A 31-Year-Old Woman With Hemoptysis and an Intrathoracic Mass

A 31-year-old woman presented with 3 months of hemoptysis (one-half cup daily), left chest pain, and intermittent expectoration. The sputum was described as having a “cheeselike” appearance, occurring every 5 to 6 days. She reported mild shortness of breath for 2 months but no weight loss, night sweats, or fevers. Her chest pain was episodic in nature and occasionally pleuritic. She denied nausea, vomiting, rashes, or joint pain. Her menses were regular and of normal quantity. Six years prior, she presented to another institution with an acute onset of hemoptysis and underwent left internal mammary arterial embolization. A diagnosis was not established, and she was lost to follow-up. The hemoptysis had subsided following the embolization but now returned. She underwent a left eye enucleation at age 4 years for retinoblastoma. She received no radiation, chemotherapy, or subsequent genetic evaluation. She reported no pneumonia, foreign-body aspiration, or coagulopathy. She was a former smoker (5 years), occasionally drank alcohol, and denied illicit drug use. She was from Denver and had not traveled outside of the United States. There were no risk factors for TB. She took no medications. There was no family history of retinoblastoma, other malignancies, or pulmonary disease. The physical examination included: temperature, 37.8°C; pulse, 64 beats/min; BP, 122/70 mm Hg; respiratory rate, 14 breaths/min; and oxygen saturation, 97% while breathing room air. She was thin and in no acute distress. There was a left prosthetic eye. An oropharyngeal examination revealed normal dentition and an absence of mucosal lesions. The neck was supple without masses or lesions. Cardiovascular exam revealed a normal S1, physiologically split S2, and no gallops, murmurs, or pericardial rub. Lung examination revealed decreased breath sounds over the anterior left thorax with early inspiratory crackles. The abdominal, lymphatic, and skin examinations were normal. Strength was normal. The WBC count was 10.0 × 103 cells/μL with 64% neutrophils, 24% lymphocytes, 6% monocytes, and 6% eosinophils; hemoglobin 14.1 g/dL; hematocrit 42.2%; and platelet count 296 × 103 cells/μL. The serum electrolytes, liver function tests, coagulation studies, and urinalysis were normal. HIV testing was negative. The chest radiograph is shown in Figures 1 and ​and2,2, and the CT scan is shown in Figure 3. Figure 1. Posteroanterior chest radiograph. Figure 2. Lateral chest radiograph. Figure 3. CT scan of the thorax. What Is the Differential Diagnosis? The patient has a large, solitary, parenchymal mass in the left hemithorax. The chest radiograph (Figs 1, ​,2)2) showed a heterogenous left paracardiac mass with central calcifications and embolization coils within the left internal mammary artery. A chest CT scan (Fig 2) showed a large 7 × 7 cm complex mass in the left anterior hemithorax containing fat, nonfat soft tissue, and osseous structures with partial lingular atelectasis. The thyroid gland appeared normal, and there were small bilateral axillary reactive lymph nodes. The presence of continued hemoptysis suggested a communication between the mass and an adjacent bronchus, and her transient improvement following embolization of the left internal mammary artery suggested altered vascular architecture. The possibilities include the following: (1) a benign or malignant parenchymal tumor, (2) a tumor located in the anterior mediastinum, (3) a fungal or mycobacterial infection, (4) a lung abscess, or (5) a hydatid cyst. The leading possibilities are an anterior mediastinal mass or a benign solitary parenchymal tumor. The absence of constitutional symptoms, lymphadenopathy, and further radiographic lesions argued against a diffuse or rapidly growing metastatic disease. The patient had no history of exposure to TB, and the mass involved the inferior and anterior aspect of the thorax, making TB less likely. A lung abscess of this size with bronchial communication more likely than not would result in fevers, purulent and foul-smelling sputum, and weight loss. In addition to the solid structure and marked heterogeneity of the computed tomographic image, there was no exposure history suggesting the possibility of a hydatid cyst. Hamartoma is the most common benign lung tumor, accounting for 75% of all benign lung nodules and 5% to 10% of solitary pulmonary nodules. Hamartomas most often present in the sixth or seventh decades1,2 but can occur in young adults as well. The diameter is usually < 6 cm,1,3 but they have been reported as large as 25.5 cm.4 The mass in this patient had a heterogenous appearance with a radiographically apparent focus of calcification. Calcification has been reported to occur in 3% to 32% of hamartoma cases but tends to have a punctate or “popcornlike” pattern.1,5,6 Calcification can also occur in other benign pulmonary tumors, including plasma cell granulomas and desmoid tumors. Plasma cell granulomas, also referred to as an inflammatory pseudotumor, most often present in childhood or adolescence and usually do not exceed 6 cm in transverse diameter.7 Desmoid tumors most often arise from the chest wall and invade surrounding structures, although they sometimes arise from the lung parenchyma.8 An intrapulmonary or mediastinal lipoma is unlikely to have this CT scan appearance because they tend to have uniform fatty attenuation. Additionally, intrapulmonary lipoma more often occurs on the right and in middle-aged men.9 Pulmonary leiomyoma would also have a homogenous radiographic appearance.10 Finally, in a young woman with recurrent, episodic hemoptysis, pulmonary endometrioma should be considered. Radiographically this presents as small pulmonary nodules and not a large heterogenous mass.11,12 It was difficult to determine based on the CT scan appearance whether this large complex mass originated from the anterior mediastinum or from the lung parenchyma. Thymoma, the most common anterior mediastinal mass, rarely has calcification, and if it occurs, the calcifications are small, curvilinear, or punctate.13 Invasive thymomas are rare, but sometimes they can extend into the lung causing hemoptysis.13 This is an unusual presentation of lymphoma because there are no systemic symptoms or diffuse lymphadenopathy. A mediastinal or intrapulmonary teratoma can present as a large, heterogenous mass with fat, soft tissue, and osseous structures, and can invade an adjacent bronchus. Occasionally patients can expectorate tissue from an intrabronchial tumor, which could account for this patient’s cheeselike sputum. What Should be Done Next? This patient had hemoptysis with recurrent symptoms. She was hemodynamically stable and did not have a coagulopathy. Although a tuberculin skin test should be performed and sputum could be examined for the presence of atypical cells or infection, the next logical step is to obtain tissue for a histologic diagnosis. Consideration should be given to performing a bronchoscopy to determine whether there is endobronchial involvement. Although surgical resection is not required for many hamartomas, the large size, bronchial invasion, and persistent symptoms caused by this mass represent indications for surgical resection. The sampling error of a percutaneous needle aspiration or transbronchial biopsy in such a heterogenous lesion is sufficiently high. Thus, surgical resection is recommended. What is the diagnosis? Diagnosis: mature intrapulm0onary teratoma The results of the workup revealed the following. Her tuberculin skin test was negative. At the time of surgery, a fiberoptic bronchoscopy was performed and did not reveal any abnormalities. Next, the patient was intubated with a double-lumen endotracheal tube, and a left lateral thoracotomy incision at the fifth intercostal space was undertaken. The large heterogenous mass appeared to originate from the lingula and was adherent to the mediastinal pleura and the anterolateral chest wall. During surgical manipulation of the mass, several hairs were noticed within the endotracheal tube. A complete lingulectomy was performed along with excision of an encapsulated 7×5.5×6.5-cm mass without complication. Pathologic evaluation revealed a thick, fibrous capsule with visible areas of calcification. The internal contents of the mass were separated into loculations by the fibrous capsule and included areas of a green-yellow granular substance with intermixed hair, soft adipose tissue, and a mucous white substance. Histologically, the resected specimen displayed a mature, intrapulmonary teratoma; it specifically showed well-differentiated tissues derived from all three germinal layers: respiratory, pancreatic, and intestinal epithelium from endoderm, pilosebaceous units from mesoderm, and hair follicles and squamous epithelium of skin from the ectoderm (Figs 4, ​,55). Figure 4. Hematoxylin-eosin-stained image from the resected intrathoracic mass demonstrating skin with hair follicle and sebaceous glands on the right. There is laminated keratin material on the left. Original magnification ×200. Figure 5. This hematoxylin-eosin-stained section demonstrates cartilage on the right and pancreatic acini on the left. Original magnification ×200.