal bone cyst should be considered in a pediatric patient with an osteolytic lesion and may be found incidentally without associated systemic signs.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Mendenhall WM, Zlotecki RA, Gibbs CP, Reith JD, Scarborough MT, Mendenhall NP. Aneurismal bone cyst. Am J Clin Oncol. 2006;29:311-315. 2. Jaffe HL, Lichtenstein L. Solitary unicameral bone cyst with emphasis on the roentgen picture, the pathologic appearance and the pathogenesis. Arch Surg. 1942;44:1004-1025. 3. Mankin HJ, Hornicek FJ, Ortiz-Cruz E, Villafuerte J, Gebhardt MC. Aneurismal bone cyst: a review of 150 patients. J Clin Oncol. 2005;23:6756-6762. 4. Subramaniam CS, Mathias PF. Aneurysmal bone cyst. J Bone Joint Surg Br. 1962;44:93-101. 5. Vergel De Dios AM, Bond JR, Shives TC, McLeod RA, Unni KK. Aneurysmal bone cyst. A clinicopathologic study of 238 cases. Cancer. 1992;69:2921-2931.
Downloaded from cpj.sagepub.com at UNIV OF CO HEALTH SCIENCE CTR on December 13, 2014
