Fibrous Histiocytoma of the Trachea
ROBERT E. SANDSTROM, M.D., KARL H. PROPPE, M.D., AND ROBERT L TRELSTAD, M.D.
Sandstrom, Robert E., Proppe, Karl H., and Trelstad, Robert
L.: Fibrous histiocytoma of the trachea. Am J Clin Pathol 70:
429-433, 1978. The light and electron microscopic features
of a fibrous histiocytoma of the trachea that occurred in a
15-year-old Caucasian girl are presented. Emphasis is placed
on the aggressive behavior and the importance of early
recognition of the lesion in an unusual location. (Key words:
Fibrous histiocytoma; Trachea.)
FIBROUS LESIONS vary greatly in their microscopic appearances and clinical behaviors. The term
"fibrous histiocytoma" was used by Kauffman and
Stout 7 and O'Brien and Stout 11 to describe fibrous
lesions with a histiocytic character. Subsequently,
several studies have further defined the characteristics
of fibrous histiocytomas. 4,813
We here report a case of aggressive fibrous histiocytoma arising in the trachea. Emphasis is placed on
early recognition and expeditious therapy.
Materials and Methods
Light
Microscopy
The tissue was fixed in 10% buffered formalin
and processed according to routine procedures at
Massachusetts General Hospital. The sections were
stained by hematoxylin and eosin, Masson's trichrome,
and Verhoeff-van Gieson stains. Frozen sections were
stained with Sudan IV.
James Homer Wright Pathology Laboratories,
Department of Pathology, Massachusetts General
Hospital, and Shriners Burn Institute, Harvard
Medical School, Boston, Massachusetts
cut on a Porter-Blum MT-2 microtome, mounted on
naked 200-mesh copper grids, and stained with lead
citrate and uranyl acetate.
Case Report
History
A 15-year-old Caucasian female had been in good health until
early 1973, when she noticed the onset of cough, progressive
exertional dyspnea, and stridor. In June 1973, an irregular tracheal
mass was visualized radiographically and locally resected via
bronchoscope. The pathologic diagnosis was disputed. Chondrosarcoma, pseudotumor, and lymphoma were considered. Stridor
and dyspnea persisted, and two months after the bronchoscopic
resection a portion of one tracheal ring and the visualized recurrent tumor were excised. In January 1974, the patient was
seen at another hospital, where multiple bronchoscopic biopsies
failed to reveal tumor. However, continued dyspnea and stridor
led to admission to the Massachusetts General Hospital, where
bronchoscopy revealed an irregularly surfaced tumor mass of
rubbery to firm consistency. The tumor occupied 50% of the
tracheal lumen and was situated 2 cm below the vocal cord centered
on the right lateral and posterior tracheal wall. En-bloc surgical
excision was advised.
Operative
Electron
Microscopy
Tissues for electron microscopy were fixed at room
temperature for two hours in 2.5% glutaraldehyde and
4.0% paraformaldehyde in 0.1 M sodium cacodyiate
buffer at pH 7.4, washed briefly in 0.1% M sodium
cacodyiate, and postfixed at 4 C in 1.3% osmium
tetroxide in collidine buffer atpH 7.1. Following osmication, the tissues were stained en bloc with 2.0%
uranyl acetate in collidine buffer a t p H 5.1. The tissues
were dehydrated in ethanol and embedded in Araldite
(6005). Sections with silver interference colors were
Received April 11, 1977; received revised manuscript July 28,
1977; accepted for publication July 28, 1977.
Supported in part by a grant from the National Institutes of Health,
HL 18714. Dr. Trelstad is recipient of a Faculty Research Award
ACS #PR-I07.
Address reprint requests to Dr. Proppe: Department of Pathology,
Massachusetts General Hospital, Boston, Massachusetts 02114.
Findings
An endotracheal tube was passed beyond the obstructing mass
and halothane anesthesia administered. Dissection revealed extensive irregular infiltration at the base of the mass. The right
lobe of the thyroid was firmly adherent to the external tracheal
surface near the mass. The dissection was carried to the cricoid
cartilage. The right and left sides of the tumor were mobilized,
and the adherent thyroid lobe resected. A proximal margin of
subcutaneous tissue and a portion of the cricoid cartilage were
examined by frozen section and found to be free of tumor. A segment of five tracheal rings was removed, and the tracheal margins
anastomosed.
Postoperative
Course
The patient did well after the operation and was discharged on
the eighth postoperative day. She was readmitted a month after
operation for bronchoscopic examination, at which time no residual
tumor was visualized. A right hemithyroidectomy was undertaken,
and no residual tumor was seen in the thyroid specimen. One
year after operation the patient was well.
0002-9173/78/0900/0429 $00.75 © American Society of Clinical Pathologists
429
430
SANDSTROM, PROPPE AND TRELSTAD
A.J.C.P. • September 1978
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FIG. 1. Transected trachea and adjacent thyroid tissue. There is a polypoid intraluminal projection of tumor with a superficial resemblance to granulation tissue. On the right side of the picture, the submucosa is replaced by a tumor that invades through the
tracheal cartilate (arrow) and sweeps anteriorly and posteriorly in the paratracheal tissue, with extensive replacement and
destruction of the thyrpid gland. Hematoxylin and eosin. x9.
Gross Pathology (MGH75-12365)
The specimen was 3 cm long and consisted of five
tracheal rings and a portion of the cricoid cartilage.
The average tracheal luminal diameter was 1.5 cm. On
the right wall of the trachea, 1 cm below the superior
margin of excision, was an irregularly surfaced brown,
focally hemorrhagic tumor projecting 0.8 cm into the
lumen. The transsected tumor mass measured 0.9
x 0.8 cm. A fragment of thyroid adherent to the
tracheal wall was focally invaded by gray-tan tissue
tongues that spread through the tracheal wall. The
tracheal wall below the tumor was thickened with a
yellow-tan discoloration and irregular fibrotic appearance.
Light Microscopic Findings
The tumor was present in the submucosa and projected in a polypoid fashion into the tracheal lumen
(Fig. 1). It was ulcerated and showed acute and chronic
inflammation with granulation tissue and squamous
metaplasia. Tumor cells were partially obscured in the
intraluminal lesion by granulation tissue and chronic
inflammation, but were easily recognized in the sub-
mucosa, where they eroded focally through the tracheal
cartilage and formed a tumor mass outside the trachea.
The adjacent thyroid tissue was invaded in an irregular
manner by solid sheets of tumor, which blended
imperceptably with stromal connective tissue and capsule. Vascular invasion by tumor was seen in a small
vein in the capsule.
Individual cells varied somewhat in appearance but
were generally spindle shaped and associated with
connective tissue (Figs. 2-4). They formed interlacing fascicles, which in some areas had a storiform
appearance. Some cells were more histiocytic in appearance in that they were round or oval with abundant
cytoplasm. Occasional cells contained vacuoles in the
cytoplasm (Fig. 4). Multinucleated giant cells were not
infrequent, and occasional mitotic figures were seen.
Sudan stains revealed fat globules within large histiocytic and multinucleated cells.
Electron Microscopic Findings
A spectrum of cytoplasmic features consistent with
both fibrocytic and histiocytic cells were apparent
(Figs. 5 and 6). The fibrocytic cells contained a well-
vol. 70 . No. 3
CASE REPORTS
43 |
•I
FIG. 2 (/e//). Photomicrograph from the submucosal area, showing irregularly arranged
plump fibroblasts. Hematoxylin and eosin. x600.
FIG. 3 (right). An illustration of a multinucleated giant cell from the submucosal
area. Hematoxylin and eosin. x600.
developed endoplasmic reticulum and matrix secretory
vacuoles which were generally of a fusiform shape,
and were closely associated with bundles of crossstriated extracellular collagen fibrils (Fig. 5). The
nuclei in the fibrocytic cells were often convoluted
and multilobulated, and showed peripheral chromatin
condensation.
The histiocytic cells were generally more rounded in
shape, with cytoplasmic vacuoles containing in some
cases an amorphous fibrillar material and in others a
more homogeneous material resembling lipid. Some
vacuoles in addition contained apparent fragments of
cytoplasm. The histiocytic cells consistently had a relatively well developed endoplasmic reticulum and
were also closely associated with amorphous and
fibrillar extracellular material (Fig. 6). Nuclear pleomorphism with extensive convolution was frequent,
and in some regions suggested nuclear budding and
fragmentation.
Five cell types have been described in previous
studies of fibrous histiocytomas: fibroblastic; histio-
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FIG. 4. Photomicrograph, showing invasion and replacement
of thyroid tissue. The invasive portions of the lesion had this
appearance of rather uniform spindle fibroblasts. Only a rare
mitotic figure was observed. Hematoxylin and eosin. x240.
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432
SANDSTROM, PROPPE AND TRELSTAD
A.J.C.P. • September 1978
FIG. 6. Electronmicrograph of spindle-shaped fibroblastic cells
from the invasive portion of the lesion. Bundles of collagen are
apparent between the cells. The nuclei are irregular, with pronounced convolution and lobulation, x3,200.
FIG. 5. Photomicrograph of plastic-embedded tissue from submucosal area, showing plump histiocytic cells, including a typical
foamy or xanthomatous cell. Fat stains on fresh tissue revealed
the presence of sudanophilic material in such cells, x 1,176.
cytic; immature, or stem; xanthomatous and multinucleated giant cells. Comparison of the cells present
in this case with those reported by Fu and associates4
suggests a similar distribution and type, with fewer
xanthomatous cells.
Discussion
Fibrous histiocytomas have been most frequently reported to occur in the skin and subcutaneous tissue,
although few sites appear spared.7,813
Histiologically, fibrous histiocytoma varies widely in
appearance. Some are predominantly fibrous, and
others are largely histiocytic or xanthomatous. There
is frequently variation from one microscopic field to
another. The characteristic features have been thoroughly reviewed by Soule and Enriquez.13
Histiocytic cells, giant cells, and spindle cells
grouped in a characteristic storiform, pinwheel, or
whorled pattern are the main features essential for
diagnosis. Cytologically benign giant cells with centrally grouped or peripherally located nuclei are seen.
Bizarre, anaplastic giant cells with irregular folded
nuclei are frequently present. Lipid-laden vesiculated
single or multinucleated cells are typical and have
been studied at the ultrastructural level by Fu and
associates.4 Mitotic activity is variable. A mixed
chronic inflammatory infiltrate is often present. Outgrowths from tumor tissue have revealed fibroblasts,
histiocytic cells, and intermediate forms and evidence
of phagocytosis by tumor cells.12
Kempson and Kyriakos8 reported 30 cases of malig-
Table I. Reported Cases of Fibrous Histiocytoma of the Trachea
Reference
Patient's
Age (Yr.), Sex, Race
Location
of Tumor
Size
(cm)
Presenting
Complaint
Treatment
Comments
Karlen el a/. 6
57, F, Cauc.
4 cm below
glottis
2.5 x 2.5
x 0.8
Wheezing;
hemoptysis
Local bronchoscopic resection
No recurrence
at 2 years
Hakimi el at.i
26, M, Cauc.
Level of
thoracic
inlet
2.0 x 1.5
x 1.5
Cough;
hemoptysis
Wide excision
and tracheal
reconstruction
No recurrence
at 27 months
Present case
15, F, Cauc.
2 cm below
vocal
cords
0.9 x 0.8
x 0.8
Wheezing
Wide excision at
tracheal reconstruction and
partial
thyroidectomy
No recurrence
at 1 year
Vol. 70 • No. 3
CASE REPORTS
nant fibrous histiocytomas and set forth their criteria
for malignancy. The presence of many mitoses, bizarre
giant cells, and foam cells portend a poor prognosis.
However, the appearance of individual tumor cells often may be misleading. Even benign-appearing lesions
frequently recur locally. Metastatic spread to local
lymph nodes and occasionally widespread metastases
have been reported.1114
In the present case the tumor had a benign histologic
appearance. The giant cells were not bizarre and anaplastic, and only a few foam cells were present.
Yet the lesion recurred after curettage and grew in an
aggressive manner, invading and destroying nearby
tissues.
Fibrous histiocytomas have been reported arising
in the lung.3,810 The first such case was reported by
Liebow and Hubbel9 in 1956. The lesion arose within
pulmonary parenchyma and had a fibrous storiform
appearance, and the histology was suggestive of a
sclerosing hemangioma.
Endobronchial lesions have been described by
Bates and Hull2 and by Armstrong and co-workers.1
In each instance the lesion occurred in a young Caucasian male. The case reported by Bates and Hull
manifested as recurrent right-lower-lobe pneumonia.
The patient described by Armstrong had intractable
wheezing, cough, and hemoptysis. Pneumonectomy
(Bates and Hull) and lobectomy (Armstrong and coworkers) were performed, and no recurrence was found
on the four-to-five-year follow up examination.
Two fibrous histiocytomas of the trachea have been
reported (see Table 1). In each instance the tumor
was associated with wheezing, cough, dyspnea, or
hemoptysis.
This case illustrates the difficulty in diagnosis when
433
a tumor that is usually associated with soft tissue occurs
as a primary intratracheal tumor. Recognition of fibrous
histiocytoma and awareness of its occurrence in this
unusual location greatly facilitate prompt and appropriate treatment, which should be complete excision where
feasible. In the present case many recurrences might
have been avoided if early histologic recognition and
complete excision had been undertaken early in the
course of the disease.
References
1. Armstrong P, Elston C, Sanderson M: Endobronchial histiocytoma. Br J Radiol 48:221-222, 1975
2. Bates T, Hull OH: Histiocytoma of the bronchus. Dis Child
95:53-56, 1958
3. Dubilier LD, Bryant LR, Danielson GK: Histiocytoma (fibrous
xanthoma) of the lung. Am J Surg 115:420-426, 1968
4. Fu YS, Gabbian G, Kaye GI, et al: Malignant soft tissue tumors
of probable histiocytic origin (malignant fibrous histiocytoma): General considerations and electron microscopic
and tissue culture studies. Cancer 35:176-198, 1975
5. Hakimi M, Pai RP, Fine G, et al: Fibrous histiocytoma of the
trachea. Chest 68:367-368, 1975
6. Karlen MS, Livingston PA, Baker DC: Diagnosis of tracheal
tumors. Ann Otol Rhinol Laryngol 82:790-799, 1973
7. Kauffman SG, Stout AP: Histiocytic tumors (fibrous xanthoma
and histiocytoma) in children. Cancer 14:469-482, 1961
8. Kempson RL, Kyriakas M: Fibroxanthosarcoma of the soft
tissues. Cancer 29:961-976, 1972
9. Liebow AA, Hubbel DS: Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer 9:53-75, 1956
10. Nair S, Nair K, Weisbrot 1M: Fibrous histiocytoma of the lung
(sclerosing hemangioma variant?) Chest 65:465-468. 1974
11. O'Brien JE, Stout AP: Malignant fibrous xanthomas. Cancer
17:1445-1455, 1964
12. Ozello L, Stout AP, Murray MR: Cultural characteristics of
malignant histiocytomas and fibrous xanthomas. Cancer 16:
331-344, 1963
13. Soule EH, Enriquez P: Atypical fibrous histiocytoma, malignant fibrous histiocytoma, malignant histiocytoma and
epitheloid sarcoma. Cancer 30:128-143, 1972
14. Wasserman TH, Stuard ID: Malignant fibrous histiocytomas
with widespread metastases. Cancer 33:141-146, 1974