Fine-Needle Aspiration Cytology in Fibromatoses Paul Zaharopoulos, M.D., M.I.A.C., and Jick Y. Wong, M.s., CT(ASCP)MT(ASCP)
Fine-needle aspiration (FNA) cytology was pevformed in seven cases offibromatosis of variable types with tumorous clinicalpresentation. These included: four cases of musculoaponeurotic fibromatosis, two in posterior neck muscles, one in anterior neck muscles and one in intercostal muscles; one case offibromatosis of the breast; and two cases of fibromatosis colli in neonates. In all cases the specimens contained connective tissue with many fibroblast-like cells, lacking features which could indicate a malignant lesion. The findings in these cases indicate that, although by FNA cytology in fibrornatoses a specific diagnosis for each pathologic entity may not be easily reached, in the proper clinical setting the cytologic findings can be of suficient relevance to offset the need for an open tissue biopsy, where there are valid reasons against a surgical intervention. Diagn Cytopathol 1992;8:73-78.
preparing initially cell spreads and then rinsing the syringe and needle in normal saline, which was used for cytocentrifuge preparations (Cytospin 3, Shandon Inc., Pittsburgh, PA). The surgical tissues were fixed in 10% formaldehyde and stained by the hematoxylin and eosin method. The seven cases included: four deep musculoaponeurotic fibromatoses (two in the posterior neck, one in the anterior neck and one in the intercostal muscles), one fibromatosis of the breast and two fibromatoses colli. Clinical data as they relate to the fibromatoses are summarized in Table I.
Results Key Words: Benign fibrous proliferations; Cytologic diagnosis
The fibromatoses encompass a broad variety of lesions, divided ito (1) superficial (fascial), (2) deep, which are musculoaponeurotic abdominal or extraabdominal, intraabdominal and others involving various organs of the body, and (3) fibromatoses of infancy and childhood. Fine-needle aspiration (FNA) cytology has been reported in a few of such cases, which show the relative value of this method in the diagnosis of these proliferations. 2-5 We report seven additional cases of variable nature, in an attempt to demonstrate the value as well as some of the shortcomings of FNA in the diagnosis of such lesions.
'
Materials and Methods Seven cases of fibromatosis were examined by FNA cytology, three of which were further investigated by tissue biopsy or excision of the lesion. The cytologic specimens were processed routinely by the Papanicolaou method, Received April 23, 1991. Accepted August 22, 1991. From the Department of Pathology, The University of Texas Medical Branch at Galveston, TX. Address reprint requests to Dr. Paul Zaharopoulos, Department of Pathology, University of Texas Medical Branch, Galveston, TX 77550. 0 1992 WILEY-LISS.
INC
FNA cytology findings and histologic findings available in three of the seven cases are summarized in Table I.
Discussion The fibromatoses examined pathologically are included in the benign proliferations of the fibrous connective tissue, which encompass a large number of entities forming diffuse masses and tumor-like lesions. In all such entities besides the fibroblast, the myofibroblast is a cell uniformly encountered, denoting an early phase of fibroblastic differentiation associated with cellular motility through the presence of intracellular contractile filaments of desmin and actin. Included in such proliferations are inflammatory processes, as nodular fascitis, showing immature fibroblastic cell forms even with increased mitosis and variable inflammatory cell response, and cytoproliferative tumor-like processes or fibromatoses the commonest of which are superficial (palmar, plantar, penile, etc.) and deep musculoaponeurotic or desmoids, affecting the abdominal wall and other sites, such as the neck muscles. 1*8 The fibromatoses can be localized or infiltrative and multicentric and can involve internal tissues and organs as the mesentery and the retroperitoneum (often associated Diagnostic Cytopathology, Vol 8, No I
13
ZAHAROPOULOS A N D WONG Table I. Clinical Data
Case
Anatomic site and clinical jindings
Duration
Radiographic findings
Age
Sex
1
52 yr
M
Right posteroauricular, posteriorly to right sternocleidomastoid muscle. 4 cm mass smooth, firm, well circumscibed. Pain in neck, shoulder.
3 Yr
MRI: Lesion in levator scapulae muscle with density lower than muscle.
2
57 yr
F
Right posterolateral neck. 5 cm diffuse mass. Pain in right shoulder and arm.
6 mon
CT scan: Paraspinal soft tissue mass from C, to C, lateral to erector spinae muscle.
3
59 yr
M
4
66 yr
F
Left anterior neck, behind and medial to insertion of sternocleidomastoid muscle extending to cricoid cartilage. 2 cm mass, smooth, firm, adherent to surrounding tissues. Right hypochrondium, posterior region. Fullness to palpation and tenderness.
5
60 yr
F
6
2 mon
7
2 mon
CT scan: low density lesion, origin: thyroid or soft tissues. Thyroid radioisotope scan: mass not related to thyroid.
9 mon Hx of local pain
C T scan: 3 cm soft tissue mass in distal 1 lth intercostal space.
Right breast. 3 cm subareolar mass firm, irregular, well circumscribed, retracting nipple.a
1 Yr
Right mammogram: Dense lesion with irregular margins.
M
Left sternocleidomastoid muscle, middle portion. 1.5 crn hard, nontender mass.
Gradual growth since birth.
M
Right sternocleidomastoid muscle, middle portion. 3.5 cm hard smooth, nontender mass.
Gradual growth since birth.
MRI: Mass arising and confined in sternocleidomastoid muscle.
aThere was history of multiple polyposis of the large intestine and resection of a benign fibrous lesion of the transverse mesocolon encroaching upon the colon.
with familial intestinal polyposis), the breast, and almost every organ and region of the body, including the bones, the meninges and the CNS. The fibromatoses of infancy and childhood are similar to the adult types, such as the aforementioned varieties, but also include types peculiar to this age group, as the digital inclusion type, characterized by cytoplasmic inclusion-like aggregates of filamentous actin in myofibroblasts, the juvenile hyalin fibromatosis of skin of head, showing on histology hyaline nodules of altered mucopolysaccharides, and the fibromatosis colli of the sternocleidomastoid muscle, which resolves within a few years not requiring 74
Diagnostic Cytopathology, Vol 8, No I
resection unless torticollis (persistent muscle spasm) develops. The etiology of the fibromatoses is variable. Trauma, genetic factors, tissue injury on a genetic predisposition toward an excessive fibroblastic growth, an altered production of connective tissue ground substances and hormonal factors have been implicated. Their prognosis varies, depending on the type. They can be self-limited and resolving (as the fibromatosis colli of the neonates), can respond to local excision (as the superficial fibromatoses), or can be aggressive, infiltrative, multicentric and recurring even after wide exci-
FNA CYTOLOGY IN FIBROMATOSES
~~
Clinical and
Histology findings and diagnosis
radiological differenrial diagnosis
FNA cytology findings and diagnosis
Hemangioma, chronic myositis, fibromatosis (desrnoid).
Connective tissue with immature appearing fibroblasts dissecting between skeletal muscle fibers (Fig. 1). Diagn: Consistent with fibromatosis.
Tissue biopsy: Fibrous connective tissue with benign features, consistent with fibromatosis. Patient refused surgical resection.
3 yr: Minimal increase in size. Chronic pain continues but stable.
Probable fibromatosis.
Fibroblastic tissue active and forming collagen, dissecting skeletal muscle and infiltrating fat. Degeneration of muscle fibers (multiplication of sarcolemmal nuclei) (Fig. 2). Diagn: Probable fibromatosis.
1 yr: C T scan: No recurrence.
Soft tissue mass, possible fibromatosis.
FNA repeated X 3 because of low yield. In all 3 aspirations: Scattered fibroblastic cells with benign features. Diagn: Fibrosis, consistent with fibromatosis. Fragments of fibrous connective tissue consisting of fibroblasts and collagen bundles haphazardly distributed (Fig. 4). Diagn: Consistent with fibromatosis. FNA repeated X 2 because of low yield. Fibrous connective tissue with immature appearing fibroblasts infiltrating fat. Microunits of ductal epithelium with atypia suspicious for carcinoma. (Fig. 5). Diagn: Suspicious for ductal carcinoma with fibrosis.
Surgical resection: Mass of fibrous connective tissue of variable maturation and collagenization with remnants of degenerated skeletal muscle in it, suggesting origin from muscle. Peripheral spread of fibrosis into skeletal muscle and fat (Fig. 3). Diagn: Fibromatosis. Tissue biopsy, surgical resection deferred (adherence of lesion to vital structures).
Soft tissue lesion low grade malignant or benign (possible fibromatosis). Carcinoma, likely inflammatory type.
Fibromatosis colli.
Fibromatosis colli.
Fibrous connective tissue with immature appearing fibroblasts and capillary vessels in association with skeletal muscle showing degeneration and repair (multiplication of sarcolemmal nuclei) (Fig. 7). Diagn: Fibromatosis colli. Findings as in case 6 (Fig. 8). Diagn: Fibromatosis colli.
sion (as many cases of deep musculoaponeurotic fibromatoses and those of internal organs). 8,2@22 In such cases surgical excision is at times supplemented by radiotherapy or chemotherapy. Surgical biopsy is usually necessary to provide a definitive diagnosis of benign fibrous connective tissue proliferations, including fibromatoses, distinguishing them from neoplastic lesions of connective tissue. l 3 FNA cytology has been reported in relatively few cases of benign fibromatoses, which include a case of fibromatosis colli, a case of infantile myofibromatosis and two cases of fibromatosis of breast. 2-5 Our cases supplement this experience 14720,21
Tissue biopsy surgical resection
Follow-up
4 yr: Minimal increase in size.
Tissue biopsy, surgical resection deferred (serious medical problems including schizophrenia).
1 yr: CT scan: Minimal increase in size.
Surgical excision: 3 cm mass of fibrous connective tissue undergoing collagenization, extending to overlying skin and surrounding fat and encroaching upon proximal and distal ducts showing focal atypical epitheliosis (Fig. 6 ) . Diagn: Fibrosis of breast Tissue biopsy, surgical resection deemed unnecessary.
2 yr: No recurrence.
Tissue biopsy, surgical resection deemed unnecessary.
Follow-up data not available
3 mon: mass did not increase in size. No muscle spasm present.
and demonstrate that FNA is of value in providing a tentative pathologic diagnosis of these tumor-like lesions, which under the appropriate clinical setting can be used for management, if surgical intervention is contraindicated or not warranted. They also emphasize that since fibromatoses of certain organs such as the breast can faithfully imitate malignancy, care should be exercised in the interpretation of the cytologic findings in order to minimize diagnostic errors. 23,24 A point to stress is that, if a fibrous proliferation such as fibromatosis or another lesion containing increased amount of connective tissue particularly collagenous is Diagnostic Cytopathology, Val 8, No 1
75
Fig. 1. Case 1. F N A cytology. Fibrous connective tissue with immature appearing fibroblasts between skeletal muscle fibers (Papanicolaou stain,
x 400).
3. Case 2. Histology. A: Center of lesion. Mature fibrous connective tissue with remnants of skeletal muscle fibers. B: Periphery of lesion. Active fibroblastic tissue within skeletal muscle (hematoxylin and eosin, x 250).
Fig. 2. Case 2. F N A cytology. Fibrous connective tissue with haphazardly arranged immature appearing fibroblasts and partial collagenization (Papanicolaou stain, X 250).
Fig. 4. Case 4.F N A cytology. Fibrous connective tissue with irregular collagen bundles and mature spindly fibroblasts (Papanicolaou stain, X 250).
76
Diagnostic Cytopathology, Vol 8, No I
Fig. 5. Case 5. FNA cytology. A Dysplastic ductal epithelium from area of atypical epitheliosis (Papanicolaou stain, X 400). B Fibrous connective tissue with immature appearing fibroblasts (Papanicolaou stain, x 250).
Fig. 7. Case 6. F N A cytology. Fibrous connective tissue with immature appearing fibroblasts in association with degenerated skeletal muscle fibers showing multinucleation (Papanicolaou stain, X 400).
Fig. 6 . Case 5. Histology. Fibrous connective tissue with irregular collagen bundles encroaching upon mammary ducts (hematoxylin and eosin, x 100).
Fig. 8, Case 7. F N A cytology. Fibrous connective tissue with fibroblastic cell activity in association with multinucleated skeletal muscle fibers (Papanicolaou stain, x400). Diagnostic Cytopathology, Vol 8, No 1
17
ZAHAROPOULOS AND WONG
anticipated, the aspiration should be more vigorous than the ordinary and thorough, as dense connective tissues are not as easy to sample by aspiration as epithelial and other tissues. In most of the present cases we used a 21-gauge needle (instead of 22-gauge which we routinely use) making two or three passes from different directions, so as to avoid the same track and seeing that the in and out needle movements were multiple, multidirectional and more rapid than the average despite the resistance encountered by the fibrous tissue. Fibromatosis of the breast presents as a localized induration, usually deep seated, but at times subareolar, producing considerable skin retraction imitating carcinoma. 2426 Despite such deceitful presentation, our case had in retrospect by clinical history hallmarks of a fibromatosis syndrome, namely localized fibrosis of the mesocolon and associated multiple polyposis of the large intestine, awareness of which could have facilitated a correct diagnosis by FNA cytology and histology as well. Indeed, in many of these fibrous proliferations knowledge of the clinical setting, including history, is indispensable for a correct interpretation of the cytologic and histologic findings, particularly in unusual presentations of such lesions. In conclusion, although open biopsy is usually indispensable in the diagnosis of the various types of fibromatosis, FNA has its place, if a genuine effort is made to obtain representative material and the clinical setting is relied upon for interpretation of the cytologic findings.
References 1. Enzinger FM, Weis SW. Soft tissue tumors. 2nd ed. St. Louis: CV
Mosby, 1988:102-200. 2. el-Naggar A, Abdul-Karim FW, Marshalleck JJ, Sorensen K. Fineneedle aspiration of fibromatosis of the breast. Diagn Cytopathol 1989;3:32&2. 3. Ostrowski ML, Bradshaw J, Garrison D. Infantile myofibromatosis: diagnosis suggested by fine-needle aspiration biopsy. Diagn Cytopathol 19906:284-8. 4. Wakely PE, Price WG, Frable WJ. Sternomastoid tumor of infancy (fibromatosis colli): diagnosis by aspiration cytology. Mod Pathol 1989;2:378-8 1. 5 . Tani EM, Stanley MW, Skoog L. Fine-needle aspiration of bilateral mammary fibromatosis. Acta Cytol 1988;32:555-8. 6. Hasegawa T, Hirose T, Kudo E, Abe J, Hizawa K. Cytoskeletdl characteristics of myofibroblasts in benign neoplastic and reactive fibroblastic lesions. Virchows Arch [A] 1990416:375-82.
78
Diagnostic Cytopathology, Vol 8, No I
7. Kiryu H, Tsuneyoshi M, Enjoji M. Myofibroblasts in fibromatoses. An electron microscopic study. Acta Pathol Jpn 1985;35:533-47. 8. Delgadillo LA, Arenson DJ. Plantar fibromatosis. Surgical considerations with case histories. J Foot Surg 1985;24:258-65. 9. Adickes ED, Goodrich P, AuchMoedy J, Bickers G, Bowden B, Koh J, Nelson RM, Shuman RM, Wilson RB. Central nervous system involvement in congenital visceral fibromatosis. Pediatr Pathol 1985;3:329-40. 10. Altemani AM, Amstalden EI, Martins-Filho J. Congenital generalized fibromatosis causing spinal cord compression. Hum Pathol 1985;16:1063-5. 11. Chan YF, Lau JH, Tong CY. Congenital generalized fibromatosis with predominant osseous involvement in a Chinese newborn. J Pediatr Orthop 1989;9:64-8. 12. Uranus S, Beham A, Stenzl W. Fibromatosis. A rare retroperitoneal tumour. Langenbecks Arch Chir 1990;375:51-4. 13. Haidu SI, Haidu EO. Cytopathology of soft tissue and bone tumours. In: Wied GL, ed. Monographs in clinical cytology, vol. 12. New York: Karger, 1989:66-70. 14. Ayala AG, Ro JY, Goepfert H, Cangir A, Khorsand J, Flake G. Desmoid fibromatosis: a clinicopathologic study of 25 children. Semin Diagn Pathol 1986;3:138-50. 15. Fayad MN, Yacoub A, Salman S, Khudr A, Der Kaloustian VM. Juvenile hyaline fibromatosis: two new patients and review of the literature. Am J Med Genet 1987;26:123-31. 16. Fringes B, Thais H, Bohm N, Altmannsberger M, Osborn M. Identification of actin microfilaments in the intracytoplasmic inclusions present in recurring infantile digital fibromatosis (Reye tumor). Pediatr Pathol 1986;6:311-24. 17. Mayer-da-Silva A, Poiares-Baptista A, Rodrigo FG, Teresa-Lopes M. Juvenile hyaline fibromatosis. A histologic and histochemical study. Arch Pathol Lab Med 1988;112:928-31. 18. Yun K. Infantile digital fibromatosis. Immunohistochemical and ultrastructural observations of cytoplasmic inclusions. Cancer 1988;61:500-7. 19. Purdy LJ, Colby TV. Infanite digital fibromatosis occurring outside the digit. Am J Surg Pathol 1984:8:787-90. 20. Oberthaler W, Rhomberg W. Aggressive fibromatosis. A rare use for lumbar bulging. Arch Orthop Trauma Surg 1988;107:388-90. 21. Taylor LJ. Musculoaponeurotic fibromatosis. A report of 28 cases and review of the literature. Clin Orthop 1987;224:294-302. 22. Marty-Double C, Balmes P, Mary H, Allieu Y, Pignodel C, Targhetta R, Lesbros D, Metge L. Juvenile fibromatosis resembling aponeurotic fibroma and congenital multiple fibromatosis. One case with pleuropulmonary involvement. Cancer 1988;61:146-52. 23. Hanna WM, Jambrosic J, Fish E. Aggressive fibromatosis of the breast. Arch Pathol Lab Med 1985;109:26&2. 24. Sommerville JE, Biggart JD. Fibromatosis of the breast: a benign lesion which simulates a carcinoma. Ulster Med J 1989;58:97-9. 25. Rosen PP, Ernsberger D. Mammary fibromatosis. A benign spindle cell tumor with significant risk for local recurrence. Cancer 1989; 63:1363-9. 26. Wargotz ES, Norris HJ, Austin RM, Enzinger FM. Fibromatosis of the breast. A clinical and pathological study of 28 cases. Am J Surg Pathol 1987;11:38-45.
Fine-needle aspiration (FNA) cytology was pevformed in seven cases offibromatosis of variable types with tumorous clinicalpresentation. These included: four cases of musculoaponeurotic fibromatosis, two in posterior neck muscles, one in anterior neck muscles and one in intercostal muscles; one case offibromatosis of the breast; and two cases of fibromatosis colli in neonates. In all cases the specimens contained connective tissue with many fibroblast-like cells, lacking features which could indicate a malignant lesion. The findings in these cases indicate that, although by FNA cytology in fibrornatoses a specific diagnosis for each pathologic entity may not be easily reached, in the proper clinical setting the cytologic findings can be of suficient relevance to offset the need for an open tissue biopsy, where there are valid reasons against a surgical intervention. Diagn Cytopathol 1992;8:73-78.
preparing initially cell spreads and then rinsing the syringe and needle in normal saline, which was used for cytocentrifuge preparations (Cytospin 3, Shandon Inc., Pittsburgh, PA). The surgical tissues were fixed in 10% formaldehyde and stained by the hematoxylin and eosin method. The seven cases included: four deep musculoaponeurotic fibromatoses (two in the posterior neck, one in the anterior neck and one in the intercostal muscles), one fibromatosis of the breast and two fibromatoses colli. Clinical data as they relate to the fibromatoses are summarized in Table I.
Results Key Words: Benign fibrous proliferations; Cytologic diagnosis
The fibromatoses encompass a broad variety of lesions, divided ito (1) superficial (fascial), (2) deep, which are musculoaponeurotic abdominal or extraabdominal, intraabdominal and others involving various organs of the body, and (3) fibromatoses of infancy and childhood. Fine-needle aspiration (FNA) cytology has been reported in a few of such cases, which show the relative value of this method in the diagnosis of these proliferations. 2-5 We report seven additional cases of variable nature, in an attempt to demonstrate the value as well as some of the shortcomings of FNA in the diagnosis of such lesions.
'
Materials and Methods Seven cases of fibromatosis were examined by FNA cytology, three of which were further investigated by tissue biopsy or excision of the lesion. The cytologic specimens were processed routinely by the Papanicolaou method, Received April 23, 1991. Accepted August 22, 1991. From the Department of Pathology, The University of Texas Medical Branch at Galveston, TX. Address reprint requests to Dr. Paul Zaharopoulos, Department of Pathology, University of Texas Medical Branch, Galveston, TX 77550. 0 1992 WILEY-LISS.
INC
FNA cytology findings and histologic findings available in three of the seven cases are summarized in Table I.
Discussion The fibromatoses examined pathologically are included in the benign proliferations of the fibrous connective tissue, which encompass a large number of entities forming diffuse masses and tumor-like lesions. In all such entities besides the fibroblast, the myofibroblast is a cell uniformly encountered, denoting an early phase of fibroblastic differentiation associated with cellular motility through the presence of intracellular contractile filaments of desmin and actin. Included in such proliferations are inflammatory processes, as nodular fascitis, showing immature fibroblastic cell forms even with increased mitosis and variable inflammatory cell response, and cytoproliferative tumor-like processes or fibromatoses the commonest of which are superficial (palmar, plantar, penile, etc.) and deep musculoaponeurotic or desmoids, affecting the abdominal wall and other sites, such as the neck muscles. 1*8 The fibromatoses can be localized or infiltrative and multicentric and can involve internal tissues and organs as the mesentery and the retroperitoneum (often associated Diagnostic Cytopathology, Vol 8, No I
13
ZAHAROPOULOS A N D WONG Table I. Clinical Data
Case
Anatomic site and clinical jindings
Duration
Radiographic findings
Age
Sex
1
52 yr
M
Right posteroauricular, posteriorly to right sternocleidomastoid muscle. 4 cm mass smooth, firm, well circumscibed. Pain in neck, shoulder.
3 Yr
MRI: Lesion in levator scapulae muscle with density lower than muscle.
2
57 yr
F
Right posterolateral neck. 5 cm diffuse mass. Pain in right shoulder and arm.
6 mon
CT scan: Paraspinal soft tissue mass from C, to C, lateral to erector spinae muscle.
3
59 yr
M
4
66 yr
F
Left anterior neck, behind and medial to insertion of sternocleidomastoid muscle extending to cricoid cartilage. 2 cm mass, smooth, firm, adherent to surrounding tissues. Right hypochrondium, posterior region. Fullness to palpation and tenderness.
5
60 yr
F
6
2 mon
7
2 mon
CT scan: low density lesion, origin: thyroid or soft tissues. Thyroid radioisotope scan: mass not related to thyroid.
9 mon Hx of local pain
C T scan: 3 cm soft tissue mass in distal 1 lth intercostal space.
Right breast. 3 cm subareolar mass firm, irregular, well circumscribed, retracting nipple.a
1 Yr
Right mammogram: Dense lesion with irregular margins.
M
Left sternocleidomastoid muscle, middle portion. 1.5 crn hard, nontender mass.
Gradual growth since birth.
M
Right sternocleidomastoid muscle, middle portion. 3.5 cm hard smooth, nontender mass.
Gradual growth since birth.
MRI: Mass arising and confined in sternocleidomastoid muscle.
aThere was history of multiple polyposis of the large intestine and resection of a benign fibrous lesion of the transverse mesocolon encroaching upon the colon.
with familial intestinal polyposis), the breast, and almost every organ and region of the body, including the bones, the meninges and the CNS. The fibromatoses of infancy and childhood are similar to the adult types, such as the aforementioned varieties, but also include types peculiar to this age group, as the digital inclusion type, characterized by cytoplasmic inclusion-like aggregates of filamentous actin in myofibroblasts, the juvenile hyalin fibromatosis of skin of head, showing on histology hyaline nodules of altered mucopolysaccharides, and the fibromatosis colli of the sternocleidomastoid muscle, which resolves within a few years not requiring 74
Diagnostic Cytopathology, Vol 8, No I
resection unless torticollis (persistent muscle spasm) develops. The etiology of the fibromatoses is variable. Trauma, genetic factors, tissue injury on a genetic predisposition toward an excessive fibroblastic growth, an altered production of connective tissue ground substances and hormonal factors have been implicated. Their prognosis varies, depending on the type. They can be self-limited and resolving (as the fibromatosis colli of the neonates), can respond to local excision (as the superficial fibromatoses), or can be aggressive, infiltrative, multicentric and recurring even after wide exci-
FNA CYTOLOGY IN FIBROMATOSES
~~
Clinical and
Histology findings and diagnosis
radiological differenrial diagnosis
FNA cytology findings and diagnosis
Hemangioma, chronic myositis, fibromatosis (desrnoid).
Connective tissue with immature appearing fibroblasts dissecting between skeletal muscle fibers (Fig. 1). Diagn: Consistent with fibromatosis.
Tissue biopsy: Fibrous connective tissue with benign features, consistent with fibromatosis. Patient refused surgical resection.
3 yr: Minimal increase in size. Chronic pain continues but stable.
Probable fibromatosis.
Fibroblastic tissue active and forming collagen, dissecting skeletal muscle and infiltrating fat. Degeneration of muscle fibers (multiplication of sarcolemmal nuclei) (Fig. 2). Diagn: Probable fibromatosis.
1 yr: C T scan: No recurrence.
Soft tissue mass, possible fibromatosis.
FNA repeated X 3 because of low yield. In all 3 aspirations: Scattered fibroblastic cells with benign features. Diagn: Fibrosis, consistent with fibromatosis. Fragments of fibrous connective tissue consisting of fibroblasts and collagen bundles haphazardly distributed (Fig. 4). Diagn: Consistent with fibromatosis. FNA repeated X 2 because of low yield. Fibrous connective tissue with immature appearing fibroblasts infiltrating fat. Microunits of ductal epithelium with atypia suspicious for carcinoma. (Fig. 5). Diagn: Suspicious for ductal carcinoma with fibrosis.
Surgical resection: Mass of fibrous connective tissue of variable maturation and collagenization with remnants of degenerated skeletal muscle in it, suggesting origin from muscle. Peripheral spread of fibrosis into skeletal muscle and fat (Fig. 3). Diagn: Fibromatosis. Tissue biopsy, surgical resection deferred (adherence of lesion to vital structures).
Soft tissue lesion low grade malignant or benign (possible fibromatosis). Carcinoma, likely inflammatory type.
Fibromatosis colli.
Fibromatosis colli.
Fibrous connective tissue with immature appearing fibroblasts and capillary vessels in association with skeletal muscle showing degeneration and repair (multiplication of sarcolemmal nuclei) (Fig. 7). Diagn: Fibromatosis colli. Findings as in case 6 (Fig. 8). Diagn: Fibromatosis colli.
sion (as many cases of deep musculoaponeurotic fibromatoses and those of internal organs). 8,2@22 In such cases surgical excision is at times supplemented by radiotherapy or chemotherapy. Surgical biopsy is usually necessary to provide a definitive diagnosis of benign fibrous connective tissue proliferations, including fibromatoses, distinguishing them from neoplastic lesions of connective tissue. l 3 FNA cytology has been reported in relatively few cases of benign fibromatoses, which include a case of fibromatosis colli, a case of infantile myofibromatosis and two cases of fibromatosis of breast. 2-5 Our cases supplement this experience 14720,21
Tissue biopsy surgical resection
Follow-up
4 yr: Minimal increase in size.
Tissue biopsy, surgical resection deferred (serious medical problems including schizophrenia).
1 yr: CT scan: Minimal increase in size.
Surgical excision: 3 cm mass of fibrous connective tissue undergoing collagenization, extending to overlying skin and surrounding fat and encroaching upon proximal and distal ducts showing focal atypical epitheliosis (Fig. 6 ) . Diagn: Fibrosis of breast Tissue biopsy, surgical resection deemed unnecessary.
2 yr: No recurrence.
Tissue biopsy, surgical resection deemed unnecessary.
Follow-up data not available
3 mon: mass did not increase in size. No muscle spasm present.
and demonstrate that FNA is of value in providing a tentative pathologic diagnosis of these tumor-like lesions, which under the appropriate clinical setting can be used for management, if surgical intervention is contraindicated or not warranted. They also emphasize that since fibromatoses of certain organs such as the breast can faithfully imitate malignancy, care should be exercised in the interpretation of the cytologic findings in order to minimize diagnostic errors. 23,24 A point to stress is that, if a fibrous proliferation such as fibromatosis or another lesion containing increased amount of connective tissue particularly collagenous is Diagnostic Cytopathology, Val 8, No 1
75
Fig. 1. Case 1. F N A cytology. Fibrous connective tissue with immature appearing fibroblasts between skeletal muscle fibers (Papanicolaou stain,
x 400).
3. Case 2. Histology. A: Center of lesion. Mature fibrous connective tissue with remnants of skeletal muscle fibers. B: Periphery of lesion. Active fibroblastic tissue within skeletal muscle (hematoxylin and eosin, x 250).
Fig. 2. Case 2. F N A cytology. Fibrous connective tissue with haphazardly arranged immature appearing fibroblasts and partial collagenization (Papanicolaou stain, X 250).
Fig. 4. Case 4.F N A cytology. Fibrous connective tissue with irregular collagen bundles and mature spindly fibroblasts (Papanicolaou stain, X 250).
76
Diagnostic Cytopathology, Vol 8, No I
Fig. 5. Case 5. FNA cytology. A Dysplastic ductal epithelium from area of atypical epitheliosis (Papanicolaou stain, X 400). B Fibrous connective tissue with immature appearing fibroblasts (Papanicolaou stain, x 250).
Fig. 7. Case 6. F N A cytology. Fibrous connective tissue with immature appearing fibroblasts in association with degenerated skeletal muscle fibers showing multinucleation (Papanicolaou stain, X 400).
Fig. 6 . Case 5. Histology. Fibrous connective tissue with irregular collagen bundles encroaching upon mammary ducts (hematoxylin and eosin, x 100).
Fig. 8, Case 7. F N A cytology. Fibrous connective tissue with fibroblastic cell activity in association with multinucleated skeletal muscle fibers (Papanicolaou stain, x400). Diagnostic Cytopathology, Vol 8, No 1
17
ZAHAROPOULOS AND WONG
anticipated, the aspiration should be more vigorous than the ordinary and thorough, as dense connective tissues are not as easy to sample by aspiration as epithelial and other tissues. In most of the present cases we used a 21-gauge needle (instead of 22-gauge which we routinely use) making two or three passes from different directions, so as to avoid the same track and seeing that the in and out needle movements were multiple, multidirectional and more rapid than the average despite the resistance encountered by the fibrous tissue. Fibromatosis of the breast presents as a localized induration, usually deep seated, but at times subareolar, producing considerable skin retraction imitating carcinoma. 2426 Despite such deceitful presentation, our case had in retrospect by clinical history hallmarks of a fibromatosis syndrome, namely localized fibrosis of the mesocolon and associated multiple polyposis of the large intestine, awareness of which could have facilitated a correct diagnosis by FNA cytology and histology as well. Indeed, in many of these fibrous proliferations knowledge of the clinical setting, including history, is indispensable for a correct interpretation of the cytologic and histologic findings, particularly in unusual presentations of such lesions. In conclusion, although open biopsy is usually indispensable in the diagnosis of the various types of fibromatosis, FNA has its place, if a genuine effort is made to obtain representative material and the clinical setting is relied upon for interpretation of the cytologic findings.
References 1. Enzinger FM, Weis SW. Soft tissue tumors. 2nd ed. St. Louis: CV
Mosby, 1988:102-200. 2. el-Naggar A, Abdul-Karim FW, Marshalleck JJ, Sorensen K. Fineneedle aspiration of fibromatosis of the breast. Diagn Cytopathol 1989;3:32&2. 3. Ostrowski ML, Bradshaw J, Garrison D. Infantile myofibromatosis: diagnosis suggested by fine-needle aspiration biopsy. Diagn Cytopathol 19906:284-8. 4. Wakely PE, Price WG, Frable WJ. Sternomastoid tumor of infancy (fibromatosis colli): diagnosis by aspiration cytology. Mod Pathol 1989;2:378-8 1. 5 . Tani EM, Stanley MW, Skoog L. Fine-needle aspiration of bilateral mammary fibromatosis. Acta Cytol 1988;32:555-8. 6. Hasegawa T, Hirose T, Kudo E, Abe J, Hizawa K. Cytoskeletdl characteristics of myofibroblasts in benign neoplastic and reactive fibroblastic lesions. Virchows Arch [A] 1990416:375-82.
78
Diagnostic Cytopathology, Vol 8, No I
7. Kiryu H, Tsuneyoshi M, Enjoji M. Myofibroblasts in fibromatoses. An electron microscopic study. Acta Pathol Jpn 1985;35:533-47. 8. Delgadillo LA, Arenson DJ. Plantar fibromatosis. Surgical considerations with case histories. J Foot Surg 1985;24:258-65. 9. Adickes ED, Goodrich P, AuchMoedy J, Bickers G, Bowden B, Koh J, Nelson RM, Shuman RM, Wilson RB. Central nervous system involvement in congenital visceral fibromatosis. Pediatr Pathol 1985;3:329-40. 10. Altemani AM, Amstalden EI, Martins-Filho J. Congenital generalized fibromatosis causing spinal cord compression. Hum Pathol 1985;16:1063-5. 11. Chan YF, Lau JH, Tong CY. Congenital generalized fibromatosis with predominant osseous involvement in a Chinese newborn. J Pediatr Orthop 1989;9:64-8. 12. Uranus S, Beham A, Stenzl W. Fibromatosis. A rare retroperitoneal tumour. Langenbecks Arch Chir 1990;375:51-4. 13. Haidu SI, Haidu EO. Cytopathology of soft tissue and bone tumours. In: Wied GL, ed. Monographs in clinical cytology, vol. 12. New York: Karger, 1989:66-70. 14. Ayala AG, Ro JY, Goepfert H, Cangir A, Khorsand J, Flake G. Desmoid fibromatosis: a clinicopathologic study of 25 children. Semin Diagn Pathol 1986;3:138-50. 15. Fayad MN, Yacoub A, Salman S, Khudr A, Der Kaloustian VM. Juvenile hyaline fibromatosis: two new patients and review of the literature. Am J Med Genet 1987;26:123-31. 16. Fringes B, Thais H, Bohm N, Altmannsberger M, Osborn M. Identification of actin microfilaments in the intracytoplasmic inclusions present in recurring infantile digital fibromatosis (Reye tumor). Pediatr Pathol 1986;6:311-24. 17. Mayer-da-Silva A, Poiares-Baptista A, Rodrigo FG, Teresa-Lopes M. Juvenile hyaline fibromatosis. A histologic and histochemical study. Arch Pathol Lab Med 1988;112:928-31. 18. Yun K. Infantile digital fibromatosis. Immunohistochemical and ultrastructural observations of cytoplasmic inclusions. Cancer 1988;61:500-7. 19. Purdy LJ, Colby TV. Infanite digital fibromatosis occurring outside the digit. Am J Surg Pathol 1984:8:787-90. 20. Oberthaler W, Rhomberg W. Aggressive fibromatosis. A rare use for lumbar bulging. Arch Orthop Trauma Surg 1988;107:388-90. 21. Taylor LJ. Musculoaponeurotic fibromatosis. A report of 28 cases and review of the literature. Clin Orthop 1987;224:294-302. 22. Marty-Double C, Balmes P, Mary H, Allieu Y, Pignodel C, Targhetta R, Lesbros D, Metge L. Juvenile fibromatosis resembling aponeurotic fibroma and congenital multiple fibromatosis. One case with pleuropulmonary involvement. Cancer 1988;61:146-52. 23. Hanna WM, Jambrosic J, Fish E. Aggressive fibromatosis of the breast. Arch Pathol Lab Med 1985;109:26&2. 24. Sommerville JE, Biggart JD. Fibromatosis of the breast: a benign lesion which simulates a carcinoma. Ulster Med J 1989;58:97-9. 25. Rosen PP, Ernsberger D. Mammary fibromatosis. A benign spindle cell tumor with significant risk for local recurrence. Cancer 1989; 63:1363-9. 26. Wargotz ES, Norris HJ, Austin RM, Enzinger FM. Fibromatosis of the breast. A clinical and pathological study of 28 cases. Am J Surg Pathol 1987;11:38-45.
