Multiple pulmonary leiomyomatous hamartomas.Report of a case with ultrastructure examination

Abstract

The electron microscopic features of pulmonary leiom yomatous hamartoma, a very rare neoplasm, were studied. Characteristic smooth muscle cells containing microfilaments, dense bodies, plaques, pinocytotic vesicles, and basal lamina were present. Fibrosis was minimal, which supports the belief that this component is a secondary phenomena.Cancer 38:1199-1204, 1976. ULTIPLE PULMONARY FIBROLEIOMYOMATOUSM hamartomas are extremely rare benign lung lesions seen in asymptomatic middle-aged female patients. Apparently only 10 cases have been documented in the literature. 2 -~8 -1 0 -1 2 Although Kaplan et a1.3 illustrated their case with a n electron micrograph, this lesion has not been extensively studied ultrastructurally.Recently we had the opportunity to study the eleventh example of this lesion, with a thorough ultrastructural examination. CASE REPORTA 55-year-old black woman was hospitalized after a coin lesion of the right mid-zone lung had shown a gradual increase in size. Approximately 7 years previously, three coin lesions of the right lung were discovered on a routine chest-x-ray. She was followed closely, but had no complaints of dyspnea, hemoptysis, weight loss, fever, chills, or increase in size of the lesion on x-ray until the present time. A cough productive of a small amount of sputum was noted. A right supraclavicular node biopsy performed approximately 4 years previous to admission showed chronic lymphadenitis. Pertinent past medical history revealed a hysterectomy for leiomyoma approximately 15 years ago. The patient had been a diabetic controlled by diet for the past 5 years.O n admission the patient was in no distress, normotensive, afebrile, and well nourished. A systolic grade Il/VI murmur, heard best along the left sternal border and radiating to the apex and neck, was noted. This was felt to represent aortic stenosis. There was no evidence of left ventricular hypertrophy on EKG.Laboratory findings included: hemoglobin 12.5 g/ 100 ml, leukocyte count 7,00O/mm, platelet count 265,000/mm.3 A SMA 12 survey and urinalysis were within normal limits. Pulmonary ventilatory studies revealed a mild obstructive pulmonary abnormality with a slight reduction of the forced expiratory volume/ 1 second (FEV,) of 2.22 liters (76%) and a maximal mid-exploratory flow (MMEF) of 1.96 liters/ second. Arterial blood gases showed a hemoglobin saturation of 97%, pCO, of 34 mm Hg, pH of 7.43, and plasma HCO, of 22 mM/liter. Chest x-rays and tomograms showed a coin lesion in the right upper lobe which measured 2.2 cm, a coin lesion of the left upper lobe which measured 6 mm, and a coin lesion of the right mid-zone which measured 2.5 cm. No calcifications were seen. The lesions were felt to represent granulomata or, possibly, slowly growing pulmonary metastases (Fig. l).O n the fourth hospital day a right thoracotomy was performed with a wedge resection and subsegmental resection of the three coin lesions of the right lung; two in the upper lobe and one in the right middle lobe. A frozen-section diagnosis of multiple pulm...

Multiple pulmonary leiomyomatous hamartoma with secondary ossification

“…Multiple pulmonary leiomyomatous hamartoma (MPLH) is a rare lesion seen mainly in middle‐aged women 1 . The individual lesions are composed of an admixture of mature tissue, including smooth muscle, collagenous fibrous tissue and glandular elements.…”

Mesenchymal Tumors of the Lung

“…In earlier reports, the term leiomyomatous hamartoma has been used for these tumors when they appear to be multicentric. [14][15][16][17] Some researchers, however, have suggested that such neoplasms should not be considered to be primary lesions of the lung but should rather be regarded as metastatic tumors.…”

Clonal expansion of multiple pulmonary leiomyomatous hamartoma: 12‐year follow‐up

“…In 1999 we reported on a 31‐year‐old woman with multiple pulmonary leiomyomatous hamartoma (MPLH) 1 . MPLH is a peculiar lesion of the lung, the individual lesions of which are composed of a mixture of smooth muscle and glandular elements 2 . There is no apparent topographical relationship between the lesion and the bronchial trees or vascular structures, although a previous ultrastructural study suggests a possible relation to the contractile system of the lung acini 3 .…”

“…There is no apparent topographical relationship between the lesion and the bronchial trees or vascular structures, although a previous ultrastructural study suggests a possible relation to the contractile system of the lung acini 3 . MPLH is not associated with specific pathological conditions of the lung, including interstitial pneumonia or lymphangioleiomyomatosis, whereas there has been controversy regarding whether MPLH is equivalent to benign metastasizing leiomyoma 2,4–8 . The clinical course of MPLH also remains unclear, because there have been a few reports of long‐term follow up of patients with MPLH 9,10…”

“…First, the clinical course strongly indicated that MPLH is distinct from benign metastasizing uterine leiomyoma (BML). For a long time, there has been controversy regarding whether MPLH is equivalent to BML 2,4–8 . BML is an ill‐defined clinicopathological condition, which features “metastatic” histologically benign smooth muscle tumor deposits in the lung, lymph node, or abdomen, which appear to be derived from a benign uterine leiomyoma 11 .…”