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Inflammatory pseudotumor (plasma cell granuloma) of the lung is an uncommon benign, slowly growing lesion of unknown origin. 

As the origin and pathology of inflammatory pseudotumor is not clearly understood, there has recently been some confusion regarding the diagnosis of inflammatory pseudotumor.

Because it is difficult to make a preoperative diagnosis of inflammatory pseudotumor by radiological findings or histological findings using specimens obtained by transbronchial lung biopsy, surgical resection, such as video-assisted thoracic surgery or open lung biopsy, is required to differentiate inflammatory pseudotumors from primary or metastatic lung cancers.

Although inflammatory pseudotumors constitute less than 1% of all lung tumors, they are reported to be the most common cause of solitary lung masses in children. 

Patients with inflammatory pseudotumor are usually asymptomatic. It can behave as a malignant tumor both clinically and radiologically.

The tumour can present with symptoms secondary to obstruction when arising in a endobronchial location.

This tumour typically manifests as a solitary, peripheral, and sharply circumscribed mass. Multiple lesions are seen in about 5% of cases.

Cough, fever, dyspnea, and hemoptysis are the usual presenting symptoms.

Histologically the two main variants are:   

- Plasma cell granuloma variant:  Characterized by hyalinized fibroconnective tissue, sheets of plasma cells together with plump fibroblasts, histiocytes, mast cells and lymphocytes.

- Fibrohistiocytic variant: Characterized by proliferation of spindle cells arranged in whorled or storiform pattern. (Differential diagnosis: Sarcoma).

Microscopic Image of Inflammatory Pseudotumour of the Lung: click here

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Mitotic figures may be present. No abnormal mitotic figures are present.

Complete surgical excision is the treatment of choice and should be performed as early as possible. Relapse after incomplete resection and recurrence of aggressive forms have been described.

It has been suggested that probably these lesions are neoplastic and related to inflammatory myofibroblastic tumours located at other sites.

                 

Inflammatory pseudotumor of the lung. Case report. Rev Port Cir Cardiotorac Vasc. 2007 January-March;14(1):21-25

Inflammatory pseudotumor of the lung is a rare entity, of unknown etiology and variable clinical evolution. The histological variety of this entity makes the diagnosis difficult, which is generally obtained after surgical removal of the lesion. The authors report the clinical case of a 32 years old woman presenting with hemoptysis and radiologic appearance of aspergilloma. The lesion was surgically removed and the diagnosis of inflammatory pseudotumor of the lung was confirmed by pathologic and immunohistochemical analysis.

Inflammatory pseudotumor of the lung: clinicopathological analysis in seven adult patients. Int J Clin Oncol. 2006 Dec;11(6):461-6. 

BACKGROUND: As the origin and pathology of inflammatory pseudotumor is not clearly understood, there has recently been some confusion regarding the diagnosis of inflammatory pseudotumor. METHODS: We performed clinicopathological analyses in seven patients with inflammatory pseudotumors of the lung histologically diagnosed after surgical resection. The seven patients, selected from patients admitted to Kawasaki Medical School Hospital between April 1989 and December 2005, consisted of four men and three women (average age, 60.4 years). The detection method was from clinical symptoms in two patients and from health examinations in five patients. RESULTS: Five patients had a solitary nodular shadow on chest radiographs and the other two patients had multiple shadows. Because it was difficult to distinguish pseudotumor from lung cancer by chest computed tomography in the five patients showing a solitary nodular shadow, the final diagnosis was obtained by surgical resection. In the other two patients, with multiple shadows, one of whom had nodular shadows with cavitary lesions, surgical resection was performed to distinguish the pseudotumor from metastatic lung cancer. The histological types according to the criteria of Matsubara were fibrous histiocytic in five patients, organizing pneumonia in one, and lymphoplasmocytic in one. The histological types according to the World Health Organization criteria were compact spindle-cell pattern in six patients and hypocellular fibrous pattern in one. CONCLUSION: Because it is difficult to make a preoperative diagnosis of inflammatory pseudotumor by radiological findings or histological findings using specimens obtained by transbronchial lung biopsy, surgical resection, such as video-assisted thoracic surgery or open lung biopsy, is required to differentiate inflammatory pseudotumors from primary or metastatic lung cancers.

Plasma cell granuloma associated with pulmonary actinomycosis: a case report.J Korean Med Sci. 2006 Dec;21(6):1117-20.

Plasma cell granuloma (PCG) of the lung is a rare disease that usually presents as a pulmonary nodule or mass on incidental radiographic examination without symptoms. Although the etiology of PCG is still controversial, many findings have lent support to the lesion being a reactive inflammatory process rather than a neoplastic one. We describe a 53-yr-old male who presented with a hemoptysis and have a lung mass at the left upper lobe on chest radiograph. The lung mass was primarily diagnosed as PCG by percutaneous needle aspiration and biopsy, and the patient was treated with oral steroid because he and relatives refused the operation. However, the size of the lung mass did not change and open thoracotomy and lobectomy were done therefore. He was confirmed as having pulmonary actinomycosis with PCG after surgery. To our knowledge, this is the first report of PCG associated with actinomycosis in Korea.

Pulmonary inflammatory pseudotumor: difficulties in diagnosis and prognosis.Tunis Med. 2006 Mar;84(3):205-8.

Inflammatory pseudotumors are uncommon benign lesions of the lung of unknown origin, that may show aggressive behavior. Although pseudotumors constitute less than 1% of all lung tumors, they are reported to be the most common cause of solitary lung masses in children. Complete resection, when possible, is safe and leads to excellent survival. The case presented here highlights difficulties in clinical and histologic diagnosis.

Inflammatory pseudotumor: a rare cause of proximal bronchial obstruction. Rev Pneumol Clin. 2006 Jun;62(3):183-6.

Inflammatory pseudotumor of the lung is a rare disease with usual benign course. Relapse after incomplete resection and aggressive forms have already been described. We report the case of a 67-year-old woman with atelectasis of the left lung related to an endobronchial inflammatory pseudotumor. After laser resection, relapse occurred 3 months later, requiring surgical treatment.

A case of pulmonary inflammatory pseudotumor: Recurrence appearing as several consolidative lesions after complete resection.Korean J Intern Med. 2005 Jun;20(2):168-72.

Inflammatory pseudotumor (plasma cell granuloma) of the lung is an uncommon nonneoplastic tumor of unknown origin. This tumor typically manifests as a solitary, peripheral, and sharply circumscribed mass. Multiple lesions are seen in about 5% of cases. Resection is recommended for both diagnosis and treatment, and this tumor does not generally recur after complete resection. Here, we report a case of recurrent inflammatory pseudotumor after complete resection; the recurrence was detected as a series of bilateral consolidated lesions with an internal air bronchogram. This is an unusual finding with regard to inflammatory pseudotumors.

IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung.Hum Pathol. 2005 Jul;36(7):710-7.

The association between IgG4 dysregulation and inflammatory pseudotumor (IPT) was first reported in sclerosing pancreatitis. Recently, we described IPTs of the liver and breast, into both of which many IgG4-positive plasma cells had infiltrated. In this study, we examined the clinical and histological features of 9 cases of IPT (histologically corresponding to plasma cell granuloma) of the lung with an emphasis on IgG4-positive plasma cell infiltration. The lesions were characterized histologically by dense lymphoplasmacytic infiltrates intermixed with fibrosis and, in some cases, prominent eosinophilic infiltration, irregular narrowing of bronchioles entrapped in nodules, and an interstitial pneumonia pattern at the boundaries of nodules. Obliterative phlebitis was easily found in all cases, and 5 lesions also had obliterative arteritis. Immunostaining revealed many IgG4-positive plasma cells diffusely distributed within nodules, and the ratios of IgG4-positive to other plasma cells were extraordinarily high. Of the 9 patients, 8 underwent surgical treatment and in 1 patient, lesion was diagnosed on transbronchial biopsy and effectively treated with corticosteroid. Two cases were associated with chronic sclerosing sialadenitis or lymphadenopathy, in which many IgG4-positive plasma cells were also identified by immunostaining. The clinicopathologic similarities between IPT of the lung and sclerosing pancreatitis suggest that IgG4-related immunopathologic processes might be involved in the pathogenesis of the pulmonary lesions.

Inflammatory pseudotumors of the lung with spontaneous regression.J Med Liban. 2005 Oct-Dec;53(4):229-33.

Inflammatory pseudotumors of the lung can be confusing in their presentation because they lack specific symptoms clinically and can easily be mistaken for malignancy on imaging studies. Their definite diagnosis requires pathologic analysis of tissue which is usually obtained only after surgical resection of the lesion. We report herein the case of a 65-year-old man who presented with recent onset of cough, shortness of breath and hemoptysis. CT scan of the chest showed several ill-defined suspicious densities in both lungs. CT guided needle core biopsy of one of the nodules showed changes consistent with inflammatory pseudotumor of the lung without any evidence of malignancy. The pulmonary lesions, which had rapidly progressed initially, resolved completely with simple clinical observation. The total regression of the "tumor nodules" in this case, without any treatment, illustrates a very unusual outcome of these pseudotumors.

Unusual cause of dysphagia: inflammatory pseudotumor of the lung.South Med J. 2005 Jun;98(6):665-8.

Inflammatory pseudotumor (IPT) is a rare disease that usually occurs in the lung. Patients with IPT are usually asymptomatic, with a solitary pulmonary nodule or mass detected on routine chest roentgenogram. IPT can behave as a malignant tumor both clinically and radiologically. Cough, fever, dyspnea, and hemoptysis are the usual presenting symptoms. This report describes the case of a 37-year-old man with a 4-month course of dysphagia secondary to lower esophageal invasion by the posterior mediastinal extension of a lung IPT.

Diagnosis and surgical treatment of inflammatory pseudotumor of the lung: a report of 51 cases.Ai Zheng. 2005 Feb;24(2):219-21.

BACKGROUND & OBJECTIVE: Clinical symptoms and imaging presentations of inflammatory pseudotumor of the lung (IPL) lack of specialties, and always result in misdiagnoses. This article was to summarize features of IPL, and to improve diagnosis accuracy and appropriate operation selection. METHODS: Records of 51 patients with pathologically confirmed IPL, treated surgically in Department of Thoracic Surgery, Cancer Institute/Hospital, Chinese Academy of Medical Sciences from Jan. 1990 to Mar. 2000, were retrospectively reviewed. All patients were followed-up for at least 3 years. RESULTS: Of the 51 patients, before operation, only 3 (5.9%) had been correctly diagnosed, 24 (47.1%) were suspected having tuberculoma or other benign lung tumors, 10 (19.6%) with undefined occupied lesion in the lung, 14 (27.4%) were misdiagnosed as lung cancer. All patients received surgery, including lobectomy in 23 patients (45.1%), tumor excision in 22 patients (43.1%), wedge resection in 4 patients (7.8%), and segmental resection in 2 patients (3.9%). Only 1 (2.0%) had postoperative complication. No death occurred with a follow-up rate of 94.1% (48/51). CONCLUSION: Preoperative diagnosing IPL is quite difficult. Surgery should be performed as early as possible. The selection of surgery patterns, mainly include lobectomy and limited resection, depends on intraoperative pathology.

Inflammatory pseudotumor of the lung in adults. Ann Thorac Surg. 2005 Feb;79(2):426-32.

BACKGROUND: Thoracic surgeons have limited experience of inflammatory pseudotumors of the lung owing to their rare occurrence in routine clinical practice. METHODS: We retrospectively investigated the clinicopathologic features of 18 patients with inflammatory pseudotumor of the lung observed between 1992 and 2002. RESULTS: There were 13 men and 5 women. Median age was 57 years. Eight patients (44%) were symptomatic. Computed tomographic scan showed a solitary nodule (< or =3 cm) in 12 patients, bilateral nodules in 1, and a mass in 5. Two patients had undergone prior incomplete resections. Lobectomy was performed in 5 patients, bilobectomy in 1, segmentectomy in 1, and wedge resection in 11. Complete resection was achieved in 13 patients (72%). There was no operative mortality. Follow-up was complete in all patients (range, 13 to 134 months; median, 63 months). Overall 3-year and 5-year survival rates were 82% and 74%, respectively. Thirteen patients are currently alive with no evidence of disease, 1 is alive with disease, 1 died of unrelated causes, and 3 had a relapse and died. Completeness of resection and lesion size less than or equal to 3 cm were associated with a better survival (p < 0.001 and p = 0.007, respectively). Multivariate Cox analysis confirmed the association between completeness of resection and better survival, which is independent of other clinicopathologic variables (p = 0.02). CONCLUSIONS: This series shows that a significant number of patients with inflammatory pseudotumor of the lung have a poor prognosis and confirms the need for radical resection in the treatment of this unusual entity.

Paraneoplasic syndrome in inflammatory pseudotumor of the lung.Cir Pediatr. 2004 Apr;17(2):98-100.

BACKGROUND: Inflammatory pseudotumor (IPT) is the most frequent pulmonary mass in childhood. It is histologically benign but locally aggressive. Atelectasis and recurrent airway infections are the most frequent presenting findings. We present two children in whom first clinical signs were paraneoplasic syndromes. MATERIAL AND METHODS: Retrospective study of two cases of IPT treated in our clinic from 1998 to 2002. Age, clinical presentation, preoperative diagnosis, treatment, histological diagnosis and postoperative outcome were reviewed from clinical chart. RESULTS: Case 1: 7 year old male with incidental diagnosis of superior right lobe IPT in routine study because of diabetes. The mass collapsed superior and median lobar arteries and compressed superior cava vein. Right pneumonectomy was necesary to complete removal of the mass. From immediate postoperatory the child became normoglycemic and is free of insulin. Case 2: 11 year old male with rheumatologic clinic consisting in hypertrophic osteoarthropathy, arthralgy and knees liquid lasting for two years. X-ray examination showed mediastinic mass. He underwent complete removal of a pulmonary mass. Few months after the operation the rheumatologic-like symptoms were resolved. CONCLUSIONS: IPT can present with paraneoplasic syndromes, although physiopathology is not well understood. In children with recurrent respiratory infections one diagnosis to take in mind is IPT, even more when clinic is associated with symptoms compatible with paraneoplasic syndrome.

Pulmonary inflammatory pseudotumor--a report of 28 cases.Korean J Intern Med. 2002 Dec;17(4):252-8.

BACKGROUND: Pulmonary inflammatory pseudotumor is an uncommon benign lesion of the lung. In Korea, most literature of the pulmonary inflammatory pseudotumor was case reports. METHODS: We collected 28 cases of pulmonary inflammatory pseudotumor in Korea. This collective series included 4 cases from our hospital and 24 cases were reviewed from the literature since 1977. The analysis involved the age, sex, chief complaint, hematologic examination, size and location of the lesion, cavity formation, presence of calcification and treatment method. RESULTS: Male was more prevalent (81.5%) than female and mean age was 37.9 years old (6-63 yrs). Chief complaints were cough (44.4%), chest pain (29.6%), fever (22.2%), hemoptysis (15%), sputum (15%) and dyspnea (11.1%). There were asymptomatic cases in 11.1%. Hematologic examination revealed normal finding (53.3%) and anemia (20%). The mean size of the lesion was 4.76 cm (1.5-14 cm) and the locations were parenchymal (85.7%), endobronchial (10.7%) and endotracheal (3.6%). Except the endotracheal case, the lesions were in the right (46.4%), the left (42.8%) and bilateral (7.1%). Calcifications (18.5%) and cavitations (11.1%) were present. Diagnostic methods were open thoracotomy (82.1%), bronchoscopy (3.6%), needle aspiration biopsy (7.1%) and core needle gun biopsy (7.1%). Treatments were surgery (85.2%), steroid therapy (7.4%), rigid bronchoscopic removal (3.7%) and observation (3.7%). Postoperative recurrence occurred in only 1 case (4.3%). CONCLUSION: Pulmonary inflammatory pseudotumor was more prevalent in the male, and patients presented with the respiratory symptoms were common. It was necessary to do surgery in most cases for diagnosis and/or treatment.

A case report of inflammatory pseudotumor of the lung: rapid recurrence appearing as multiple lung nodules.Ann Thorac Cardiovasc Surg. 2002 Aug;8(4):224-7.

An inflammatory pseudotumor (IPT), known as a plasma cell granuloma, is a relatively uncommon neoplasm with an unidentified etiology. To our knowledge, an early relapse with multiple lung nodules following lung resection and occurrences in multiple organs is extremely rare. The patient was a 49-year-old man who presented with left chest pain and fever. A chest film demonstrated an 8x8 cm mass in the left lower lobe. During thoracotomy in April 2001, a mass was seen to have invaded the diaphragm with remarkable pleural adhesion. The intraoperative pathological diagnosis was infiltration of inflammatory cells with no malignancy. Therefore, a partial resection of the left lower lobe was performed. Three months after the thoracotomy, a chest CT scan disclosed multiple nodular opacities bilaterally, and an open lung-biopsy of the right lung was performed in January 2002. His past history included an excision of a mass on the penis in another hospital in 1994 and a subcutaneous mass that appeared on the right thigh and disappeared spontaneously following a needle biopsy in 1999. Pathologically there was no fundamental difference among his present lesion and the former two. The pathological diagnosis at each occurrence was inflammatory pseudotumor (IPT). In immunohistochemical study, the staining with smooth muscle actin cells was positive, but was negative for the staining with anaplastic lymphoma kinase (ALK). With no evidence of a neoplastic process, these histopathological and immunohistochemical findings could imply that this case may be a postinflammatory reparative reaction, although his condition exhibited the clinically aggressive behavior of suspected lung metastasis.

 

December 2007

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