Incidental Finding of PSMA-avid Pulmonary Sclerosing Pneumocytoma on 68Ga PSMA PET-CT.

Background: Rare pulmonary tumors have uncommon histology. Overall, these tumors account for less than 1 % of all lung primary tumors. Aim: To review clinic-pathologic, radiological and therapeutic features of these tumors. Methods: A retrospective analysis was designed in a pulmonary department in Tunis during 13 years period. Patients with histological diagnosis of rare primary lung tumor were included. Tumors were classified according to the World Health Organization classification of 2014. Staging was made according to 2009 TNM classification. Clinical, radiological and histo-pathologic data were analyzed. Metastatic lung tumors were excluded. Results: Among 1742 patients with a primary lung malignancy, 10 (6 men, 4 women) patients had a rare lung tumor (0.5%). The mean age was 59 years [19-82]. The main respiratory symptoms were hemoptysis, dyspnea and chest pain. CT scan findings consisted of a huge mass with obstructive pneumonitis in 7cases. Local invasion was noted in 4cases. Diagnosis was obtained by bronchial biopsy (3cases), CT guided biopsy (2cases), surgical resection (4cases) and lymph node biopsy (1case). Histological types were sarcomatoid carcinoma (4cases), sarcoma (2cases) and carcinoid tumor (4cases). At diagnosis, 5 patients had a metastatic disease. Pulmonary resection with lymph node dissection was performed in 4 cases. Chemotherapy was prescribed in 5 patients.Four patients died, the remaining patients are still followed up. Conclusion: In addition to a low incidence, rare pulmonary tumors are usually characterized by the absence of clinical and radiological updated features which makes their recognition difficult.Prognosis depends on histological type.

In a review of pulmonary tumors diagnosed at our institute from 1976 to 1995, we found 20 malignant and 12 benign rare tumors, which accounted for 0.57 and 0.34% of all pulmonary tumors, respectively. The histological types of these rare malignant tumors were malignant lymphoma (6/20), carcinosarcoma (3/20), mucoepidermoid carcinoma (2/20), bronchial gland mixed tumor (2/20), adenocystic carcinoma (1/20), myoepithelioma (1/20), leiomyosarcoma (1/20), epitheloid hemangioendothelioma (1/20), hemangiopericytoma (1/20), malignant melanoma (1/20) and choriocarcinoma (1/20). Benign rare tumors involved papilloma (3/12), lipoma (3/12), leiomyoma (3/12), adenoma (1/12), fibroma (1/12), and meningioma (1/12). The clinical and pathological features of malignant tumors were roughly the same as those of common pulmonary carcinomas. In contrast, benign tumors were never larger than 3 cm and were more commonly located in the central parts of the lung, which explained the relatively frequent symptoms of wheezing and fever associated with obstructive pneumonia.

Tumeurs thoraciques rares

Cytomorphology of giant cell tumor of bone in pleural fluid

Basaloid squamous cell carcinoma (BSCC) of the lung is a rare pulmonary neoplasm. Its histopathological features fulfill those of squamous cell carcinoma and basaloid carcinoma with its prominent peripheral palisading of nuclei at the edge of the tumor cell nests. BSCC is a high-grade malignancy and histologically characterized by a high rate of spontaneous apoptosis and high degree of proliferative activity. Herein, we present a case of pulmonary BSCC in the superior segment of the right lower lobe of the lung, which was treated with a right lower lobe lobectomy with radical lymph node dissection. The histological tumor stage was llla (T2N2MO). The patient refused postoperative chemotherapy. Unfortunately, 2 metastatic hepatic nodules developed 6 months after primary surgical treatment. Combination chemotherapy with navelbine and cisplatin, accompanied with percutaneous ultrasound-guided radiofrequency ablation (PUG-RFA) of the two hepatic nodules, was performed with a complete response. However, the patient refused further chemotherapy, and multiple liver metastases occurred again. The patient died of carcinomatosis and hepatic failure 18 months after surgery. This unique histopathological tumor of the lung, BSCC, was associated with a more aggressive disease progression, compared with typical squamous cell lung cancers. Surgical resection combined with adjuvant chemotherapy might be the treatment of choice for such a rare pulmonary neoplasm.

BackgroundPulmonary sclerosing pneumocytoma is a kind of rare benign pulmonary tumor with potential malignancy. The clinical features, risk factors for prognosis, and optimal treatment have not been identified yet. This study aimed to investigate the clinical features and prognosis of pulmonary sclerosing pneumocytoma.MethodsWe retrospectively performed a review of pulmonary sclerosing pneumocytoma patients in West China Hospital from 2009 to 2019. The basic characteristics, treatment regimens, operation detail, postoperative variables, and follow-up time were recorded for each case. Differences in features between patients undergoing lobectomy and segmentectomy were compared. We also performed a case review and summarized reported clinical features in former studies.ResultsAltogether 61 pulmonary sclerosing pneumocytoma patients were retrospectively reviewed. Fifty-six patients were female and 5 were male. The patients’ median age was 51 (23-73). Seven (11.48%) patients had smoking history. Twenty tumors were located in the right lung [upper lobe (n = 7), middle (n = 2), and lower (n = 11)] and 41 in the left [upper (n = 12) and lower (n = 29)]. The median tumor size was 2 (0.9-7) cm. Thirty-six (59.02%) patients underwent sublobectomy (segmentectomy or wedge resection) whereas 25 (40.98%) underwent lobectomy. All patients recovered uneventfully, and no perioperative mortality was identified. Sublobectomy showed a trend towards reduced chest tube duration and shorter postoperative hospital stays compared with lobectomy.ConclusionsThe findings showed good prognosis of pulmonary sclerosing pneumocytoma and proved its benign characteristics. Sublobectomy showed advanced efficacy regarding chest tube duration and postoperative hospital stay compared with lobectomy.

Rare pulmonary tumors (RPT) pose challenges for diagnosis, treatments and clinical decision making. Information about them is scant. Aim. To supplement information on diagnosis and surgical treatment of the RPT. Methods. A retrospective study of 499 lung resections for primary tumors, performed in St. Petersburg Federal hospital 122 in 2010-2019 was done. Every specimen has been investigated by immunostaining. Selection criteria for RPT: the RARECARE project principles (the incidence less than 6/100000 new cases per year), WHO Classification of Lung Tumors (2015). Results. After morphological investigation, 89/499 (17.84%) patients with RPT were diagnosed. There were 45 male, 44 female; mean age 54.02±14.7 years. According to ICD-O classification 74 tumors (83.1%) were malignant (/3); 9 (10.1%) – uncertain (/1); 6 (6.8%) tumors were benign (/0). Epithelial tumors comprise 79.8% (71 cases) with domination of atypical carcinoid (33); 15 tumors were mesenchymal (16.8%) with 6 myofibroblastic and 3 – primary pulmonary lymphomas. A correct preoperative diagnosis received in 22 (24.7%) patients. 66 (85.4%) patients underwent anatomical pulmonary resections, and the rest wedge resections performed for carcinoid tumors and low grade sarcomas. 77 (86.5%) procedures was done by VATS. All resections were R0. A frozen section was incorrect as well in 4 out of 7 cases, which required staged reVATS to expand the volume of surgery in 1 case. Conclusion. The study demonstrates a high incidence of RPT and complexity of its preoperative diagnosis. VATS resection is a good diagnostic and treatment method, especially then R0 resection is reached. For ICD-O /3 RPT the anatomical pulmonary resection with lymphadenectomy is recommended.

BackgroundPulmonary granular cell tumor (PGCT) is a rare neoplastic disease. We assessed the clinicopathological characteristics and gene analysis of PGCT with a systematic review of literature.MethodsWe studied the clinical presentation, radiological image, pathological features, treatment option and follow-up of three cases of PGCT, in whom two had next generation sequencing (NGS) performed for identification of genetic mutation. We also systematically analyzed 78 cases from the literature that met inclusion criteria.ResultsThree cases of PGCT (two benign cases and one malignant case) with clinical, radiological, pathological and therapeutic information are presented. There were 81 cases in total enrolled in this study for review. PGCT occurred predominantly in women (63.0%). Fifty-five patients (67.9%) were symptomatic with cough, dyspnea and chest pain being the most common symptoms. The bronchus was the most common site of PGCT (46.9%). The majority of patients were benign PGCT, apart from seven patients with malignant PGCT (8.6%). Forty-one patients received surgical treatment, 12 received bronchoscopic treatment and 28 patients had no relevant information. Forty-two patients had no recurrence at the end of follow-up, 7 patients had residual disease and 8 patients died (non-PGCT related) during the follow-up. The NGS test of the malignant patient showed a significant increase in WRN, KMT2A, RPA1, NSD1, DDR2, ZNRF3, NOTCH4, CSF1R, FAT3, GRIN2A and RAD50 gene expression, while the benign patient showed no difference in gene data.ConclusionsPGCT is a rare pulmonary tumor with no specific features. Lung biopsy through surgery or bronchoscopy is the key for the diagnosis of PGCT. Gene sequencing of the malignant case revealed abnormal expression of genes with unclear significance via mechanisms that need to be further explored.

Pulmonary sclerosing pneumocytoma (PSP) is a rare pulmonary tumor that behaves in a benign fashion and has excellent prognosis.[1] The tumor was historically termed as sclerosing hemangioma by Liebow and Hubbell in 1956.[2] The incidence peaks in sixth decade with a striking predilection for females (F:M = 5:1).[3] [4] Although it presents as a well-circumscribed solid nodular mass, the radiological features are nonspecific and can be misconstrued as malignant especially in large tumors.[5] Frozen sections and trucut biopsy interpretation of such tumors may also be misleading.

The Papanicolaou Society of Cytopathology recommendations for respiratory cytology utilize a neoplastic category that contains both clearly benign neoplasms and neoplasms with a low but undetermined malignant potential. Assignment to the neoplastic category requires that sufficient morphologic features are present to definitively define a smear as representative of a specific benign neoplasm. Neoplasms with low malignant potential and some low-grade carcinomas overlap morphologically and are cytologically difficult to separate. Clearly malignant neoplasms including a variety of well-differentiated adenocarcinomas and low-grade sarcomas morphologically overlap entities with low malignant potential. Thus, separation of the neoplastic category into two subcategories has importance for cytologic evaluation, differential diagnosis, and assessment of malignancy risk. This subdivision of the neoplastic category into those lesions which are clearly benign and those that have a low risk for recurrence or metastasis is clinically useful. This categorization allows the treating physician discretion in his or her therapeutic approach to a patient. Lesions of low or undetermined malignant risk in a young, healthy individual are often resected, while a similar neoplasm in an elderly infirm individual may receive only observational or medical therapy.

Laparoscopy has been accepted for years as a management of benign ovarian tumors. The aim of this study was to estimate the feasibility and safety of laparoscopy in diagnosis and management of adnexal masses. A total of 2083 patients with benign adnexal mass were treated by laparoscopy at Peking Union Medical College Hospital from January 2000 to December 2003. Their clinical data were reviewed retrospectively. All the adnexal masses suspicious of malignancy at the time of laparoscopy were sent for frozen section evaluation intraoperatively. The rates of unexpected intracystic vegetation and low malignant potential (LMP) tumor or malignancy were investigated. The sensitivity, specificity, positive predictive value, and negative predictive value of laparoscopic diagnosis for LMP or ovarian malignancies were calculated. The ratios were compared by Chi-square test and the continuous variables were tested using two-tailed t test. Of the 2083 patients, 16 had LMP or invasive tumors (0.77%), among which 14 were diagnosed histologically intraoperatively and 2 postoperatively. Fifty-five (2.6%) of the 2083 patients had unexpected intracystic vegetations. Their frozen sections showed benign tumors in 41 (74.5%), LMP tumors in 8 (14.5%), and focal invasive ovarian cancers (stage Ic) in 6 (10.9%). The final pathological diagnosis were benign tumors in 41 (74.5%), LMP tumors 7 (12.7%), and focal invasive ovarian cancers (stage Ic) in 7 (12.7%). Laparoscopy achieved a sensitivity of 87.5%, specificity of 98%, positive predictive value of 25.5%, and negative predictive value of 99.9% in the diagnosis of ovarian malignancies. 2067 cases with benign adnexal masses underwent laparoscopy successfully. No conversion to laparotomy, or intra- and postoperative complications in this series. Of the 16 patients with LMP or invasive ovarian cancer, seven underwent laparoscopic surgery including immediate staging laparoscopy in 3. The mean follow-up was 17.3 months for the 16 patients. Among them, 1 developed a recurrent LMP tumor in the contralateral ovary 36 months after laparoscopic salpingo-oophorectomy, and received subsequent laparoscopic cystectomy and pelvic lymph node sampling; the others had no evidence of recurrent tumor during the follow-up. Laparoscopy is feasible for diagnosis of adnexal masses, and the surgery is safe for patients with benign ovarian tumors.

Ovarian carcinomas exhibit extensive heterogeneity, and their etiology remains unknown. Histological and genetic evidence has led to the proposal that low grade ovarian serous carcinomas (LGOSC) have a different etiology than high grade carcinomas (HGOSC), arising from serous tumours of low malignant potential (LMP). Common regions of chromosome (chr) 3 loss have been observed in all types of serous ovarian tumours, including benign, suggesting that these regions contain genes important in the development of all ovarian serous carcinomas. A high-density genome-wide genotyping bead array technology, which assayed >600,000 markers, was applied to a panel of serous benign and LMP tumours and a small set of LGOSC, to characterize somatic events associated with the most indolent forms of ovarian disease. The genomic patterns inferred were related to TP53, KRAS and BRAF mutations. An increasing frequency of genomic anomalies was observed with pathology of disease: 3/22 (13.6%) benign cases, 40/53 (75.5%) LMP cases and 10/11 (90.9%) LGOSC cases. Low frequencies of chr3 anomalies occurred in all tumour types. Runs of homozygosity were most commonly observed on chr3, with the 3p12-p11 candidate tumour suppressor region the most frequently homozygous region in the genome. An LMP harboured a homozygous deletion on chr6 which created a GOPC-ROS1 fusion gene, previously reported as oncogenic in other cancer types. Somatic TP53, KRAS and BRAF mutations were not observed in benign tumours. KRAS-mutation positive LMP cases displayed significantly more chromosomal aberrations than BRAF-mutation positive or KRAS and BRAF mutation negative cases. Gain of 12p, which harbours the KRAS gene, was particularly evident. A pathology review reclassified all TP53-mutation positive LGOSC cases, some of which acquired a HGOSC status. Taken together, our results support the view that LGOSC could arise from serous benign and LMP tumours, but does not exclude the possibility that HGOSC may derive from LMP tumours.

A Primary Pulmonary Glomus Tumor Complicated With Hyperpyrexia and Anemia

Accessory Lobe of Right Liver Mimicking a Pulmonary Tumor in an Adult Male

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