Inflammatory rhabdomyoblastic tumor, pheochromocytoma, and pulmonary hamartoma in a patient with neurofibromatosis type 1: a case report.

BACKGROUND: Inflammatory rhabdomyoblastic tumors are relatively recently recognized soft tissue tumors with a low malignant potential. Here, we present a case of concurrent inflammatory rhabdomyoblastic tumor (IRMT), adrenal pheochromocytoma, and pulmonary hamartoma in a patient with neurofibromatosis type 1 (NF1). To our knowledge, this is the first time that this constellation of tumors has been described in the literature. CASE PRESENTATION: A female patient in her late 20s with known NF1 was diagnosed with an inflammatory rhabdomyoblastic tumor, pheochromocytoma, and pulmonary hamartoma in a short succession. IRMT was found to harbor a near-haploid genome and displayed a typical immunohistochemical profile as well as a focal aberrant p53 expression pattern. CONCLUSIONS: This case report strengthens the theory that defects in the tumor suppressor NF1 play a central role in the pathogenesis of inflammatory rhabdomyoblastic tumors and that IRMT may be part of the spectrum of neurofibromatosis type 1 related tumors.

A rare case of asymptomatic giant pulmonary hamartoma.

BACKGROUND: Pulmonary hamartomas are benign lung lesions. Histopathologically, pulmonary hamartoma is composed of varying amounts of mesenchymal elements, including chondroid tissue, mature adipose tissue, fibrous stroma, smooth muscle, and entrapped respiratory epithelium. Most pulmonary hamartoma cases are asymptomatic and found incidentally during imaging. They usually appear as well-circumscribed lesions with the largest dimension of less than 4 cm. Asymptomatic giant pulmonary hamartomas that more than 8 cm are rare. CASE PRESENTATION: In the current case report, a 12.0 × 9.5 × 7.5 cm lung mass was incidentally noticed in a 59-year-old female during a heart disease workup. Grossly, the lesion was lobulated with pearly white to tan-white solid cut surface and small cystic areas. Microscopically, representative tumor sections demonstrate a chondromyxoid appearance with relatively hypocellular stroma and entrapped respiratory epithelium at the periphery. No significant atypia is noted. No mitosis is noted, and the proliferative index is very low (< 1%) per Ki-67 immunohistochemistry. Mature adipose tissue is easily identifiable in many areas. Histomorphology is consistent with pulmonary hamartoma. A sarcoma-targeted gene fusion panel was further applied to this case. Combined evaluation of microscopic examination and sarcoma-targeted gene fusion panel results excluded malignant sarcomatous transformation in this case. The mediastinal and hilar lymph nodes are histologically benign. After surgery, the patient had an uneventful postoperative period. CONCLUSIONS: Giant pulmonary hamartoma is rare; our case is an example of a huge hamartoma in an asymptomatic patient. The size of this tumor is concerning. Thus, careful and comprehensive examination of the lesion is required for the correct diagnosis and to rule out co-existent malignancy.

Pulmonary Endobronchial Hamartoma Presenting With Post-obstructive Pneumonia.

Pulmonary hamartomas (PH) are rare but are the most common benign tumors found in the lungs. They are slow-growing and are usually found incidentally on chest imaging during the sixth decade of life. Approximately 10% of pulmonary hamartomas are endobronchial. Rarely, pulmonary hamartomas can cause a spectrum of pulmonary symptoms depending on their size and location. We present a case of endobronchial hamartoma causing airway obstruction and recurrent post-obstructive pneumonia.

A rare case report: co-occurrence of two types of lung cancer with hamartoma and pulmonary tuberculosis.

With the widespread use of low-dose chest Computed Tomography (CT), lung nodules are being increasingly detected. Common pulmonary conditions such as lung adenocarcinoma, lung squamous cell carcinoma, and tuberculosis are typically diagnosable through imaging examinations. Nevertheless, when multiple types of lung cancer are combined with other benign tumors, how can an accurate diagnosis be made? In this report, we present a rare case of a patient with the simultaneous occurrence of lung adenocarcinoma, lung squamous cell carcinoma, pulmonary tuberculosis, and pulmonary hamartoma, which has not been previously reported. This patient underwent surgical intervention in the Department of Thoracic Surgery at the Second Hospital of Jilin University and has now fully recovered and been discharged. The patient's preoperative positron emission tomography-CT(PET-CT)results did not align with the postoperative pathological diagnosis. The imaging findings were atypical, and the pathological diagnosis was exceptionally rare. We share this case report to contribute to the accumulation of clinical experience.

Pulmonary placental transmogrification: a difficult pattern in differential diagnosis of pulmonary hamartomas from a tertiary care hospital in Turkey.

OBJECTIVE: Pulmonary placental transmogrification (PT) is a benign lesion curable by resection, represented by an unusual peculiar morphological variation including placentoid bullous change in the pulmonary hamartoma. In this retrospective study, we aimed to examine the histopathological features of pulmonary hamartomas in lung, to evaluate the different histological components, especially PT, and to investigate importance of PT pattern and its relationship with other clinicopathological features. METHODS: Thirty-five cases of pulmonary hamartomas were recruited from the records between 2001 and 2021, divided into two groups according to presence of PT, as PT (-) and PT (+) in pathological examination. RESULTS: 77.1% of all patients were male. There was no significant difference between the two groups in terms of age, sex, comorbidity, presence of symptoms, tumor localization, and radiological findings (P > 0.05). Pulmonary hamartomas were resected totally from 28 patients (80%). Five of these patients (17.9%) had PT components in resection materials with varying degree between 5 and 80%, and all were from male patients. Examination with frozen sections were performed in 15 PT (-) and 5 PT (+) patients but diagnosis with frozen sections was not achieved in any of PT (+) patients. Most of materials included chondroid components (52.22 ± 29.7%) in both groups (P < 0.05). CONCLUSION: The placental papillary projections are available patterns associated with a pulmonary hamartoma and these projections observed especially in frozen sections are very crucial to recognize PT pattern in hamartomas, as they can result in confusions in differential diagnosis of malignities.

Diagnosis value of 18F-Fluoro-D-glucose positron emission tomography-computed tomography in pulmonary hamartoma: a retrospective study and systematic review.

PURPOSE: The diagnosis of pulmonary hamartoma (PH) based on computed tomography (CT) is a challenge, especially in patients with atypical imaging characteristics. This study was aimed at summarizing the imaging characteristic of 18F-Fluoro-D-glucose positron emission tomography-computed tomography (18F-FDG PET-CT) in PH and exploring the application value of PET-CT in the diagnosis of PH. DATA AND METHODS: Patients diagnosed with PH who had undergone PET-CT from literature pertaining were retrospectively analyzed, which were cases of publications from the Cochrane Library, PubMed, Excerpta Medica Database (EMBASE), China National Knowledge Infrastructure (CNKI) and Wanfang databases, from 2008 to June 2022. The other 20 cases of the collection were patients from our hospital from 2008 to June 2022. Patients' symptoms, imaging characteristics of chest CT, PET-CT characteristics, the reason for PET-CT and the complications were analyzed. RESULTS: In this retrospective study, a total of 216 patients were diagnosed with PH and had been examined by PET-CT. 20 of the cases were patients of our hospital from January 2008 to June 2022. The other cases were collected from the literature. The mean diameter of most PH lesions is 1.7 ± 1.0 cm. The mean maximum standardized uptake value (SUVmax) of the PH lesions was 1.2 ± 1.1. Most of their SUVmax were lower than internationally recognized cut-off value (SUVmax = 2.5). PET-CT was superior to CT in the diagnosis of PH but there was a correlation of between CT diagnosis and PET-CT diagnosis for the PH lesions. In order to draw the Receiver operating characteristic (ROC), we selected 29 patients with a clear SUVmax value of their PH lesion, and 29 lung cancer patients with clear SUVmax value in our hospital were collected as a control group. ROC curve analysis showed that the area under curve (AUC) of SUVmax was 0.899, and the optimal diagnostic threshold was SUVmax > 2.65. PET-CT could distinguish PH from malignant lesions with a sensitivity of 89.66% by applying a SUVmax of 2.65 as a cut-off in this study. CONCLUSION: PET-CT might be a useful tool to diagnose PH, which shows a better diagnostic sensitivity than CT. But PET-CT can not be used as a single diagnostic approach, which should be combined with other methods and the patients' history to make the most correct diagnosis.

Pulmonary leiomyosarcoma arising in pulmonary hamartoma: an exceptional occurrence in a rare tumor.

A solitary peripheral lung nodule was found in the left lung of a 52-year-old man. It was located in the lower lobe and measured 18.5 cm of major axis on chest computed tomography. A tru-cut core biopsy was obtained and a proliferation of bland, monomorphic, spindle cells in interlacing fascicles was observed. Accordingly, a surgical resection of the neoplasm was subsequently carried out. Macroscopically, the tumor appeared as a well-circumscribed nodule with a firm and whitish cut surface. Histologically, the neoplasm was predominantly composed of bland and monomorphic spindle cells, with a predominantly fascicular growth pattern, in which many tubular and cleft-like spaces of entrapped normal respiratory epithelium were involved. Myxoid change, stromal hyalinization and scattered bizarre mononucleated and multinucleated cells were also observed. Based on clinico-morphological, immunophenotypical and molecular features, we made a diagnosis of malignant transformation of pulmonary adenoleiomyomatous hamartoma into pulmonary leiomyosarcoma. As far as we know, this is the first described case of this exceptionally rare occurrence in an already rare neoplasm.

Lung hamartoma resembling lung cancer: a report of three cases.

Pulmonary hamartoma is a benign lung tumor. However, it is difficult to distinguish this lesion from other diseases via imaging. Three patients with pathologically confirmed pulmonary hamartoma in our department were analyzed. We believe it is necessary to combine imaging, pathology, clinical testing, and individual patient assessments to enable an earlier and more definitive diagnosis of pulmonary hamartoma. Therefore, it is necessary to analyze and summarize the clinical manifestations and imaging features of patients with pulmonary hamartoma to improve the early recognition of the disease by clinicians.

Differential diagnostic value of 18F-FDG PET/CT in pulmonary carcinoids versus hamartomas.

RATIONALE AND OBJECTIVE: To assess the possibility of differentiating pulmonary carcinoids from hamartomas and typical from atypical carcinoids by means of 18F-FDG PET/CT. MATERIALS AND METHODS: We retrospectively reviewed 139 patients with pathologically proven pulmonary carcinoids and hamartomas who underwent 18F-FDG PET/CT before surgical resection. Receiver operating characteristics curves were calculated to determine the potential of SUVmax to discriminate between pulmonary carcinoids and hamartomas, typical and atypical carcinoids. The correlation between SUVmax and tumor size was analyzed by Spearman correlation analysis. RESULTS: SUVmax was significantly higher in pulmonary carcinoids than in hamartomas (p<0.001), and also higher in atypical carcinoids than in typical carcinoids (p = 0.034). With a SUVmax of 2.0 as a cutoff, the sensitivity, specificity, positive predictive value, and negative predictive value for 18F-FDG PET/CT to differentiate pulmonary carcinoids from hamartomas were 85.3%, 82.9%, 61.7%, and 94.6%, respectively. The cutoff value of SUVmax for differentiating atypical carcinoids from typical carcinoids was 4.1. The area under the receiver operating characteristics curve of SUVmax was 0.900 for carcinoids and hamartomas, and 0.722 for typical and atypical carcinoids. SUVmax was correlated with maximum tumor size in pulmonary carcinoids (r = 0.658, p <0.001) and in pulmonary hamartomas (r = 0.672, p <0.001). CONCLUSION: 18F-FDG PET/CT might be a useful tool in the differential diagnosis of carcinoids and hamartomas, and can also distinguish atypical from typical carcinoids. This may facilitate improved selection of patients for surgical resection and radiological follow-up.

Tracheal Hamartoma: A Case Report.

Hamartoma is the most frequently observed benign lung tumor, but its tracheal form is still exceptionally encountered. Cough, dyspnea, hemoptysis, and chest pain are all possible symptoms of tracheal hamartoma. The non-specific symptoms may also lead to a delayed diagnosis, and while the choice of treatment varies depending on the size and location of the lesion, conservative treatments remain strongly recommended. This report presents the case of a 57-year-old male who presented to our department with inspiratory dyspnea. Clinico-radiological data and bronchoscopy revealed a benign tracheal tumor of the lipomatous hamartoma type. The patient underwent a tumor resection by rigid bronchoscopy with satisfactory clinical results.

An unusual finding of tumorlet carcinoid and endobronchial hamartoma in the lobar bronchus / Hallazgo inusual de tumorlet carcinoide y hamartoma endobronquial en el bronquio lobar

SUMMARY: Neuroendocrine lung tumours are a group of different tumours that have similar morphological, immunohistochemical and molecular characteristics, and represents 1-2 % of all malignant lung tumours. Tumorlet carcinoids represent the nodular proliferation of hyperplastic neuroendocrine cells that is smaller than 5 mm in size. In this study, we reported the unusual finding of tumour carcinoid and endobronchial hamartoma in the same bronchus. A 49-year-old male patient with symptoms of prolonged severe cough and fever, and was treated for pneumonia. Since he did not adequately respond to antibiotic therapy lung CT scan was performed which showed middle lobe bronchus obstruction. Bronchoscopy revealed a lobulated whitish tumour which was biopsied and histopathological diagnosis was hamartoma. Tumour could not be completely removed during bronchoscopy, it was decided to surgically remove it. On serial section, during gross examination in the same bronchus, an oval yellowish area with a diameter of 3 mm was found along the bronchial wall. According to gross and histomorphological characteristics and immunophenotype of tumour cells, the diagnosis of tumour carcinoid was set. Diagnosis of carcinoids of the tumorlet type is usually an accidental finding and it can be seen on CT in the form of subcentimeter, single or multiple, nodular changes. Considering that the clinical picture is nonspecific, they should always be kept in mind as a possible differential diagnosis.

RESUMEN: Los tumores neuroendocrinos de pulmón son un grupo de tumores de diferentes características morfológicas, inmunohistoquímicas y moleculares similares, y representan el 1- 2 % de todos los tumores malignos de pulmón. Los carcinoides tumorales representan la proliferación nodular de células neuroendocrinas hiperplásicas de tamaño inferior a 5 mm. En este estudio reportamos el hallazgo inusual de tumor carcinoide y hamartoma endobronquial en el mismo bronquio. Un paciente varón de 49 años con síntomas de tos severa prolongada y fiebre fue tratado por neumonía. Al no responder adecuadamente a la terapia con antibióticos, se realizó una tomografía computarizada de pulmón que mostró obstrucción del bronquio del lóbulo medio. La broncoscopia reveló una tumoración blanquecina lobulada de la cual se tomó biopsia y el diagnóstico histopatológico fue hamartoma. No fue posible extirpar el tumor por completo durante la broncoscopia y se decidió extirparlo quirúrgicamente. En la sección seriada, durante el examen macroscópico en el mismo bronquio, se encontró un área amarillenta ovalada de 3 mm de diámetro a lo largo de la pared bronquial. De acuerdo a las características macroscópicas e histomorfológicas y de inmunofenotipo de las células tumorales, se estableció el diagnóstico de tumor carcinoide. El diagnóstico de carcinoides de tipo tumorlet suele ser un hallazgo accidental y se observan en la TC en forma de cambios nodulares subcentimétricos, únicos o múltiples. En consideración de que el cuadro clínico es inespecífico, siempre debe tenerse en cuenta como posible diagnóstico diferencial.

Giant pulmonary hamartoma with placental transmogrification-a case report.

Pulmonary hamartomas are rare tumors and are mostly found incidentally in patients investigated for other pathologies. They are frequently small in size, though with some being reported as large as 25 centimeters (cm). We are reporting a case of a huge pulmonary hamartoma (measuring 25.5 × 17.5 × 15.5 cm and weighing about 2200 grams (g)) in a 33-year-old lady with a short duration history of breathlessness. The tumor had origin from the medial border of the left lung, extending into almost the entire left hemithorax and partly into the anterior mediastinum, with no local invasion. The left lower lobe had compression atelectasis due to mass. The mass was successfully resected. The histopathology report showed predominant adipose tissue and cartilaginous differentiation. There were also a few foci of papillary projections with predominant vascular areas, resembling immature placental villi, suggestive of placental transmogrification of the mass.

Does Pulmonary Hamartoma Increase the Risk of Lung Cancer? Outcomes of 38 Pulmonary Hamartoma Cases.

OBJECTIVES: Hamartomas are common benign tumors of the lung. Rarely, lung cancer coincidence may occur at the time of diagnosis or in the follow-up period. METHODS: Between 2016 and 2019, 38 patients who underwent a surgical procedure and diagnosed with lung hamartoma were retrospectively evaluated regarding clinicopathological features. Cases were analyzed according to age, sex, radiological findings, localization of nodules, surgical methods, and the coincidence of lung cancer. RESULTS: The mean age was 50.2±11.1 (range 28-76 years). There were 23 male (60.5%) and 15 female (39.5%) patients. Mean size was 2.7±1.8 (range 0.8-10 cm). In 28 patients, hamartoma was <3 cm in diameter (73.6%). Eighteen hamartomas were localized in the upper lobe (47.4%). Only 6 cases (15.8%) were localized at the central part of the lung. Multiple nodules were reported in 10 cases (26.3%). In 4 cases (10.5%), lung carcinoma and hamartoma were seen together at the time of diagnosis. Video-assisted thoracoscopic surgery (VATS) has been performed in 29 cases (76.3%). As a surgical method, enucleation was performed in 4 cases (10.5%), wedge resection in 28 cases (73.7%), and lobectomy in 6 cases (15.8%). No post-operative mortality appeared in the early follow-up. CONCLUSION: Pulmonary hamartomas are usually present as solitary pulmonary nodules with benign radiological findings. VATS wedge resection is a method that can be used safely in diagnosis and treatment. Hamartomas may be associated with lung cancer at the time of diagnosis or follow-up, so it should be kept in mind that a different nodule seen in patients diagnosed with hamartoma may be associated with lung cancer.

Symptomatic Pulmonary Hamartoma.

Pulmonary hamartoma is the most common benign tumor of the lungs. It is most often asymptomatic and is discovered incidentally. The condition is two to three times more common in men and is usually seen in the sixth to seventh decade of life. The authors present the case of a 44-year-old female in whom the condition was symptomatic, causing cough, shortness of breath, and fatigue. This case is unusual in that it occurred in a woman in her 40s and was symptomatic. The authors discuss the presentation, clinical features, and management of pulmonary hamartoma.

Pulmonary Adenofibroma: Clinicopathological and Genetic Analysis of 7 Cases With Literature Review.

INTRODUCTION: Pulmonary adenofibroma (PAF), characterized by biphasic differentiation composed of gland-like space lined by respiratory epithelium and stromal spindle cells, is a rare benign tumor of the lung. PAF was reported infrequently and inconsistently with diagnostic criteria and withstood higher risk of misdiagnosis as solitary fibrous tumors (SFTs) due to their morphological resemblance. In this study, we report seven cases of PAF with gene sequencing results and summarize the data of previous literature. MATERIALS AND METHODS: Seven cases of PAF with surgically resection samples were collected from Pathology department of West China Hospital, Sichuan University between 2009 to 2020. Immunohistochemical studies were performed in all cases and 3 cases underwent a 425-gene panel next-generation sequencing (NGS). RESULTS: Five female and two male patients were included in this study, with an average age of 51 years. All the patients were asymptomatic, and the lesion was identified on routine chest radiography. The tumor size measured by computed tomography (CT) ranged from 0.5 to 2.7 cm. Gland-like structures were mostly positive for glandular epithelium markers. The spindle cells in stroma expressed Desmin, SMA, ER and PR in 3 of 7 cases. No well-recognized molecular abnormalities can be identified by NGS in the 3 cases. To date, all the patients are alive, with no evidence of recurrence and metastasis. CONCLUSION: PAF is a unique benign pulmonary tumor with low incidence. Biphasic morphology, IHC stains along with molecular detection is of great significance to make a clear diagnosis.

Contribution of 18F-FDG PET/CT in the Differential Diagnosis of Pulmonary Hamartomas and Pulmonary Carcinoids

Objectives: This study aimed to evaluate 18fluorine-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) findings in the differential diagnosis of pulmonary carcinoids and pulmonary hamartomas. Methods: 18F-FDG PET/CT findings of 34 patients with pulmonary carcinoids (12 atypical, 22 typical) and 32 patients with pulmonary hamartomas were retrospectively evaluated. Both mean diameter and mean maximum standardized uptake value (SUVmax) of hamartomas and carcinoids were compared by Mann-Whitney U and Kruskall-Wallis H tests. Results: The mean longest diameter of atypical carcinoids (3.5±1.7 cm) was higher than that of hamartomas (2.1±1 cm) (p=0.038). No significant difference was found between the mean diameter of typical carcinoids and mean diameter of hamartomas (p=0.128). The mean SUVmax of atypical carcinoids (5.97±3.7) and typical carcinoids (4.22±1.7) were higher than those of hamartomas (1.65±0.9) (p=0.002 and p=0.003, respectively). There were collapse/consolidation in 55.8%, bronchiectasis or mucoid impaction in 47%, and air trapping in 14.7% in the peripheral parenchyma of the 34 carcinoids. Collapse/consolidation was detected in a patient with endobronchial hamartoma, and other finding was not found in the parenchyma around hamartomas. Conclusion: The 18F-FDG uptake of pulmonary carcinoids can vary from minimal to intense. 18F-FDG uptake can be seen in pulmonary hamartomas. However, the mean SUVmax of atypical carcinoids and typical carcinoids were higher compared to hamartomas. Pulmonary carcinoid must be suspected in cases with accompanying bronchial obstruction findings in the periphery of the mass.

Interlobar Pulmonary Hamartoma With an Unusual Neighboring Lesion: A Case Report.

Pulmonary hamartomas are benign lung tumors. They are uncommon and represent a small percentage of all solitary lung lesions. Hamartomas are composed of an abnormal mixture of epithelial and mesenchymal elements. They are usually discovered incidentally as patients are asymptomatic in most cases. On the other hand, sclerosing pneumocytomas are rarely discovered and are one of the most uncommon benign lung tumors. Herein, we present the case of a 37-year-old female who presented with hemoptysis. A computed tomography scan of her thorax revealed a right-sided round lesion in the interlobar fissure with no additional findings. Surgical excision was performed, which demonstrated an uncommon and unique finding of a pulmonary hamartoma coexisting with a sclerosing pneumocytoma found on exploration. This highlights the importance of exploration during surgical procedures in order identify missed lesions on imaging.

Pulmonary hamartoma: Feasibility of dual-energy CT detection of intranodular fat.

We have reported 2 cases of pulmonary hamartoma focusing on detecting intranodular fat, which is one of CT features suggestive of pulmonary hamartoma, using dual-energy CT analyses. For patient 1, a 73-year-old man was pointed out to have a nodular opacity on chest radiograph of pretreatment workup for retinal detachment. In patient 2, a 66-year-old woman with uterine carcinoma admitted for preoperative assessment. Both patients underwent dual-energy CT examination and the pulmonary lesions exhibited a downward-sloping curve at lower X-ray energies on attenuation curve of virtual monochromatic images, which suggested fatty tissue. Dual-energy CT analysis can help diagnose pulmonary hamartoma with detection of intralesional fatty tissue.

A pulmonary hamartoma in Covid-19 pneumonia: an interesting case studied with computed tomography.

Since the widespread of acute respiratory syndrome infection caused by Coronavirus-19, chest computed tomography (CT) was considered a useful imaging tool commonly used in early diagnosis and monitoring of patients with complicated Covid-19 pneumonia. Many typical imaging features of this disease were carefully described with chest CT, as well as the collateral CT findings in the lungs and mediastinum. Here we describe the case of a patient with Covid-19 pneumonia, that collaterally had a pulmonary hamartoma in the left lung, documented at CT.