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Primary pulmonary hemangiopericytoma
Primary pulmonary hemangiopericytoma Report of a case Zev Davis, M.D., William P. Berliner, M.D., Louis H. Weiland, M.D., and O. Theron Clagett, M.D., Rochester,
Minn.
XXemangiopericytoma is a rare vascular tumor composed of proliferating pericytes surrounding small blood vessels. This tumor is usually found in the soft tissues and is generally not considered in the differential diagnosis of a solitary pulmonary nodule. Its rarity in this location is emphasized by our finding of only 18 cases reported in the world literature (Table I) and of only 1 other case in the files of the Mayo Clinic, a case that had been referred for pathologic consultation only.
abdomen revealed no abnormality. Heberden's nodes were present on both hands, and a herpetic lesion was seen on the lower lip. Laboratory study revealed a hemoglobin value of 15.1 Gm. per 100 ml. and a leukocyte count of 10,500 per cubic millimeter. The electrolytes, creatinine, urine, sugar, and electrocardiogram were all within normal limits. Pulmonary function studies showed a forced vital capacity of 2.6 L. (predicted 2.7 L.) and a maximal midexpiratory flow of 2.4 L. (predicted 1.8 L . ) . A chest roentgenogram revealed evidence of a nodule (2 cm.) in the left lung peripherally near the ninth rib posteriorly (Fig. 1). The patient underwent left thoracotomy and left lower lobectomy.
Case report
A firm, round, tan, well-circumscribed tumor (Fig. 2) was found deep in the parenchyma of the left lower lobe. Grossly, this had the appearance of a bronchial adenoma. Histologic examination revealed that the lesion was a hemangiopericytoma with moderate mitotic activity. Regional nodes were negative for metastases.
A 52-year-old white woman had complained of indeterminate abdominal cramps, loose stools, and hematochezia. She was hospitalized in her home community for evaluation. Examination revealed no abnormality except for a solitary nodule in the left lung. She was otherwise asymptomatic, having no cough, wheezing, or dyspnea. The lung lesion had not been present on a chest roentgenogram taken 4 years earlier. Her past history was unremarkable. She had smoked one pack of cigarettes a day for the past 32 years. Physical examination on admission at our clinic revealed an obese, healthy-looking woman. The blood pressure was 156/90 mm. Hg, pulse was regular at 80 beats per minute, and the rectal temperature was 98.6° F. There was no adenopathy, and examination of the heart, lungs and From the Mayo Clinic and Mayo Foundation, Rochester, Minn. 55901 Received for publication May 30, 1972.
822
Comment Murray and Stout15 originally described this mesenchymal tumor as a distinct pathologic entity. The tumor contains an abundance of endothelium-lined spaces, surrounded by proliferating round-to-elongated cells with prominent nuclei. Presumably, these cells are derived from the pericytes, which are part of the external wall of blood vessels. The vascular spaces may not be visible on slides stained with hematoxylin and eosin (Fig. 3, A), but these spaces are
Volume 64 Number 5 November, 1972
Primary pulmonary hemangiopericytoma
823
\ Fig. 1. Anteroposterior view. Nodule (2 cm.) in periphery of left lower lobe.
Fig. 2. Lower lobe shows well-circumscribed tumor deep in pulmonary parenchyma.
Fig. 3. A, Several small, ill-defined vascular spaces surrounded by uniform plump and slightly elongated cells. (Hematoxylin and eosin; x250.) B, Vascular spaces are more obvious. Many of the individual cells are also surrounded by delicate reticulin fibers. (Reticulin stains; x440.)
824
The Journal of
Davis et al.
Thoracic and Cardiovascular Surgery
Table I. Cases of primary pulmonary hemangiopericytoma reported in the literature11'' Sex
Age (yr.)
F M M
43 38 51
Location of lesion
—
— 59
RUL RLL RLL RLL LUL
—
65 46 43 62 13 43 49 66 57 38 64 34 18
LUL RLL LLL RLL, RUL LUL LLL RUL RML RUL LLL RML RLL LLL
F
F F M M F F M M F F M M
Treatment
Follow-up
—
—
—
—
—
1 yr., well 6 yr., well 3 mo., mediastinal metastasis
Pneumonectomy Pneumonectomy Wedge excision
1 yr., left pneumonectomy; 11 yr., mediastinal mass removed; 13 yr., mediastinal recurrence and RML metastasis
Right lower lobectomy Left lower lobectomy Multiple wedge excisions
—
Left lower lobectomy Right upper lobectomy Right middle lobectomy Segmental resection Left lower lobectomy Right middle lobectomy Segmental resection Left lower lobectomy
2 3 4 1 3
yr., well yr., well mo., well yr., well yr., well
— — — — —• —. —
Legend: RUL, Right upper lobe. RLL, Right lower lobe. LUL, Left upper lobe. LLL, Left lower lobe. RML, Right middle lobe.
well-demonstrated by the reticulin stain (Fig. 3, B). This aids in differentiating hemangiopericytoma from hemangioendothelioma, nonchromaffin paraganglioma, and histiocytic tumors. The lesion usually can be differentiated from the closely related glomus tumor by the use of hematoxylin and eosin stain; the glomus tumor has an obvious organoid pattern with small epithelioid cells. Determination of malignancy in hemangiopericytoma is often difficult or impossible histologically. Although the tumor may have moderate mitotic activity (as in the present case), this does not reliably indicate its malignancy. Fortunately, most hemangiopericytomas are benign. However, in 2 of the 18 cases previously reported, the lesion had metastasized to the mediastinum.3' ■• In the cases of recurrent and metastatic lesions, the tumors seemed to have occurred within 1 year after the primary lesion had been diagnosed. In the reported cases, the average age of the patient at diagnosis was 48 years, with a range of 13 to 66 years. There appears to
be no sex predilection. Most of the patients were asymptomatic, the lesion being detected on routine chest roentgenograms. Because the tumor is vascular, hemoptysis might be expected to be a common symptom. However, only Ochsner and DeCamp2 noted hemoptysis as a problem in 1 of their patients. Because of its malignant potential and its radioresistance, hemangiopericytoma should be surgically removed. Of the 18 patients described in the literature, 10 underwent lobectomy, 3 had wedge or segmental resections, 3 had pneumonectomy, and 2 had therapy that was not specifically stated. The 2 patients with metastasis and recurrence had been treated with wedge resection and pneumonectomy, respectively. Therefore, the tumor should be treated by ample excision as determined at operation. REFERENCES 1 McCormack, L. J., and Gallivan, W. F.: Hemangiopericytoma, Cancer 7: 595, 1954. 2 Ochsner, S., and DeCamp, P. T.: Hemangio-
Volume 64
Number 5
Primary pulmonary hemangiopericytoma 8 2 5
November, 1972
3 4 5 6 7 8 9
10
pericytoma of the Lung, Am. Rev. Tuberc. 77: 496, 1958. Hess, H., and Daum, R.: Beitrag zum Thema Hamangiopericytome, Langenbecks Arch. Chir. 294: 75, 1960. Wojtowicz, M., and Salwa, W.: Hamangioperizytom der Lunge, Zentralbl. Chir. 87: 709, 1962. Feldman, F., and Seaman, W. B.: Primary Thoracic Hemangiopericytoma, Radiology 82: 998, 1964. Krenz, M., and Romer, K. H.: Mitteilung iiber ein Hamangioperizytom der Lunge, Dtsch. Gesundheitsw. 20: 257, 1965. Rotte, K. H.: Ein Beitrag zum primaren Hamangioperizytom der Lunge, Arch. Geschwulstforsch. 26: 95, 1965. Sperling, E., and Wendt, F.: Hamangioperizytomatose der Lunge, Thoraxchirurgie 13: 321, 1965. Equen, M., Roach, G., and Brown, R.: Hemangiopericytoma of Lung Found in Search for Corsage Pin, Eye Ear Nose Throat Mon. 42: 34, 1963. Wellington, J. L., and Neuman, H. W.: Primary Hemangiopericytoma of the Lung, Can. Med. Assoc. J. 88: 1295, 1963.
11 McCormack, L. J., Mclsaac, W. M., Ragde, H., Groves, L. K., and Effler, D. B.: "Functioning" Pulmonary Neoplasms. I. The Carcinoid Tumor. II. The Hemangiopericytoma, Cleve. Clin. Q. 28: 145, 1961. 12 Vecchioni, R., Cordiano, C , and Perrino, G.: Sull'emangiopericitoma del polmone (presentazione di un caso), Acta Chir. Ital. 23: 545, 1967. 13 Poulet, J., Roujeau, J., de Brux, J., Meyer, A., and Monod, O.: Les tumeurs vasculaires primitives du poumon (a partir de quatre observations), J. Fr. Med. Chir. Thorac. 22: 645. 1968. 14 Bruart, J., Parmentier, R., Vanderhoeft, P., and Remade, P.: A propos d'un cas d'hemangiopericytome pulmonaire primitif associe a un syndrome de Klippel Trenaunay, Parker, Weber, J. Fr. Med. Chir. Thorac. 25: 145, 1971. 15 Murray, M. R., and Stout, A. P.: The Glomus Tumor: Investigation of Its Distribution and Behavior, and the Identity of Its "Epitheloid" Cell, Am. J. Pathol. 18: 183, 1942.
Minn.
XXemangiopericytoma is a rare vascular tumor composed of proliferating pericytes surrounding small blood vessels. This tumor is usually found in the soft tissues and is generally not considered in the differential diagnosis of a solitary pulmonary nodule. Its rarity in this location is emphasized by our finding of only 18 cases reported in the world literature (Table I) and of only 1 other case in the files of the Mayo Clinic, a case that had been referred for pathologic consultation only.
abdomen revealed no abnormality. Heberden's nodes were present on both hands, and a herpetic lesion was seen on the lower lip. Laboratory study revealed a hemoglobin value of 15.1 Gm. per 100 ml. and a leukocyte count of 10,500 per cubic millimeter. The electrolytes, creatinine, urine, sugar, and electrocardiogram were all within normal limits. Pulmonary function studies showed a forced vital capacity of 2.6 L. (predicted 2.7 L.) and a maximal midexpiratory flow of 2.4 L. (predicted 1.8 L . ) . A chest roentgenogram revealed evidence of a nodule (2 cm.) in the left lung peripherally near the ninth rib posteriorly (Fig. 1). The patient underwent left thoracotomy and left lower lobectomy.
Case report
A firm, round, tan, well-circumscribed tumor (Fig. 2) was found deep in the parenchyma of the left lower lobe. Grossly, this had the appearance of a bronchial adenoma. Histologic examination revealed that the lesion was a hemangiopericytoma with moderate mitotic activity. Regional nodes were negative for metastases.
A 52-year-old white woman had complained of indeterminate abdominal cramps, loose stools, and hematochezia. She was hospitalized in her home community for evaluation. Examination revealed no abnormality except for a solitary nodule in the left lung. She was otherwise asymptomatic, having no cough, wheezing, or dyspnea. The lung lesion had not been present on a chest roentgenogram taken 4 years earlier. Her past history was unremarkable. She had smoked one pack of cigarettes a day for the past 32 years. Physical examination on admission at our clinic revealed an obese, healthy-looking woman. The blood pressure was 156/90 mm. Hg, pulse was regular at 80 beats per minute, and the rectal temperature was 98.6° F. There was no adenopathy, and examination of the heart, lungs and From the Mayo Clinic and Mayo Foundation, Rochester, Minn. 55901 Received for publication May 30, 1972.
822
Comment Murray and Stout15 originally described this mesenchymal tumor as a distinct pathologic entity. The tumor contains an abundance of endothelium-lined spaces, surrounded by proliferating round-to-elongated cells with prominent nuclei. Presumably, these cells are derived from the pericytes, which are part of the external wall of blood vessels. The vascular spaces may not be visible on slides stained with hematoxylin and eosin (Fig. 3, A), but these spaces are
Volume 64 Number 5 November, 1972
Primary pulmonary hemangiopericytoma
823
\ Fig. 1. Anteroposterior view. Nodule (2 cm.) in periphery of left lower lobe.
Fig. 2. Lower lobe shows well-circumscribed tumor deep in pulmonary parenchyma.
Fig. 3. A, Several small, ill-defined vascular spaces surrounded by uniform plump and slightly elongated cells. (Hematoxylin and eosin; x250.) B, Vascular spaces are more obvious. Many of the individual cells are also surrounded by delicate reticulin fibers. (Reticulin stains; x440.)
824
The Journal of
Davis et al.
Thoracic and Cardiovascular Surgery
Table I. Cases of primary pulmonary hemangiopericytoma reported in the literature11'' Sex
Age (yr.)
F M M
43 38 51
Location of lesion
—
— 59
RUL RLL RLL RLL LUL
—
65 46 43 62 13 43 49 66 57 38 64 34 18
LUL RLL LLL RLL, RUL LUL LLL RUL RML RUL LLL RML RLL LLL
F
F F M M F F M M F F M M
Treatment
Follow-up
—
—
—
—
—
1 yr., well 6 yr., well 3 mo., mediastinal metastasis
Pneumonectomy Pneumonectomy Wedge excision
1 yr., left pneumonectomy; 11 yr., mediastinal mass removed; 13 yr., mediastinal recurrence and RML metastasis
Right lower lobectomy Left lower lobectomy Multiple wedge excisions
—
Left lower lobectomy Right upper lobectomy Right middle lobectomy Segmental resection Left lower lobectomy Right middle lobectomy Segmental resection Left lower lobectomy
2 3 4 1 3
yr., well yr., well mo., well yr., well yr., well
— — — — —• —. —
Legend: RUL, Right upper lobe. RLL, Right lower lobe. LUL, Left upper lobe. LLL, Left lower lobe. RML, Right middle lobe.
well-demonstrated by the reticulin stain (Fig. 3, B). This aids in differentiating hemangiopericytoma from hemangioendothelioma, nonchromaffin paraganglioma, and histiocytic tumors. The lesion usually can be differentiated from the closely related glomus tumor by the use of hematoxylin and eosin stain; the glomus tumor has an obvious organoid pattern with small epithelioid cells. Determination of malignancy in hemangiopericytoma is often difficult or impossible histologically. Although the tumor may have moderate mitotic activity (as in the present case), this does not reliably indicate its malignancy. Fortunately, most hemangiopericytomas are benign. However, in 2 of the 18 cases previously reported, the lesion had metastasized to the mediastinum.3' ■• In the cases of recurrent and metastatic lesions, the tumors seemed to have occurred within 1 year after the primary lesion had been diagnosed. In the reported cases, the average age of the patient at diagnosis was 48 years, with a range of 13 to 66 years. There appears to
be no sex predilection. Most of the patients were asymptomatic, the lesion being detected on routine chest roentgenograms. Because the tumor is vascular, hemoptysis might be expected to be a common symptom. However, only Ochsner and DeCamp2 noted hemoptysis as a problem in 1 of their patients. Because of its malignant potential and its radioresistance, hemangiopericytoma should be surgically removed. Of the 18 patients described in the literature, 10 underwent lobectomy, 3 had wedge or segmental resections, 3 had pneumonectomy, and 2 had therapy that was not specifically stated. The 2 patients with metastasis and recurrence had been treated with wedge resection and pneumonectomy, respectively. Therefore, the tumor should be treated by ample excision as determined at operation. REFERENCES 1 McCormack, L. J., and Gallivan, W. F.: Hemangiopericytoma, Cancer 7: 595, 1954. 2 Ochsner, S., and DeCamp, P. T.: Hemangio-
Volume 64
Number 5
Primary pulmonary hemangiopericytoma 8 2 5
November, 1972
3 4 5 6 7 8 9
10
pericytoma of the Lung, Am. Rev. Tuberc. 77: 496, 1958. Hess, H., and Daum, R.: Beitrag zum Thema Hamangiopericytome, Langenbecks Arch. Chir. 294: 75, 1960. Wojtowicz, M., and Salwa, W.: Hamangioperizytom der Lunge, Zentralbl. Chir. 87: 709, 1962. Feldman, F., and Seaman, W. B.: Primary Thoracic Hemangiopericytoma, Radiology 82: 998, 1964. Krenz, M., and Romer, K. H.: Mitteilung iiber ein Hamangioperizytom der Lunge, Dtsch. Gesundheitsw. 20: 257, 1965. Rotte, K. H.: Ein Beitrag zum primaren Hamangioperizytom der Lunge, Arch. Geschwulstforsch. 26: 95, 1965. Sperling, E., and Wendt, F.: Hamangioperizytomatose der Lunge, Thoraxchirurgie 13: 321, 1965. Equen, M., Roach, G., and Brown, R.: Hemangiopericytoma of Lung Found in Search for Corsage Pin, Eye Ear Nose Throat Mon. 42: 34, 1963. Wellington, J. L., and Neuman, H. W.: Primary Hemangiopericytoma of the Lung, Can. Med. Assoc. J. 88: 1295, 1963.
11 McCormack, L. J., Mclsaac, W. M., Ragde, H., Groves, L. K., and Effler, D. B.: "Functioning" Pulmonary Neoplasms. I. The Carcinoid Tumor. II. The Hemangiopericytoma, Cleve. Clin. Q. 28: 145, 1961. 12 Vecchioni, R., Cordiano, C , and Perrino, G.: Sull'emangiopericitoma del polmone (presentazione di un caso), Acta Chir. Ital. 23: 545, 1967. 13 Poulet, J., Roujeau, J., de Brux, J., Meyer, A., and Monod, O.: Les tumeurs vasculaires primitives du poumon (a partir de quatre observations), J. Fr. Med. Chir. Thorac. 22: 645. 1968. 14 Bruart, J., Parmentier, R., Vanderhoeft, P., and Remade, P.: A propos d'un cas d'hemangiopericytome pulmonaire primitif associe a un syndrome de Klippel Trenaunay, Parker, Weber, J. Fr. Med. Chir. Thorac. 25: 145, 1971. 15 Murray, M. R., and Stout, A. P.: The Glomus Tumor: Investigation of Its Distribution and Behavior, and the Identity of Its "Epitheloid" Cell, Am. J. Pathol. 18: 183, 1942.