Dermatomyositis as a Presentation of Pulmonary Inflammatory Pseudotumor (Myofibroblastic Tumor)

doi:10.1378/chest.117.6.1793

Chest

Volume 117, Issue 6, June 2000, Pages 1793-1795

https://doi.org/10.1378/chest.117.6.1793 Get rights and content

Inflammatory pseudotumor (IPT) is a rare pulmonary tumor of uncertain etiology that usually presents as an asymptomatic radiographic finding. We describe a case of pulmonary IPT presenting as dermatomyositis with complete resolution following surgical resection.

Section snippets

Case Report

A 48-year-old white man was hospitalized with chest pain and elevated serum creatine kinase. Administration of prednisone, 20 mg daily, had been initiated 2 weeks prior to presentation to treat a rash of recent onset. In the interim, the patient had developed fever, generalized myalgia, arthralgia, and cough productive of yellow sputum. The admitting physician interpreted the chest radiograph and ECG as unremarkable.

The patient had a 25-pack-year smoking history and had mined coal for 20 years.

Discussion

This case describes a patient with clinical DM that promptly resolved after resection of a lung mass subsequently found to be IPT. IPT is a rare benign disease of unknown etiology that predominantly affects younger patients; Bahadori and Liebow¹ described 40 patients, two thirds of whom were < 30 years old, and one third of whom were < 20 years old. IPT represents a localized proliferation of mature plasma cells and reticuloendothelial cells supported by granulation tissue that usually is

References (10)

O Matsubara et al.
Inflammatory pseudotumor of the lung: progression from organizing pneumonia to fibrous histiocytoma or to plasma cell granuloma in 32 cases
Hum Pathol
(1988)
EM Tan et al.
Antinuclear antibodies: diagnostically specific immune markers and clues towards the understanding of systemic autoimmunity
Clin Immunol Immunopathol
(1988)
M Bahadori et al.
Plasma cell granulomas of the lung
Cancer
(1973)
SV Golbert et al.
On pulmonary “pseudotumors.”
Neoplasia
(1967)
H Spencer
The pulmonary plasma cell/histiocytoma complex
Histopathology
(1984)

There are more references available in the full text version of this article.

Cited by (13)

Inflammatory pseudotumor of the lung in adults
2005, Annals of Thoracic Surgery
Citation Excerpt :
Further evidence of its neoplastic origin has been the finding of rearrangements of the anaplastic lymphoma kinase gene on chromosome 2p23, resulting in the expression of the ALK-1 protein, in a subgroup of patients with inflammatory pseudotumor of the lung [17–20]. Recently Malam and associates [21] described a case of inflammatory pseudotumor of the lung associated with a paraneoplastic syndrome (dermatomyositis). From a clinical point of view, the percentage of asymptomatic inflammatory pseudotumors of the lung ranges between 30% and 70% of cases [12, 13].
Thoracic surgeons have limited experience of inflammatory pseudotumors of the lung owing to their rare occurrence in routine clinical practice.
We retrospectively investigated the clinicopathologic features of 18 patients with inflammatory pseudotumor of the lung observed between 1992 and 2002.
There were 13 men and 5 women. Median age was 57 years. Eight patients (44%) were symptomatic. Computed tomographic scan showed a solitary nodule (≤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).
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.
Tracheal localization of inflammatory myofibroblastic tumor in adults: A case report
2019, Vojnosanitetski Pregled
Primary Pleural Inflammatory Pseudotumor in a Child
2018, Indian Pediatrics
Inflammatory myofibroblastic tumor of the lung: Unusual imaging findings of three cases
2015, Polish Journal of Radiology
Case report of anti-transcription intermediary factor-1-γ/α antibody-positive dermatomyositis associated with gastric cancer and immunoglobulin G4-positive pulmonary inflammatory pseudotumor
2013, Journal of Dermatology
Adult inflammatory myofibroblastic tumor of the trachea: Case report and literature review
2013, Respiratory Care

View all citing articles on Scopus

View full text

Chest

Selected ReportsDermatomyositis as a Presentation of Pulmonary Inflammatory Pseudotumor (Myofibroblastic Tumor)

Section snippets

Case Report

Discussion

Hum Pathol

Clin Immunol Immunopathol

Plasma cell granulomas of the lung

Cancer

On pulmonary “pseudotumors.”

Neoplasia

The pulmonary plasma cell/histiocytoma complex

Histopathology

Selected Reports
Dermatomyositis as a Presentation of Pulmonary Inflammatory Pseudotumor (Myofibroblastic Tumor)