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Case Study
Intrapulmonary teratoma: Report of a case and review of literature
Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315583763 aan.sagepub.com
Kallol Dasbaksi, Suranjan Haldar, Kaushik Mukherjee, Ushnish Chakraborty, Pinaki Majumdar and Plaban Mukherjee
Abstract Intrapulmonary teratomas are rare tumors that are presumed to develop in association with mediastinal teratomas. This report describes the management of a rare case of a benign cystic intrapulmonary teratoma in the left upper lobe in a 26-year-old lady, which was successfully treated by lobectomy, with no recurrence after 4 years of follow-up.
Keywords Dermoid cyst, lung neoplasms, pneumonectomy, teratoma
Introduction Henry Christian1 first reported in detail the clinical and autopsy findings of 40 cases of mediastinal teratoma and intrapulmonary teratoma (IPT), which also included the first case reports of these entities. IPTs are rare, and up to 2010, only 81 cases had been reported.2 IPTs are postulated to develop from teratomatous foci in the mediastinum, which are carried laterally by respiratory outgrowth from the foregut into the developing lung.3 The voluminous lung allows these lesions to grow into large masses until they become symptomatic around the 3rd or 4th decade.2 This report describes the management of a rare case of benign cystic intrapulmonary teratoma in the left upper lobe (LUL) of the lung.
Case report A 26-year-old lady presented with left-sided chest pain, recurrent episodes of lower respiratory tract infection, and mild hemoptysis for one and a half years. No history of loss of weight or appetite was reportedly present. Chest radiography showed a well-defined large rounded space-occupying lesion in the LUL, close to its mediastinal aspect (Figure 1A, Figure 1B). A Mantoux test and sputum culture for acid-fast bacilli were negative. Computed tomography of the chest revealed that the mass was a heterogenous, mixedattenuating, predominantly cystic lesion in the LUL with internal septation and peripheral enhancement
(Figure 1C, Figure 1D), and without any mediastinal lymphadenopathy. Bronchoscopy was declined by the patient. Hydatid cyst was the provisional diagnosis, and fine-needle aspiration biopsy was avoided. Ultrasonography of the abdomen was normal with no evidence of any intraabdominal mass. The patient was operated on under general anesthesia via a left posterolateral thoracotomy through the 5th intercostal space. A whitish intrapulmonary thin-walled cystic mass was present in the LUL (Figure 2A), with adhesions to the chest wall and mediastinal pleura. The mass ruptured during manipulation, with extrusion of white creamy material and hairs. A multilobulated firm mass, approximately 8 6 cm in size with attached hairs, was found inside the cyst (Figure 2C, Figure 2D). A left upper lobectomy was performed (Figure 2B). The patient’s recovery was uneventful and she was discharged after 3 weeks. Histology of the mass displayed an outer lining of stratified squamous epithelium with sweat glands (Figure 3A), and sebaceous glands in the collagenous connective tissue in deeper layers Department of Cardiothoracic and Vascular Surgery, Medical College, Kolkata, West Bengal, India Corresponding author: Kallol Dasbaksi, Department of Cardiothoracic and Vascular Surgery, Medical College, Kolkata, 88 College Street, Kolkata, West Bengal 700073, India. Email: [email protected]
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Figure 1. Chest radiographs showing (A) a space-occupying lesion in the upper lobe of the left lung, adjacent to the mediastinal aspect in posteroanterior view, and (B) prominent bronchovascular markings (arrow) in lateral view. (C) Computed tomography in mediastinal window with an arrow showing the translucent areas due to fat below the folded and lobulated mass of the intrapulmonary teratoma, preoperatively presumed to be due to air within the space-occupying lesion. (D) The pulmonary window.
Figure 2. (A) Perioperative view of the partially ruptured cystic mass in the exposed left upper lobe, lying below the index finger. (B) Specimen of the left upper lobe and the excised teratoma. (C) Lobulations in the teratoma. (D) Macroscopic view of the epidermis and dermis with thick subcutaneous tissue in cut section, and hairs overlying an adjacent lobule.
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Figure 3. Histopathological evaluation of the tissue: (A) epidermis with keratinised stratified squamous epithelium and dermis with sweat glands. Original magnification40. (B) Dermis containing sebaceous glands. Original magnification40.
(Figure 3B); these findings were consistent with a diagnosis of benign cystic teratoma. There was no evidence of immature cells or malignant change in any part of the tumor. Postoperatively, serum tumor markers for malignant teratoma (serum alpha-fetoprotein and beta human chorionic gonadotrophic hormone) were found to be negative. The patient was doing well with no recurrence after 4 years of follow-up.
Discussion Mediastinal teratoma is believed to arise from sequestrated cell rests of primordial germ cells during their migration from yolk sac to the developing gonads in the genital ridge of the embryo. Migration of pluripotent primitive embryonic stem cells derived from the inner cell mass of preimplantation embryos, probably due to some disturbances during embryogenesis, are considered to be another source of mediastinal teratoma.2,4 Some investigators believe that teratomatous foci in the region of the third pharyngeal pouch may descend with the thymus into the mediastinum to form mediastinal teratoma, and subsequent migration results in IPT.2,3,5 A report of IPT developing 10 years after successful removal of an ipsilateral mediastinal teratoma validates the theory of their common origin.6 Susceptibility to teratoma formation in genetically predisposed individuals and decreased host immunity have also been postulated to be responsible for the growth of a teratoma and its malignant transformation.4 Exclusion of a gonadal tumor with pulmonary metastasis is essential to diagnose IPT. As in our case, IPT is more common in the LUL of the lungs.5 Like teratomas in other locations, IPTs are encapsulated tumors containing tissues or organ components resembling normal derivatives of all 3, and sometimes 2, embryonic germ layers. The contents are usually teeth, skin, and hair, as well as thymic, bony, and cartilaginous tissues. The tumor in our case contained derivatives of only 2 germ layers: ectoderm and mesoderm. Endodermal derivatives such as pancreatic or gastrointestinal tissues in some cystic IPT may secrete
proteolytic enzymes which make them prone to rupture.2,5 Malignant teratomas are more solid and nodular.2,5 Benign teratomas on the other hand are more often cystic, contain mature tissues and fats, and are also called mature cystic teratomas or dermoid cysts. Peripheral translucency of cystic IPT in computed tomography images can be caused by leakage of air due to erosion into the bronchial tree, and this air-fluid contrast is used to distinguish IPT from mediastinal teratoma.5 However, in the differential diagnosis, ruptured hydatid cysts, fungal masses, and lung abscess also need consideration. Although the presence of peripheral translucency and the appearance of lobulated structures in the cystic cavity on computed tomography are stated to be characteristics of benign cystic IPT, due to the presence of fat and sebaceous materials,5 the preoperative diagnosis in our case was in favor of ruptured hydatid cyst of the lung because pulmonary hydatid cysts are common in the Indian subcontinent. The true nature was unfolded at operation. A large communication of the IPT with the bronchial tree can cause expectoration of hairs. Although less common these days, this phenomenon was often reported in longstanding cases of IPT and even mediastinal teratoma in the 19th century when watchful documentation of the natural history followed by autopsy was the norm. Early excision of IPT after diagnosis is advisable because an apparently benign IPT presenting as a dermoid cyst may harbor immature tissues in 20% to 30% cases, which may turn into an invasive malignancy if left unoperated.5 Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Christian HA. Dermoid cysts and teratomata of the anterior mediastinum. J Med Res 1902; 7: 54–71.
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2. Macht M, Mitchell JD, Cool C, Lynch DA, Babu A and Schwarz MI. A 31-year-old woman with hemoptysis and an intrathoracic mass. Chest 2010; 138: 213–219. 3. Day DW and Taylor SA. An intrapulmonary teratoma associated with thymic tissue. Thorax 1975; 30: 582–587. 4. Gordeeva OF. Pluripotent cells in embryogenesis and in teratoma formation [Review]. J Stem Cells 2011; 6: 51–63.
5. Zenker D and Aleksic I. Intrapulmonary cystic benign teratoma: a case report and review of the literature formation [Review]. Ann Thorac Cardiovasc Surg 2004; 10: 290–292. 6. Pra¨uer HW, Mack D and Babic R. Intrapulmonary teratoma 10 years after removal of a mediastinal teratoma in a young man. Thorax 1983; 38: 632–634.
Downloaded from aan.sagepub.com at University of Otago Library on October 12, 2015
(AAN)
[1–4] [PREPRINTER stage]
Case Study
Intrapulmonary teratoma: Report of a case and review of literature
Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315583763 aan.sagepub.com
Kallol Dasbaksi, Suranjan Haldar, Kaushik Mukherjee, Ushnish Chakraborty, Pinaki Majumdar and Plaban Mukherjee
Abstract Intrapulmonary teratomas are rare tumors that are presumed to develop in association with mediastinal teratomas. This report describes the management of a rare case of a benign cystic intrapulmonary teratoma in the left upper lobe in a 26-year-old lady, which was successfully treated by lobectomy, with no recurrence after 4 years of follow-up.
Keywords Dermoid cyst, lung neoplasms, pneumonectomy, teratoma
Introduction Henry Christian1 first reported in detail the clinical and autopsy findings of 40 cases of mediastinal teratoma and intrapulmonary teratoma (IPT), which also included the first case reports of these entities. IPTs are rare, and up to 2010, only 81 cases had been reported.2 IPTs are postulated to develop from teratomatous foci in the mediastinum, which are carried laterally by respiratory outgrowth from the foregut into the developing lung.3 The voluminous lung allows these lesions to grow into large masses until they become symptomatic around the 3rd or 4th decade.2 This report describes the management of a rare case of benign cystic intrapulmonary teratoma in the left upper lobe (LUL) of the lung.
Case report A 26-year-old lady presented with left-sided chest pain, recurrent episodes of lower respiratory tract infection, and mild hemoptysis for one and a half years. No history of loss of weight or appetite was reportedly present. Chest radiography showed a well-defined large rounded space-occupying lesion in the LUL, close to its mediastinal aspect (Figure 1A, Figure 1B). A Mantoux test and sputum culture for acid-fast bacilli were negative. Computed tomography of the chest revealed that the mass was a heterogenous, mixedattenuating, predominantly cystic lesion in the LUL with internal septation and peripheral enhancement
(Figure 1C, Figure 1D), and without any mediastinal lymphadenopathy. Bronchoscopy was declined by the patient. Hydatid cyst was the provisional diagnosis, and fine-needle aspiration biopsy was avoided. Ultrasonography of the abdomen was normal with no evidence of any intraabdominal mass. The patient was operated on under general anesthesia via a left posterolateral thoracotomy through the 5th intercostal space. A whitish intrapulmonary thin-walled cystic mass was present in the LUL (Figure 2A), with adhesions to the chest wall and mediastinal pleura. The mass ruptured during manipulation, with extrusion of white creamy material and hairs. A multilobulated firm mass, approximately 8 6 cm in size with attached hairs, was found inside the cyst (Figure 2C, Figure 2D). A left upper lobectomy was performed (Figure 2B). The patient’s recovery was uneventful and she was discharged after 3 weeks. Histology of the mass displayed an outer lining of stratified squamous epithelium with sweat glands (Figure 3A), and sebaceous glands in the collagenous connective tissue in deeper layers Department of Cardiothoracic and Vascular Surgery, Medical College, Kolkata, West Bengal, India Corresponding author: Kallol Dasbaksi, Department of Cardiothoracic and Vascular Surgery, Medical College, Kolkata, 88 College Street, Kolkata, West Bengal 700073, India. Email: [email protected]
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Figure 1. Chest radiographs showing (A) a space-occupying lesion in the upper lobe of the left lung, adjacent to the mediastinal aspect in posteroanterior view, and (B) prominent bronchovascular markings (arrow) in lateral view. (C) Computed tomography in mediastinal window with an arrow showing the translucent areas due to fat below the folded and lobulated mass of the intrapulmonary teratoma, preoperatively presumed to be due to air within the space-occupying lesion. (D) The pulmonary window.
Figure 2. (A) Perioperative view of the partially ruptured cystic mass in the exposed left upper lobe, lying below the index finger. (B) Specimen of the left upper lobe and the excised teratoma. (C) Lobulations in the teratoma. (D) Macroscopic view of the epidermis and dermis with thick subcutaneous tissue in cut section, and hairs overlying an adjacent lobule.
Downloaded from aan.sagepub.com at University of Otago Library on October 12, 2015
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Figure 3. Histopathological evaluation of the tissue: (A) epidermis with keratinised stratified squamous epithelium and dermis with sweat glands. Original magnification40. (B) Dermis containing sebaceous glands. Original magnification40.
(Figure 3B); these findings were consistent with a diagnosis of benign cystic teratoma. There was no evidence of immature cells or malignant change in any part of the tumor. Postoperatively, serum tumor markers for malignant teratoma (serum alpha-fetoprotein and beta human chorionic gonadotrophic hormone) were found to be negative. The patient was doing well with no recurrence after 4 years of follow-up.
Discussion Mediastinal teratoma is believed to arise from sequestrated cell rests of primordial germ cells during their migration from yolk sac to the developing gonads in the genital ridge of the embryo. Migration of pluripotent primitive embryonic stem cells derived from the inner cell mass of preimplantation embryos, probably due to some disturbances during embryogenesis, are considered to be another source of mediastinal teratoma.2,4 Some investigators believe that teratomatous foci in the region of the third pharyngeal pouch may descend with the thymus into the mediastinum to form mediastinal teratoma, and subsequent migration results in IPT.2,3,5 A report of IPT developing 10 years after successful removal of an ipsilateral mediastinal teratoma validates the theory of their common origin.6 Susceptibility to teratoma formation in genetically predisposed individuals and decreased host immunity have also been postulated to be responsible for the growth of a teratoma and its malignant transformation.4 Exclusion of a gonadal tumor with pulmonary metastasis is essential to diagnose IPT. As in our case, IPT is more common in the LUL of the lungs.5 Like teratomas in other locations, IPTs are encapsulated tumors containing tissues or organ components resembling normal derivatives of all 3, and sometimes 2, embryonic germ layers. The contents are usually teeth, skin, and hair, as well as thymic, bony, and cartilaginous tissues. The tumor in our case contained derivatives of only 2 germ layers: ectoderm and mesoderm. Endodermal derivatives such as pancreatic or gastrointestinal tissues in some cystic IPT may secrete
proteolytic enzymes which make them prone to rupture.2,5 Malignant teratomas are more solid and nodular.2,5 Benign teratomas on the other hand are more often cystic, contain mature tissues and fats, and are also called mature cystic teratomas or dermoid cysts. Peripheral translucency of cystic IPT in computed tomography images can be caused by leakage of air due to erosion into the bronchial tree, and this air-fluid contrast is used to distinguish IPT from mediastinal teratoma.5 However, in the differential diagnosis, ruptured hydatid cysts, fungal masses, and lung abscess also need consideration. Although the presence of peripheral translucency and the appearance of lobulated structures in the cystic cavity on computed tomography are stated to be characteristics of benign cystic IPT, due to the presence of fat and sebaceous materials,5 the preoperative diagnosis in our case was in favor of ruptured hydatid cyst of the lung because pulmonary hydatid cysts are common in the Indian subcontinent. The true nature was unfolded at operation. A large communication of the IPT with the bronchial tree can cause expectoration of hairs. Although less common these days, this phenomenon was often reported in longstanding cases of IPT and even mediastinal teratoma in the 19th century when watchful documentation of the natural history followed by autopsy was the norm. Early excision of IPT after diagnosis is advisable because an apparently benign IPT presenting as a dermoid cyst may harbor immature tissues in 20% to 30% cases, which may turn into an invasive malignancy if left unoperated.5 Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Christian HA. Dermoid cysts and teratomata of the anterior mediastinum. J Med Res 1902; 7: 54–71.
Downloaded from aan.sagepub.com at University of Otago Library on October 12, 2015
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2. Macht M, Mitchell JD, Cool C, Lynch DA, Babu A and Schwarz MI. A 31-year-old woman with hemoptysis and an intrathoracic mass. Chest 2010; 138: 213–219. 3. Day DW and Taylor SA. An intrapulmonary teratoma associated with thymic tissue. Thorax 1975; 30: 582–587. 4. Gordeeva OF. Pluripotent cells in embryogenesis and in teratoma formation [Review]. J Stem Cells 2011; 6: 51–63.
5. Zenker D and Aleksic I. Intrapulmonary cystic benign teratoma: a case report and review of the literature formation [Review]. Ann Thorac Cardiovasc Surg 2004; 10: 290–292. 6. Pra¨uer HW, Mack D and Babic R. Intrapulmonary teratoma 10 years after removal of a mediastinal teratoma in a young man. Thorax 1983; 38: 632–634.
Downloaded from aan.sagepub.com at University of Otago Library on October 12, 2015
