Malignant breast tumors
Clinical presentation and diagnostic evaluation
Types of pleuropulmonary blastoma
Clinical presentation and diagnostic evaluation
Incidence and histology
Clinical presentation and diagnostic evaluation
Thymoma and Thymic Carcinoma
Incidence and risk factors
Clinical presentation and diagnostic evaluation
Incidence and risk factors
Thoracic cancers include breast cancer, bronchial adenomas, bronchial carcinoid tumors, pleuropulmonary blastoma, esophageal tumors, thymomas, thymic carcinomas, cardiac tumors, and mesothelioma. The prognosis, diagnosis, classification, and treatment of these thoracic cancers are discussed below. It must be emphasized that these cancers are seen very infrequently in patients younger than 15 years, and most of the evidence is derived from case series.Breast Cancer
The most frequent breast tumor seen in children is a fibroadenoma.[2,3] These tumors can be observed and many will regress without a need for biopsy. However, rare malignant transformation leading to phyllodes tumors has been reported. Sudden rapid enlargement of a suspected fibroadenoma is an indication for needle biopsy or excision. Phyllodes tumors can be managed by wide local excision without mastectomy.Malignant breast tumors
Incidence, epidemiology, and treatment
Breast cancer has been reported in both males and females younger than 21 years.[5-10] A review of the Surveillance, Epidemiology, and End Results (SEER) database shows that 75 cases of malignant breast tumors in females 19 years or younger were identified from 1973 to 2004. Fifteen percent of these patients had in situ disease, 85% had invasive disease, 55% of the tumors were carcinomas, and 45% of the tumors were sarcomas—most of which were phyllodes tumors. Only three patients in the carcinoma group presented with metastatic disease, while 11 patients (27%) had regionally advanced disease. All patients with sarcomas presented with localized disease. Of the carcinoma patients, 85% underwent surgical resection, and 10% received adjuvant radiation therapy. Of the sarcoma patients, 97% had surgical resection, and 9% received radiation. The 5- and 10-year survival rates for patients with sarcomatous tumors were both 90%; for patients with carcinomas, the 5-year survival rate was 63% and the 10-year survival rate was 54%.Treatment of adolescents and young adults
Breast cancer is the most frequently diagnosed cancer among adolescent and young adult (AYA) women aged 15 to 39 years, accounting for about 14% of all AYA cancer diagnoses. Breast cancer in this age group has a more aggressive course and worse outcome than in older women. Expression of hormone receptors for estrogen, progesterone, and human epidermal growth factor 2 (HER2) on breast cancer in the AYA group is also different than in older women and correlates with a worse prognosis. Treatment in the AYA group is similar to that in older women. However, unique aspects of management must include attention to genetic implications (i.e., familial breast cancer syndromes) and fertility.Female survivors of Hodgkin lymphoma
There is an increased lifetime risk of breast cancer in female survivors of Hodgkin lymphoma who were treated with radiation to the chest area; however, breast cancer is also seen in patients who were treated for any cancer that was treated with chest irradiation.[9,15-18] Carcinomas are more frequent than sarcomas. Mammograms with adjunctive breast magnetic resonance imaging (MRI) should start at age 25 years or 10 years postexposure to radiation therapy (whichever came last). (Refer to the PDQ summary on the Late Effects of Treatment for Childhood Cancer for more information about secondary breast cancers.) Breast tumors may also occur as metastatic deposits from leukemia, rhabdomyosarcoma, other sarcomas, or lymphoma (particularly in patients who are infected with the human immunodeficiency virus).
(Refer to the PDQ summary on adult Breast Cancer Treatment for more information.)Lung Cancer
Primary lung tumors are rare in children and histologically quite diverse. When epithelial cancers of the lung occur, they tend to be of advanced stage with prognosis dependent on both histology and stage.
The majority of pulmonary malignant neoplasms in children are due to metastatic disease, with an approximate ratio of primary malignant tumors to metastatic disease of 1:5. While primary pulmonary tumors are rare in children, the majority of these tumors are malignant. In a review of 383 primary pulmonary neoplasms in children, 76% were malignant and 24% were benign. These tumors may respond to the ALK inhibitor crizotinib in the presence of ALK translocations.[Level of evidence: 3iiiDiv]
The most common malignant primary tumors of the lung, bronchial tumors and pleuropulmonary blastoma, are discussed below.Bronchial Tumors
Bronchial tumors are a heterogeneous group of primary endobronchial lesions, and although adenoma implies a benign process, all varieties of bronchial tumors on occasion display a malignant behavior. There are three histologic types:[23-28]
- Carcinoid tumor (most frequent). Carcinoid tumors account for 80% to 85% of all bronchial tumors in children.[23-27]
- Mucoepidermoid carcinoma.
- Adenoid cystic carcinoma (least frequent).
The presenting symptoms of a cough, recurrent pneumonitis, and hemoptysis are usually due to an incomplete bronchial obstruction. Because of difficulties in diagnosis, symptoms are frequently present for months, and, occasionally, children with wheezing have been treated for asthma with delays in diagnosis as long as 4 to 5 years.
Metastatic lesions are reported in approximately 6% of carcinoid tumors, and recurrences are reported in 2% of cases. Atypical carcinoid tumors are rare but more aggressive with 50% of patients presenting with metastatic disease at diagnosis.[19,32] There is a single report of a child with a carcinoid tumor and metastatic disease who developed the classic carcinoid syndrome. Octreotide nuclear scans may demonstrate uptake of radioactivity by the tumor or lymph nodes, suggesting metastatic spread.Treatment
The management of bronchial tumors is somewhat controversial because bronchial tumors are usually visible endoscopically. Biopsy of these lesions may be hazardous because of hemorrhage, and endoscopic resection is not recommended. Bronchography or computed tomography scan may be helpful to determine the degree of bronchiectasis distal to the obstruction since the degree of pulmonary destruction may influence surgical therapy.
Conservative pulmonary resection, including sleeve segmental resection, when feasible, with the removal of the involved lymphatics, is the treatment of choice.[35,36] Adenoid cystic carcinomas (cylindroma) have a tendency to spread submucosally, and late local recurrence or dissemination has been reported. In addition to en bloc resection with hilar lymphadenectomy, a frozen section examination of the bronchial margins should be performed in children with this lesion. Neither chemotherapy nor radiation therapy is indicated for bronchial tumors, unless evidence of metastasis is documented.Pleuropulmonary Blastoma
Types of pleuropulmonary blastoma
Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy in children. Pleuropulmonary blastoma appears to progress through the following stages:
- Type I: A purely lung cystic neoplasm with subtle malignant changes that typically occurs in the first 2 years of life and has a good prognosis. However, there have been reports of Type I transitioning directly to Type III.[37,38]
- Type II: A cystic and solid neoplasm. Cerebral metastasis may occur in 11% of patients.
- Type III: A purely solid neoplasm.[40,41] Cerebral metastasis occurs in up to 50% of patients with Type III tumors.
Approximately one-third of families affected by pleuropulmonary blastoma manifest a number of dysplastic and/or neoplastic conditions comprising the Pleuropulmonary Blastoma Family Tumor and Dysplasia Syndrome. Germline mutations in the DICER1 gene are considered the major genetic determinant of the complex.[42,43] Importantly, while DICER1 mutations cause a wide range of phenotypes, pleuropulmonary blastoma does not occur in all families with DICER1 mutations; therefore, the term DICER1 syndrome is generally used for these families. Also, most mutation carriers are unaffected, indicating that tumor risk is modest.
There has been a reported association between pleuropulmonary blastoma and cystic nephroma, ciliary body medulloepithelioma of the eye, and primary ovarian neoplasms, particularly ovarian sex cord–stromal tumors.[43,47-50]Clinical presentation and diagnostic evaluation
The tumor is usually located in the lung periphery, but it may be extrapulmonary with involvement of the heart/great vessels, mediastinum, diaphragm, and/or pleura.[51,52] The International Pleuropulmonary Blastoma Registry identified 11 cases of Type II and Type III pleuropulmonary blastoma with tumor extension into the thoracic great vessels or the heart. Radiographic evaluation of the central circulation should be performed in children with suspected or diagnosed pleuropulmonary blastoma to identify potentially fatal embolic complications.Treatment
Achieving total resection of the tumor at any time during treatment is associated with improved prognosis. The tumors may recur or metastasize, in spite of primary resection.[38,41] The cerebral parenchyma is the most common metastatic site.
Responses to chemotherapy have been reported with agents similar to those used for the treatment of rhabdomyosarcoma, and adjuvant chemotherapy may benefit patients with Type I pleuropulmonary blastoma by reducing the risk of recurrence.[40,54] Chemotherapeutic agents may include vincristine, cyclophosphamide, dactinomycin, doxorubicin, and irinotecan.[55,56] Data from the International Pleuropulmonary Blastoma Registry suggest that adjuvant chemotherapy may reduce the risk of recurrence.
Radiation, either external beam or P-32, may be used when the tumor cannot be surgically removed.
High-dose chemotherapy with stem cell rescue has been used without success.
There are no standard treatment options. Current treatment regimens have been informed by consensus conferences. The rare occurrence of these tumors makes recommending treatment difficult. Some general treatment considerations from the Pleuropulmonary Blastoma Registry include the following:
- Type I: Surgery alone for select cases; adjuvant chemotherapy may decrease recurrences.[40,58] Evidence suggests a close histologic relationship between a Type 4 cystic adenomatoid malformation and a Type I pleuropulmonary blastoma.[59,60] Complete surgical lobectomy is adequate treatment for these patients, but close observation is recommended.
- Type II and Type III: Surgery followed by chemotherapy.
An independent group of researchers has established a registry and resource Web site for this rare tumor.Esophageal Tumors
Incidence and histology
Esophageal cancer is rare in the pediatric age group, although it is relatively common in older adults.[61,62] Most of these tumors are squamous cell carcinomas, although sarcomas can also arise in the esophagus. The most common benign tumor is leiomyoma.Clinical presentation and diagnostic evaluation
Symptoms are related to difficulty in swallowing and associated weight loss. Diagnosis is made by histologic examination of biopsy tissue.Treatment
Treatment options for esophageal carcinoma include either external-beam intracavitary radiation therapy or chemotherapy agents commonly used to treat carcinomas: platinum derivatives, paclitaxel, and etoposide. Prognosis is generally poor for this cancer, which rarely can be completely resected.
(Refer to the PDQ summary on adult Esophageal Cancer Treatment for more information.)Thymoma and Thymic Carcinoma
A cancer of the thymus is not considered a thymoma or a thymic carcinoma unless there are neoplastic changes of the epithelial cells that cover the organ.[63-65] The term thymoma is customarily used to describe neoplasms that show no overt atypia of the epithelial component. Thymic carcinomas have a higher incidence of capsular invasion and metastases. A thymic epithelial tumor that exhibits clear-cut cytologic atypia and histologic features no longer specific to the thymus is known as thymic carcinoma, also known as type C thymoma. Other tumors that involve the thymus gland include lymphomas, germ cell tumors, carcinomas, carcinoids, and thymomas. Hodgkin lymphoma and non-Hodgkin lymphoma may also involve the thymus and must be differentiated from true thymomas and thymic carcinomas.Incidence and risk factors
Various diseases and syndromes are associated with thymoma, including myasthenia gravis, polymyositis, systemic lupus erythematosus, rheumatoid arthritis, thyroiditis, Isaacs syndrome or neuromyotonia (continuous muscle stiffness resulting from persistent muscle activity as a consequence of antibodies against voltage-gated potassium channels), and pure red-cell aplasia.[70,71] Endocrine (hormonal) disorders including hyperthyroidism, Addison disease, and panhypopituitarism can also be associated with a diagnosis of thymoma.Clinical presentation
These neoplasms are usually located in the anterior mediastinum and are usually discovered during a routine chest x-ray. Symptoms can include cough, difficulty with swallowing, tightness of the chest, chest pain, and shortness of breath, although nonspecific symptoms may occur. These tumors generally are slow growing but are potentially invasive, with metastases to distant organs or lymph nodes. Staging is related to invasiveness.Treatment
Surgery is performed with the goal of a complete resection and is the mainstay of therapy.
Radiation therapy is used in patients with invasive thymoma or thymic carcinoma.
Chemotherapy is usually reserved for patients with advanced-stage disease who have not responded to radiation therapy or corticosteroids. Agents that have been effective include doxorubicin, cyclophosphamide, etoposide, cisplatin, ifosfamide, and vincristine.[65,69,72-75] Responses to regimens containing combinations of some of these agents have ranged from 26% to 100% and survival rates have been as high as 50%.[75,76] Response rates are lower for patients with thymic carcinoma, but 2-year survival rates have been reported to be as high as 50%.
Sunitinib has yielded clinical responses in four patients with adult thymic carcinoma.Cardiac Tumors
The most common primary tumors of the heart are benign. In adults, myxoma is the most common tumor; however, these tumors are rare in children. The most common primary benign heart tumors in children are rhabdomyomas, myxomas, teratomas, and fibromas.[80-82] Other benign tumors include histiocytoid cardiomyopathy tumors, hemangiomas, and neurofibromas (i.e., tumors of the nerves that innervate the muscles).[80,83-86]
Myxomas are the most common noncutaneous finding in Carney complex, a rare syndrome characterized by lentigines, cardiac myxomas or other myxoid fibromas, and endocrine abnormalities.[87-89] A mutation of the PRKAR1A gene is noted in more than 90% of the cases of Carney complex.[87,90]
Secondary tumors of the heart include metastatic spread of rhabdomyosarcoma, melanoma, leukemia, and carcinoma of other sites.Risk factors
The distribution of cardiac tumors in the fetal and neonatal period is different, with more benign teratomas occurring. Multiple cardiac tumors noted in the fetal or neonatal period are highly associated with a diagnosis of tuberous sclerosis. A retrospective review of 94 patients with cardiac tumors detected by prenatal or neonatal echocardiography showed that 68% of the patients exhibited features of tuberous sclerosis. In another study, 79% (15 out of 19) of patients with rhabdomyomas discovered prenatally had tuberous sclerosis, while 96% of those diagnosed postnatally had tuberous sclerosis. Most rhabdomyomas, whether diagnosed prenatally or postnatally, will spontaneously regress.Clinical presentation and diagnostic evaluation
Patients may be asymptomatic for long periods. Symptoms may include abnormalities of heart rhythm, enlargement of the heart, fluid in the pericardial sac, and congestive heart failure. Some patients present with sudden death.
The utilization of new cardiac MRI techniques can identify the likely tumor type in the majority of children. However, histologic diagnosis remains the standard for diagnosing cardiac tumors.Treatment Mesothelioma
Incidence and risk factors
This tumor can involve the membranous coverings of the lung, the heart, or the abdominal organs.[100-102] These tumors can spread over the surface of organs, without invading far into the underlying tissue, and may spread to regional or distant lymph nodes. Mesothelioma may develop after successful treatment of an earlier cancer, especially after treatment with radiation.[103,104] In adults, these tumors have been associated with exposure to asbestos, which was used as building insulation. The amount of exposure required to develop cancer is unknown, and there is no information about the risk for children exposed to asbestos.Prognosis
Benign and malignant mesotheliomas cannot be differentiated using histologic criteria. A poor prognosis is associated with lesions that are diffuse and invasive and with those that recur. In general, the course of the disease is slow, and long-term survival is common.Diagnostic evaluation
Diagnostic thoracoscopy should be considered in suspicious cases to confirm diagnosis.Treatment
Radical surgical resection has been attempted with mixed results. Treatment with various chemotherapeutic agents used for carcinomas or sarcomas may result in partial responses.[102,107] Pain is an infrequent symptom; however, radiation therapy may be used for palliation of pain.
Papillary serous carcinoma of the peritoneum is sometimes mistaken for mesothelioma. This tumor generally involves all surfaces lining the abdominal organs, including the surfaces of the ovary. Treatment includes surgical resection whenever possible and use of chemotherapy with agents such as cisplatin, carboplatin, and paclitaxel.
(Refer to the PDQ summary on adult Malignant Mesothelioma Treatment for more information.)References
- Yu DC, Grabowski MJ, Kozakewich HP, et al.: Primary lung tumors in children and adolescents: a 90-year experience. J Pediatr Surg 45 (6): 1090-5, 2010. [PUBMED Abstract]
- Chung EM, Cube R, Hall GJ, et al.: From the archives of the AFIP: breast masses in children and adolescents: radiologic-pathologic correlation. Radiographics 29 (3): 907-31, 2009 May-Jun. [PUBMED Abstract]
- Jayasinghe Y, Simmons PS: Fibroadenomas in adolescence. Curr Opin Obstet Gynecol 21 (5): 402-6, 2009. [PUBMED Abstract]
- Valdes EK, Boolbol SK, Cohen JM, et al.: Malignant transformation of a breast fibroadenoma to cystosarcoma phyllodes: case report and review of the literature. Am Surg 71 (4): 348-53, 2005. [PUBMED Abstract]
- Serour F, Gilad A, Kopolovic J, et al.: Secretory breast cancer in childhood and adolescence: report of a case and review of the literature. Med Pediatr Oncol 20 (4): 341-4, 1992. [PUBMED Abstract]
- Drukker BH: Breast disease: a primer on diagnosis and management. Int J Fertil Womens Med 42 (5): 278-87, 1997 Sep-Oct. [PUBMED Abstract]
- Rogers DA, Lobe TE, Rao BN, et al.: Breast malignancy in children. J Pediatr Surg 29 (1): 48-51, 1994. [PUBMED Abstract]
- Rivera-Hueto F, Hevia-Vázquez A, Utrilla-Alcolea JC, et al.: Long-term prognosis of teenagers with breast cancer. Int J Surg Pathol 10 (4): 273-9, 2002. [PUBMED Abstract]
- Kaste SC, Hudson MM, Jones DJ, et al.: Breast masses in women treated for childhood cancer: incidence and screening guidelines. Cancer 82 (4): 784-92, 1998. [PUBMED Abstract]
- Costa NM, Rodrigues H, Pereira H, et al.: Secretory breast carcinoma--case report and review of the medical literature. Breast 13 (4): 353-5, 2004. [PUBMED Abstract]
- Gutierrez JC, Housri N, Koniaris LG, et al.: Malignant breast cancer in children: a review of 75 patients. J Surg Res 147 (2): 182-8, 2008. [PUBMED Abstract]
- Keegan TH, DeRouen MC, Press DJ, et al.: Occurrence of breast cancer subtypes in adolescent and young adult women. Breast Cancer Res 14 (2): R55, 2012. [PUBMED Abstract]
- Anders CK, Hsu DS, Broadwater G, et al.: Young age at diagnosis correlates with worse prognosis and defines a subset of breast cancers with shared patterns of gene expression. J Clin Oncol 26 (20): 3324-30, 2008. [PUBMED Abstract]
- Gabriel CA, Domchek SM: Breast cancer in young women. Breast Cancer Res 12 (5): 212, 2010. [PUBMED Abstract]
- Metayer C, Lynch CF, Clarke EA, et al.: Second cancers among long-term survivors of Hodgkin's disease diagnosed in childhood and adolescence. J Clin Oncol 18 (12): 2435-43, 2000. [PUBMED Abstract]
- Swerdlow AJ, Barber JA, Hudson GV, et al.: Risk of second malignancy after Hodgkin's disease in a collaborative British cohort: the relation to age at treatment. J Clin Oncol 18 (3): 498-509, 2000. [PUBMED Abstract]
- van Leeuwen FE, Klokman WJ, Veer MB, et al.: Long-term risk of second malignancy in survivors of Hodgkin's disease treated during adolescence or young adulthood. J Clin Oncol 18 (3): 487-97, 2000. [PUBMED Abstract]
- Henderson TO, Amsterdam A, Bhatia S, et al.: Systematic review: surveillance for breast cancer in women treated with chest radiation for childhood, adolescent, or young adult cancer. Ann Intern Med 152 (7): 444-55; W144-54, 2010. [PUBMED Abstract]
- Lal DR, Clark I, Shalkow J, et al.: Primary epithelial lung malignancies in the pediatric population. Pediatr Blood Cancer 45 (5): 683-6, 2005. [PUBMED Abstract]
- Weldon CB, Shamberger RC: Pediatric pulmonary tumors: primary and metastatic. Semin Pediatr Surg 17 (1): 17-29, 2008. [PUBMED Abstract]
- Hancock BJ, Di Lorenzo M, Youssef S, et al.: Childhood primary pulmonary neoplasms. J Pediatr Surg 28 (9): 1133-6, 1993. [PUBMED Abstract]
- Kim SJ, Kim DW, Kim TM, et al.: Remarkable tumor response to crizotinib in a 14-year-old girl with ALK-positive non-small-cell lung cancer. J Clin Oncol 30 (16): e147-50, 2012. [PUBMED Abstract]
- Vadasz P, Palffy G, Egervary M, et al.: Diagnosis and treatment of bronchial carcinoid tumors: clinical and pathological review of 120 operated patients. Eur J Cardiothorac Surg 7 (1): 8-11, 1993. [PUBMED Abstract]
- Kulke MH, Mayer RJ: Carcinoid tumors. N Engl J Med 340 (11): 858-68, 1999. [PUBMED Abstract]
- Oliaro A, Filosso PL, Donati G, et al.: Atypical bronchial carcinoids. Review of 46 patients. J Cardiovasc Surg (Torino) 41 (1): 131-5, 2000. [PUBMED Abstract]
- Moraes TJ, Langer JC, Forte V, et al.: Pediatric pulmonary carcinoid: a case report and review of the literature. Pediatr Pulmonol 35 (4): 318-22, 2003. [PUBMED Abstract]
- Al-Qahtani AR, Di Lorenzo M, Yazbeck S: Endobronchial tumors in children: Institutional experience and literature review. J Pediatr Surg 38 (5): 733-6, 2003. [PUBMED Abstract]
- Roby BB, Drehner D, Sidman JD: Pediatric tracheal and endobronchial tumors: an institutional experience. Arch Otolaryngol Head Neck Surg 137 (9): 925-9, 2011. [PUBMED Abstract]
- Soga J, Yakuwa Y: Bronchopulmonary carcinoids: An analysis of 1,875 reported cases with special reference to a comparison between typical carcinoids and atypical varieties. Ann Thorac Cardiovasc Surg 5 (4): 211-9, 1999. [PUBMED Abstract]
- Fauroux B, Aynie V, Larroquet M, et al.: Carcinoid and mucoepidermoid bronchial tumours in children. Eur J Pediatr 164 (12): 748-52, 2005. [PUBMED Abstract]
- Abuzetun JY, Hazin R, Suker M, et al.: Primary squamous cell carcinoma of the lung with bony metastasis in a 13-year-old boy: case report and review of literature. J Pediatr Hematol Oncol 30 (8): 635-7, 2008. [PUBMED Abstract]
- Rizzardi G, Marulli G, Calabrese F, et al.: Bronchial carcinoid tumours in children: surgical treatment and outcome in a single institution. Eur J Pediatr Surg 19 (4): 228-31, 2009. [PUBMED Abstract]
- Lack EE, Harris GB, Eraklis AJ, et al.: Primary bronchial tumors in childhood. A clinicopathologic study of six cases. Cancer 51 (3): 492-7, 1983. [PUBMED Abstract]
- Ahel V, Zubovic I, Rozmanic V: Bronchial adenoid cystic carcinoma with saccular bronchiectasis as a cause of recurrent pneumonia in children. Pediatr Pulmonol 12 (4): 260-2, 1992. [PUBMED Abstract]
- Gaissert HA, Mathisen DJ, Grillo HC, et al.: Tracheobronchial sleeve resection in children and adolescents. J Pediatr Surg 29 (2): 192-7; discussion 197-8, 1994. [PUBMED Abstract]
- Jalal A, Jeyasingham K: Bronchoplasty for malignant and benign conditions: a retrospective study of 44 cases. Eur J Cardiothorac Surg 17 (4): 370-6, 2000. [PUBMED Abstract]
- Shivastava R, Saha A, Mehera B, et al.: Pleuropulmonary blastoma: transition from type I (cystic) to type III (solid). Singapore Med J 48 (7): e190-2, 2007. [PUBMED Abstract]
- Hill DA, Jarzembowski JA, Priest JR, et al.: Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma registry. Am J Surg Pathol 32 (2): 282-95, 2008. [PUBMED Abstract]
- Priest JR, Magnuson J, Williams GM, et al.: Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma. Pediatr Blood Cancer 49 (3): 266-73, 2007. [PUBMED Abstract]
- Priest JR, Hill DA, Williams GM, et al.: Type I pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. J Clin Oncol 24 (27): 4492-8, 2006. [PUBMED Abstract]
- Miniati DN, Chintagumpala M, Langston C, et al.: Prenatal presentation and outcome of children with pleuropulmonary blastoma. J Pediatr Surg 41 (1): 66-71, 2006. [PUBMED Abstract]
- Hill DA, Ivanovich J, Priest JR, et al.: DICER1 mutations in familial pleuropulmonary blastoma. Science 325 (5943): 965, 2009. [PUBMED Abstract]
- Slade I, Bacchelli C, Davies H, et al.: DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome. J Med Genet 48 (4): 273-8, 2011. [PUBMED Abstract]
- Priest JR, McDermott MB, Bhatia S, et al.: Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 80 (1): 147-61, 1997. [PUBMED Abstract]
- Cross SF, Arbuckle S, Priest JR, et al.: Familial pleuropulmonary blastoma in Australia. Pediatr Blood Cancer 55 (7): 1417-9, 2010. [PUBMED Abstract]
- Gutweiler JR, Labelle J, Suh MY, et al.: A familial case of pleuropulmonary blastoma. Eur J Pediatr Surg 18 (3): 192-4, 2008. [PUBMED Abstract]
- Bouron-Dal Soglio D, Harvey I, Yazbeck S, et al.: An association of pleuropulmonary blastoma and cystic nephroma: possible genetic association. Pediatr Dev Pathol 9 (1): 61-4, 2006 Jan-Feb. [PUBMED Abstract]
- Boman F, Hill DA, Williams GM, et al.: Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumors: a report from the International Pleuropulmonary Blastoma Registry. J Pediatr 149 (6): 850-854, 2006. [PUBMED Abstract]
- Priest JR, Williams GM, Manera R, et al.: Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastoma--a report from the International Pleuropulmonary Blastoma Registry. Br J Ophthalmol 95 (7): 1001-5, 2011. [PUBMED Abstract]
- Schultz KA, Pacheco MC, Yang J, et al.: Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry. Gynecol Oncol 122 (2): 246-50, 2011. [PUBMED Abstract]
- Indolfi P, Casale F, Carli M, et al.: Pleuropulmonary blastoma: management and prognosis of 11 cases. Cancer 89 (6): 1396-401, 2000. [PUBMED Abstract]
- Indolfi P, Bisogno G, Casale F, et al.: Prognostic factors in pleuro-pulmonary blastoma. Pediatr Blood Cancer 48 (3): 318-23, 2007. [PUBMED Abstract]
- Priest JR, Andic D, Arbuckle S, et al.: Great vessel/cardiac extension and tumor embolism in pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. Pediatr Blood Cancer 56 (4): 604-9, 2011. [PUBMED Abstract]
- Schmaltz C, Sauter S, Opitz O, et al.: Pleuro-pulmonary blastoma: a case report and review of the literature. Med Pediatr Oncol 25 (6): 479-84, 1995. [PUBMED Abstract]
- Ohta Y, Fujishima M, Hasegawa H, et al.: High therapeutic effectiveness of postoperative irinotecan chemotherapy in a typical case of radiographically and pathologically diagnosed pleuropulmonary blastoma. J Pediatr Hematol Oncol 31 (5): 355-8, 2009. [PUBMED Abstract]
- Venkatramani R, Malogolowkin MH, Wang L, et al.: Pleuropulmonary blastoma: a single-institution experience. J Pediatr Hematol Oncol 34 (5): e182-5, 2012. [PUBMED Abstract]
- de Castro CG Jr, de Almeida SG, Gregianin LJ, et al.: High-dose chemotherapy and autologous peripheral blood stem cell rescue in a patient with pleuropulmonary blastoma. J Pediatr Hematol Oncol 25 (1): 78-81, 2003. [PUBMED Abstract]
- Pleuropulmonary Blastoma Registry. St. Paul, Minn: Children's Hospitals and Clinics of St. Paul. Available online. Last accessed April 04, 2013.
- MacSweeney F, Papagiannopoulos K, Goldstraw P, et al.: An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation. Am J Surg Pathol 27 (8): 1139-46, 2003. [PUBMED Abstract]
- Hill DA, Dehner LP: A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4. Am J Surg Pathol 28 (4): 554-5; author reply 555, 2004. [PUBMED Abstract]
- Gangopadhyay AN, Mohanty PK, Gopal SC, et al.: Adenocarcinoma of the esophagus in an 8-year-old boy. J Pediatr Surg 32 (8): 1259-60, 1997. [PUBMED Abstract]
- Issaivanan M, Redner A, Weinstein T, et al.: Esophageal carcinoma in children and adolescents. J Pediatr Hematol Oncol 34 (1): 63-7, 2012. [PUBMED Abstract]
- Verley JM, Hollmann KH: Thymoma. A comparative study of clinical stages, histologic features, and survival in 200 cases. Cancer 55 (5): 1074-86, 1985. [PUBMED Abstract]
- Hsueh C, Kuo TT, Tsang NM, et al.: Thymic lymphoepitheliomalike carcinoma in children: clinicopathologic features and molecular analysis. J Pediatr Hematol Oncol 28 (12): 785-90, 2006. [PUBMED Abstract]
- Stachowicz-Stencel T, Bien E, Balcerska A, et al.: Thymic carcinoma in children: a report from the Polish Pediatric Rare Tumors Study. Pediatr Blood Cancer 54 (7): 916-20, 2010. [PUBMED Abstract]
- Furman WL, Buckley PJ, Green AA, et al.: Thymoma and myasthenia gravis in a 4-year-old child. Case report and review of the literature. Cancer 56 (11): 2703-6, 1985. [PUBMED Abstract]
- Yaris N, Nas Y, Cobanoglu U, et al.: Thymic carcinoma in children. Pediatr Blood Cancer 47 (2): 224-7, 2006. [PUBMED Abstract]
- Yalçin B, Demir HA, Ciftçi AO, et al.: Thymomas in childhood: 11 cases from a single institution. J Pediatr Hematol Oncol 34 (8): 601-5, 2012. [PUBMED Abstract]
- Carretto E, Inserra A, Ferrari A, et al.: Epithelial thymic tumours in paediatric age: a report from the TREP project. Orphanet J Rare Dis 6: 28, 2011. [PUBMED Abstract]
- Souadjian JV, Enriquez P, Silverstein MN, et al.: The spectrum of diseases associated with thymoma. Coincidence or syndrome? Arch Intern Med 134 (2): 374-9, 1974. [PUBMED Abstract]
- Coulter D, Gold S: Thymoma in the offspring of a patient with Isaacs syndrome. J Pediatr Hematol Oncol 29 (11): 797-8, 2007. [PUBMED Abstract]
- Cowen D, Richaud P, Mornex F, et al.: Thymoma: results of a multicentric retrospective series of 149 non-metastatic irradiated patients and review of the literature. FNCLCC trialists. Fédération Nationale des Centres de Lutte Contre le Cancer. Radiother Oncol 34 (1): 9-16, 1995. [PUBMED Abstract]
- Carlson RW, Dorfman RF, Sikic BI: Successful treatment of metastatic thymic carcinoma with cisplatin, vinblastine, bleomycin, and etoposide chemotherapy. Cancer 66 (10): 2092-4, 1990. [PUBMED Abstract]
- Niehues T, Harms D, Jürgens H, et al.: Treatment of pediatric malignant thymoma: long-term remission in a 14-year-old boy with EBV-associated thymic carcinoma by aggressive, combined modality treatment. Med Pediatr Oncol 26 (6): 419-24, 1996. [PUBMED Abstract]
- Casey EM, Kiel PJ, Loehrer PJ Sr: Clinical management of thymoma patients. Hematol Oncol Clin North Am 22 (3): 457-73, 2008. [PUBMED Abstract]
- Giaccone G, Ardizzoni A, Kirkpatrick A, et al.: Cisplatin and etoposide combination chemotherapy for locally advanced or metastatic thymoma. A phase II study of the European Organization for Research and Treatment of Cancer Lung Cancer Cooperative Group. J Clin Oncol 14 (3): 814-20, 1996. [PUBMED Abstract]
- Loehrer PJ Sr, Jiroutek M, Aisner S, et al.: Combined etoposide, ifosfamide, and cisplatin in the treatment of patients with advanced thymoma and thymic carcinoma: an intergroup trial. Cancer 91 (11): 2010-5, 2001. [PUBMED Abstract]
- Ströbel P, Bargou R, Wolff A, et al.: Sunitinib in metastatic thymic carcinomas: laboratory findings and initial clinical experience. Br J Cancer 103 (2): 196-200, 2010. [PUBMED Abstract]
- Wu KH, Mo XM, Liu YL: Clinical analysis and surgical results of cardiac myxoma in pediatric patients. J Surg Oncol 99 (1): 48-50, 2009. [PUBMED Abstract]
- Burke A, Virmani R: Pediatric heart tumors. Cardiovasc Pathol 17 (4): 193-8, 2008 Jul-Aug. [PUBMED Abstract]
- Becker AE: Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. Pediatr Cardiol 21 (4): 317-23, 2000 Jul-Aug. [PUBMED Abstract]
- Padalino MA, Vida VL, Boccuzzo G, et al.: Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation 126 (1): 22-30, 2012. [PUBMED Abstract]
- Isaacs H Jr: Fetal and neonatal cardiac tumors. Pediatr Cardiol 25 (3): 252-73, 2004 May-Jun. [PUBMED Abstract]
- Elderkin RA, Radford DJ: Primary cardiac tumours in a paediatric population. J Paediatr Child Health 38 (2): 173-7, 2002. [PUBMED Abstract]
- Uzun O, Wilson DG, Vujanic GM, et al.: Cardiac tumours in children. Orphanet J Rare Dis 2: 11, 2007. [PUBMED Abstract]
- Bruce CJ: Cardiac tumours: diagnosis and management. Heart 97 (2): 151-60, 2011. [PUBMED Abstract]
- Boikos SA, Stratakis CA: Carney complex: the first 20 years. Curr Opin Oncol 19 (1): 24-9, 2007. [PUBMED Abstract]
- Carney JA, Young WF: Primary pigmented nodular adrenocortical disease and its associated conditions. Endocrinologist 2: 6-21, 1992.
- Stratakis CA, Kirschner LS, Carney JA: Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab 86 (9): 4041-6, 2001. [PUBMED Abstract]
- Boikos SA, Stratakis CA: Carney complex: pathology and molecular genetics. Neuroendocrinology 83 (3-4): 189-99, 2006. [PUBMED Abstract]
- Kogon B, Shehata B, Katzenstein H, et al.: Primary congenital infantile fibrosarcoma of the heart: the first confirmed case. Ann Thorac Surg 91 (4): 1276-80, 2011. [PUBMED Abstract]
- Tworetzky W, McElhinney DB, Margossian R, et al.: Association between cardiac tumors and tuberous sclerosis in the fetus and neonate. Am J Cardiol 92 (4): 487-9, 2003. [PUBMED Abstract]
- Bader RS, Chitayat D, Kelly E, et al.: Fetal rhabdomyoma: prenatal diagnosis, clinical outcome, and incidence of associated tuberous sclerosis complex. J Pediatr 143 (5): 620-4, 2003. [PUBMED Abstract]
- Beroukhim RS, Prakash A, Buechel ER, et al.: Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience. J Am Coll Cardiol 58 (10): 1044-54, 2011. [PUBMED Abstract]
- Michler RE, Goldstein DJ: Treatment of cardiac tumors by orthotopic cardiac transplantation. Semin Oncol 24 (5): 534-9, 1997. [PUBMED Abstract]
- Stiller B, Hetzer R, Meyer R, et al.: Primary cardiac tumours: when is surgery necessary? Eur J Cardiothorac Surg 20 (5): 1002-6, 2001. [PUBMED Abstract]
- Günther T, Schreiber C, Noebauer C, et al.: Treatment strategies for pediatric patients with primary cardiac and pericardial tumors: a 30-year review. Pediatr Cardiol 29 (6): 1071-6, 2008. [PUBMED Abstract]
- Nagata S, Nakanishi R: Malignant pleural mesothelioma with cavity formation in a 16-year-old boy. Chest 127 (2): 655-7, 2005. [PUBMED Abstract]
- Rosas-Salazar C, Gunawardena SW, Spahr JE: Malignant pleural mesothelioma in a child with ataxia-telangiectasia. Pediatr Pulmonol 48 (1): 94-7, 2013. [PUBMED Abstract]
- Kelsey A: Mesothelioma in childhood. Pediatr Hematol Oncol 11 (5): 461-2, 1994 Sep-Oct. [PUBMED Abstract]
- Moran CA, Albores-Saavedra J, Suster S: Primary peritoneal mesotheliomas in children: a clinicopathological and immunohistochemical study of eight cases. Histopathology 52 (7): 824-30, 2008. [PUBMED Abstract]
- Cioffredi LA, Jänne PA, Jackman DM: Treatment of peritoneal mesothelioma in pediatric patients. Pediatr Blood Cancer 52 (1): 127-9, 2009. [PUBMED Abstract]
- Hofmann J, Mintzer D, Warhol MJ: Malignant mesothelioma following radiation therapy. Am J Med 97 (4): 379-82, 1994. [PUBMED Abstract]
- Pappo AS, Santana VM, Furman WL, et al.: Post-irradiation malignant mesothelioma. Cancer 79 (1): 192-3, 1997. [PUBMED Abstract]
- Hyers TM, Ohar JM, Crim C: Clinical controversies in asbestos-induced lung diseases. Semin Diagn Pathol 9 (2): 97-101, 1992. [PUBMED Abstract]
- Maziak DE, Gagliardi A, Haynes AE, et al.: Surgical management of malignant pleural mesothelioma: a systematic review and evidence summary. Lung Cancer 48 (2): 157-69, 2005. [PUBMED Abstract]
- Milano E, Pourroy B, Rome A, et al.: Efficacy of a combination of pemetrexed and multiple redo-surgery in an 11-year-old girl with a recurrent multifocal abdominal mesothelioma. Anticancer Drugs 17 (10): 1231-4, 2006. [PUBMED Abstract]
- Wall JE, Mandrell BN, Jenkins JJ 3rd, et al.: Effectiveness of paclitaxel in treating papillary serous carcinoma of the peritoneum in an adolescent. Am J Obstet Gynecol 172 (3): 1049-52, 1995. [PUBMED Abstract]