Multiple Pulmonary Hamartomas Research Articles

Tumefactive Pulmonary amyloidosis in an elderly male: A Diagnostic Dilemma

Abstract Introduction/Objective Tumefactive pulmonary amyloidosis is a rare variant of pulmonary amyloidosis characterized by the extracellular deposition of abnormal protein fibrils in the lung parenchyma, forming tumor-like masses called amyloidomas. Although most cases are clinically silent, it may present with findings that mimic a wide range of respiratory conditions (eg. primary or metastatic malignant nodules, granulomatous disease, multiple pulmonary hamartomas etc), thereby necessitating a comprehensive and broad-based approach to differential diagnosis. Methods/Case Report We present a case of an 88-year-old male with a history of smoking and multiple comorbidities. He presented with numerous bilateral pulmonary nodules identified on a CT scan, concerning for malignancy. A biopsy from a left upper lobe lesion revealed necrotic tissue and was non-diagnostic. A PET scan to assess for metastatic disease was unremarkable, and demonstrated mild hypermetabolic activity, confined to the lungs, with standardized uptake values (SUV) ranging from 4.3 to a maximum SUV of 8.9. A subsequent right lung biopsy was positive for amyloid, confirmed by Congo red staining. A Mass spectrometry analysis further identified the amyloid as AL type. Serum, and urine protein electrophoresis with immunofixation were negative for M protein and the serum free light chain assay was normal as well. Significantly, the bone marrow biopsy also yielded normal results, and the patient was diagnosed with a localized form of AL amyloidosis. Results (if a Case Study enter NA) NA Conclusion This case highlights how tumefactive pulmonary amyloidosis poses a diagnostic challenge due to its rarity and resemblance to malignancy on imaging. Careful histopathological examination, including immunohistochemical stains and amyloid typing, was pivotal in establishing the correct diagnosis and underscores the essential role of pathology and laboratory medicine in differentiating rare pulmonary conditions, along with the need for a multidisciplinary approach in complex diagnostic scenarios.

Multiple pulmonary hamartomas with malignant transformation of a giant pulmonary hamartomas: a case report

Pulmonary hamartomas are the most common benign tumors of the lung, typically solitary with a diameter≤4 cm, and rarely undergo malignant transformation. We reported a case of multiple pulmonary hamartomas with malignant transformation of a giant pulmonary hamartoma (approximately 10 cm×12 cm in size). The patient was a 61-year-old female who sought medical attention for the discovery of multiple lesions in the right lung during a routine examination. Computed tomography revealed multiple soft tissue density lesions in the right lung. Surgical resection was performed to remove the lower lobe of the right lung and the lesion located in the posterior segment of the right upper lobe. The pathological results indicated that the right lower lobe lesion had undergone malignant transformation to a high-grade chondrosarcoma originating from a hamartoma, while the right upper lobe lesion remained a hamartoma. By analyzing this case together with the relevant literature, we aimed to emphasize the need for clinical awareness of the malignant potential of pulmonary hamartomas.

A child with multiple pulmonary hamartomas initially thought to be metastasis of Wilms' tumour.

A child with multiple pulmonary hamartomas initially thought to be metastasis of Wilms' tumour.

DIAGNOSTIC STRATEGIES FOR MULTIPLE PULMONARY NODULES

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Multiple pulmonary nodules are often challenging in terms of their workup. Having non-invasive diagnostic methods to exclude benign lesions such as pulmonary hamartomas is desirable and useful in avoiding risky invasive procedures. We present a case highlighting the non-invasive approach which we believe is under recognized. CASE PRESENTATION: A 49 year-old-female with an extensive smoking history was referred for known pulmonary nodules. Chest CT showed multiple ground glass opacities (GGO) within peripheral parenchyma of the lungs [Figures 1-3]. Given the patients history of smoking and the presence of GGO, malignancy was considered. CT imaging was evaluated for intranodular calcification or fat. No calcifications were observed but given the measured fat density of -124 HU a diagnosis of pulmonary hamartomas was made. Comparison with CTs from 15-years prior confirmed the stability and benign nature of the nodules. DISCUSSION: Pulmonary hamartomas are the most common benign neoplasms of the lung, representing 75% of non-malignant lung tumors. Most hamartomas are asymptomatic and are incidentally found on imaging within the peripheral parenchyma, however 10% are endobronchial. They are relatively stable with a volume doubling time over 400 days. Malignant transformation is rare thus observation is safe. However, studies have found an association with primary lung cancer. While typically solitary, pulmonary hamartomas have been observed in multiples, with one study showing an incidence of 3%. Extrapulmonary involvement, especially mucocutaneous and gastrointestinal locations, indicates Cowden disease. Characteristically, pulmonary hamartomas contain fat and/or “popcorn” calcifications, found in 60% and 5-50% of nodules respectively. Fat can be identified on CT with a Hounsfield values of -40 to -120 HU, and when found with smooth, sharply marginated nodules it provides a confident diagnosis of hamartoma. MRI is a less common imaging modality, but chemical-shift MRI shows potential in identifying intracellular lipid when CT is negative for intranodular fat. Additionally, CT texture mapping shows promise in classifying nodules via heterogeneity, something ordinarily reserved to histopathology of biopsied specimens. Lastly, PET-CT is available for characterizing intermediate to large pulmonary nodules, but increased FDG avidity of hamartomas can lead to misdiagnosis. CONCLUSIONS: Multiple pulmonary hamartomas can be diagnosed on chest CT by measuring fat density within nodules. Thin section CT is essential to avoid missing small foci of fat. When inconclusive, other imaging modalities such as chemical-shift MRI and CT texture mapping should be considered before invasive biopsy or PET-CT. Reference #1: Provenzale, James M., Rendon C. Nelson, and Emily N. Vinson, eds. Duke radiology case review: imaging, differential diagnosis, and discussion. Lippincott Williams & Wilkins; 2011. Reference #2: “Primary Lung Tumors Other than Bronchogenic Carcinoma: Benign and Malignant.” Grippi MA et al. Fishman's Pulmonary Diseases and Disorders, Fifth Edition. New York: McGraw-Hill; 2015. Reference #3: Ekinci, G. H., et al. "The frequency of lung cancer in patients with pulmonary hamartomas: An evaluation of clinical, radiological, and pathological features and follow-up data of 96 patients with pulmonary hamartomas.” Revista Portuguesa de Pneumologia (English Edition) 2017; 23(5): 280-286. DISCLOSURES: No relevant relationships by jayanth keshavamurthy, source=Web Response No relevant relationships by Matthew Potter, source=Web Response No relevant relationships by Varsha Taskar, source=Web Response No relevant relationships by Thomas Whitton, source=Web Response

Multiple Pulmonary Chondroid Hamartoma

A 30-year-old male was admitted to our hospital for a 3-month history of cough. The computed tomography (CT) scan of chest revealed multiple lesions with sporadic calcification (popcorn calcification) in the upper and middle lobes of the right lung (Fig. ​(Fig.1).1). A presumptive diagnosis of chondroid hamartoma was made according to the CT-guided percutaneous biopsy. Systemic positron emission tomography-CT (PET-CT) scan was also negative for other lesions. A posterolateral thoracotomy was performed and innumerous firm round yellowish-white nodules were found and spread over the right upper and middle lobes (Fig. ​(Fig.2,2, arrow). The two lobes involved were successfully excised. The final specimen was 25 cm × 15 cm × 10 cm in size, firm, and full of nodules (Fig. ​(Fig.3).3). Postoperative pathological examination confirmed the diagnosis of pulmonary chondroid hamartomas (Fig. ​(Fig.4).4). The patient was discharged 7 days after the surgery without any main complication, and there is no sign of recurrence 3 months later. FIGURE 1. Computed tomography scan of chest showed multiple lesions of the right upper lobe, whereas the lower lobe seemed to be tumor free. Popcorn calcification, a typical imaging manifestation of pulmonary chondroid harmatoma, could be observed. FIGURE 2. The two lobes involved (arrow) seemed to out of physical function because they had fused and presented abnormal color. FIGURE 3. More yellow-white granules were found after the dissecting of the specimen. FIGURE 4. The tumor was mainly composed of chondroid tissue which was surrounded by epithelium, adipose tissue, and smooth muscle tissue could also be observed (hematoxylin and eosin stain, 200X). Pulmonary chondroid hamartoma, as a subtype of pulmonary harmatoma, is defined when chondroid tissue predominates in the composition of the tumor. Harmatoma is the most common benign tumor of the lung, which accounts for 8% of pulmonary neoplasms. It is well known that harmatomas are composed of tissue elements normally found at that site, but which are growing in a disorganized mass, usually seem as single, round nodules with distinct boundaries.1 A cytogenetic analysis of the pulmonary hamartomas showed an abnormal karyotype and revealed recombinations between chromosomal bands 6p21 and 14q24,2 which supported that a hamartoma of the lung was a true neoplasm. Solitary pulmonary hamartoma is a common benign tumor that is usually seen in males, whereas multiple pulmonary hamartomas are rare and predominate in females.3,4 Only a few reports of multiple pulmonary chondroid hamartomas were described so far. Here we presented an extremely rare case of multiple pulmonary chondroid hamartomas with innumerous nodules in the right upper and middle lobes. Most patients with pulmonary hamartoma are asymptomic, whereas some patients may have respiratory symptoms such as hemoptysis, cough, phlegm, or chest pain.5 Generally speaking, a non-anatomical resection is curative for a benign lesion although the location of the lesion plays an important role in the decision of surgical type.1 Video-assisted thoracic surgery now seems to be a trend in dealing with benign tumors because of less morbidity of surgical complications and hospital stay compared with traditional thoracotomy. On the other hand, endobronchial hamartomas can be well managed by endoscopy and laser ablation.6

Multiple Pulmonary Hamartomas Penetrating the Visceral Pleura: Report of a Case

We report a case of multiple pulmonary hamartomas with a very unusual growth pattern. A 63-year-old woman underwent video-assisted thoracic surgery to investigate a round shadow seen on a routine chest radiograph. We found two tumors in the right S4, penetrating the visceral pleura and adhered to the right upper and lower lobes, respectively. Both tumors were removed and diagnosed histologically as pulmonary hamartoma. We hypothesized that these subpleural hamartomas might have grown invasively to penetrate the visceral pleura.

Multiple Pulmonary Hamartomas Masquerading as Metastatic Lung Tumors: A Case Report

Background. The majority of pulmonary hamartomas are recognized as solitary tumors. It is difficult to differentiate multiple pulmonary hamartomas from pulmonary malignant nodules in a patient who has undergone previous surgery for metastatic lung tumor. We report an extremely rare surgical case of multiple pulmonary hamartomas. Case. A 57-year-old woman with an abnormal chest radiograph was referred to our hospital in August 2004 for further evaluation. Chest radiography and computed tomography showed multiple nodular lesions in the posterior basal segment of the right lung. She had undergone surgery for rectal cancer in September 2002 and had undiagnosed bilateral pulmonary nodules identified at that time. Because the pulmonary nodules in the left lung increased in size, partial resection of the left upper lobe was performed in February 2003. Postoperative histopathologic diagnosis of the lung tumor was metastatic adenocarcinoma originating from the rectal cancer. She underwent chemotherapy starting in March 2003, but an increase in number and size of the pulmonary nodules in the right lower lobe was demonstrated on chest films. She underwent partial resection of the posterior basal segment of the right lung by video-assisted thoracoscopic surgery in September 2004 to remove all nodules. Postoperative histopathologic examination of the resected specimens revealed multiple pulmonary hamartomas consisting of various-sized bronchioles without a cartilageous component. She recovered uneventfully and there have been no signs of recurrence for 40 months since the pulmonary resection in February 2003. Conclusion. We encountered an extremely rare surgical case of multiple pulmonary hamartomas. If it is difficult to differentiate it from metastatic lung tumors in this case, minimally invasive surgery such as video-assisted thoracoscopic surgery should be performed to establish a definitive diagnosis.

Intrapulmonary lipoma associated with multiple pulmonary hamartomas

Abstract A pulmonary lipoma is described in a 64-year-old male, involving the right middle lobe, associated with a separate area of intrapulmonary ossification and a central intrabronchial hamartoma. It is postulated that the lipoma most probably arose from one of the multiple pulmonary hamartomas.

Multiple Pulmonary Hamartomas: A Case Report

Hamartomas are the most common benign neoplasms of the lung. However, multiple pulmonary hamar-tomas are veryrare. We describe the radiographic findings of multiple pulmonary hamartomas without Carney’s or pulmonaryhamartoma syndrome with review the literature describing to this entity.

Primary pulmonary amyloidosis: Report of two cases

Two differing examples of primary parenchymal nodular amyloid of the lung are reported. Both cases were proved by biopsy of the lung lesion as well as extensive evaluation for evidence of generalized amyloidosis. Review of the literature reveals 35 previously reported cases which fit the same description. Amyloidosis is a benign condition, usually causing no symptoms, requiring no treatment, but generally requiring biopsy for diagnosis. Pathological and roentgenologic characteristics are reviewed, and studies to establish its primary and localized nature are recommended. The etiology is not clear. The differential diagnosis includes a variety of metastatic neoplasms, granulomatous diseases, metastatic lung abscesses, multiple pulmonary hamartomas, bronchopneumonia, and infections.