8
395 Benign Soft-Tissue Tumors in a Large Referral Population: Distribution of Specific Diagnoses by Age, Sex, and Location Mark J. Knansdorf1 Received July 26, 1 994; accepted after revision August30, 1994. I Departmentof Radiology, SaintMary’s Hospital, Richmond, VA 23226 and Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000. Address correspon- dence to M. J. Kransdorf at Saint Mary’s Hospital. 0361-803X195/1642-395 © American Roentgen Ray Society OBJECTIVE. The purpose of this study was to determine the specific diagnoses, rela- tive prevalence, and the age, sex, and skeletal distribution of benign soft-tissue tumors and to ascertain the relative frequency of these tumors in specific anatomic locations and age groups among a population of patients in a large pathologic consultation service. MATERIALS AND METHODS. The computer diagnoses of 39,179 lesions occurring in 38,484 patients seen by the Armed Forces Institute of Pathology soft-tissue pathol- ogists during the 10-year period starting January 1 , 1980, and ending December 31, 1989, were retrospectively reviewed. All lesions were placed In one of 121 major cate- genes in accordance with the classification system used by the World Health Organi- zation and coded to one of 32 anatomic locations such as hand, wrist, and forearm. Age and sex of the patients were also recorded. For purposes of analysis, all lesions were placed in one of 1 0 categories: hand and wrist, upper extremity, proximal limb girdle (axilla and shoulder), foot and ankle, lower extremity, hip and buttocks region, head and neck, trunk, retroperitoneum, and other lesions. The study group included 31 ,047 mesenchymal lesions, of which 18,677 were benign. RESULTS. Approximately two thirds of soft-tissue tumors were classified into seven diagnostic categories: lipoma and lipoma variants (16%), fibrous histiocytoma (13%), nodular fasciitis (11%), hemangioma (8%), fibromatosis (7%), neurofibroma (5%), and schwannoma (5%). Approximately 80% of all benign tumors were placed in seven diagnostic categories for each age and location. In the retroperitoneum, for example, approximately half the benign lesions in the 16- to 25-year old group were fibromatosis (20%), schwannoma (14%), and neurofibroma (13%). For the same boa- tion in children 5 years old or younger, almost two thirds of the benign tumors were lipoblastoma (37%) or lymphangioma (26%). CONCLUSION. Despite the large number of pathologic possibilities, most benign soft-tissue tumors are classified into a small number of specific diagnostic catego- ries. These may be further defined when the location of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist the radiologist in establishing a suitably ordered differential diagnosis when a soft-tissue tumor has a nonspecific radiologic appearance. AJR 1995;164:395-402 The imaging evaluation of soft-tissue tumors has undergone a dramatic evolu- tion with the advent of CT and MR imaging. Despite these sophisticated technolo- gies and the increasing number of lesions that may have a characteristic imaging appearance, the vast majority of lesions remain nonspecific, with a correct histo- logic diagnosis reached on the basis of imaging studies in only approximately one quarter of cases [1-3]. Unlike with their intraosseous counterparts, it is often not possible to establish a meaningful differential diagnosis for these nonspecific lesions or to reliably deter- mine if they are benign or malignant. In these cases, knowledge of the tumor’s prevalence, along with the patient’s age and the lesion’s location, will allow a suit- ably ordered differential diagnosis. The purpose of this study was to determine the relative prevalence and the age, sex, and skeletal distribution of benign soft-tissue tumors and to ascertain the relative frequency ofthese tumors in specific anatomic Downloaded from www.ajronline.org by Dartmouth - Hitchcock Ctr on 02/12/19 from IP address 130.189.218.69. Copyright ARRS. For personal use only; all rights reserved

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Page 1: BenignSoft-Tissue Tumorsina LargeReferral Population...soft-tissue tumors areclassified intoasmallnumber ofspecific diagnostic catego-ries.Thesemaybefurtherdefined whenthelocation

395

Benign Soft-Tissue Tumors in aLarge Referral Population:Distribution of Specific Diagnoses by Age,Sex, and Location

Mark J. Knansdorf1

Received July 26, 1 994; accepted after revisionAugust30, 1994.

I Departmentof Radiology, SaintMary’s Hospital,

Richmond, VA 23226 and Department of RadiologicPathology, Armed Forces Institute of Pathology,Washington, DC 20306-6000. Address correspon-dence to M. J. Kransdorf at Saint Mary’s Hospital.

0361-803X195/1642-395© American Roentgen Ray Society

OBJECTIVE. The purpose of this study was to determine the specific diagnoses, rela-tive prevalence, and the age, sex, and skeletal distribution of benign soft-tissue tumorsand to ascertain the relative frequency of these tumors in specific anatomic locations andage groups among a population of patients in a large pathologic consultation service.

MATERIALS AND METHODS. The computer diagnoses of 39,179 lesions occurringin 38,484 patients seen by the Armed Forces Institute of Pathology soft-tissue pathol-ogists during the 10-year period starting January 1 , 1980, and ending December 31,1989, were retrospectively reviewed. All lesions were placed In one of 121 major cate-genes in accordance with the classification system used by the World Health Organi-zation and coded to one of 32 anatomic locations such as hand, wrist, and forearm.Age and sex of the patients were also recorded. For purposes of analysis, all lesionswere placed in one of 1 0 categories: hand and wrist, upper extremity, proximal limbgirdle (axilla and shoulder), foot and ankle, lower extremity, hip and buttocks region,head and neck, trunk, retroperitoneum, and other lesions. The study group included31 ,047 mesenchymal lesions, of which 18,677 were benign.

RESULTS. Approximately two thirds of soft-tissue tumors were classified intoseven diagnostic categories: lipoma and lipoma variants (16%), fibrous histiocytoma(13%), nodular fasciitis (11%), hemangioma (8%), fibromatosis (7%), neurofibroma(5%), and schwannoma (5%). Approximately 80% of all benign tumors were placed inseven diagnostic categories for each age and location. In the retroperitoneum, forexample, approximately half the benign lesions in the 16- to 25-year old group werefibromatosis (20%), schwannoma (14%), and neurofibroma (13%). For the same boa-tion in children 5 years old or younger, almost two thirds of the benign tumors werelipoblastoma (37%) or lymphangioma (26%).

CONCLUSION. Despite the large number of pathologic possibilities, most benignsoft-tissue tumors are classified into a small number of specific diagnostic catego-ries. These may be further defined when the location of the lesion and the age of thepatient are considered. Knowledge of tumor prevalence will assist the radiologist inestablishing a suitably ordered differential diagnosis when a soft-tissue tumor has anonspecific radiologic appearance.

AJR 1995;164:395-402

The imaging evaluation of soft-tissue tumors has undergone a dramatic evolu-tion with the advent of CT and MR imaging. Despite these sophisticated technolo-gies and the increasing number of lesions that may have a characteristic imagingappearance, the vast majority of lesions remain nonspecific, with a correct histo-logic diagnosis reached on the basis of imaging studies in only approximately onequarter of cases [1-3].

Unlike with their intraosseous counterparts, it is often not possible to establish ameaningful differential diagnosis for these nonspecific lesions or to reliably deter-mine if they are benign or malignant. In these cases, knowledge of the tumor’sprevalence, along with the patient’s age and the lesion’s location, will allow a suit-ably ordered differential diagnosis. The purpose of this study was to determine therelative prevalence and the age, sex, and skeletal distribution of benign soft-tissuetumors and to ascertain the relative frequency ofthese tumors in specific anatomic

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Page 2: BenignSoft-Tissue Tumorsina LargeReferral Population...soft-tissue tumors areclassified intoasmallnumber ofspecific diagnostic catego-ries.Thesemaybefurtherdefined whenthelocation

396 KRANSDORF AJR:164, February 1995

locations and age groups among a population of patients in alarge pathologic consultation service.

Materials and Methods

The computer records of all patients seen in consultation by theDepartment of Soft-Tissue Pathology, Armed Forces Institute of

Pathology, during the 10-year period starting January 1 , 1980, andending December 31 , 1989, were reviewed retrospectively. Only mes-enchymal lesions originating in soft tissue were included in the study.lntraabdominal and retropenitoneal lesions were also included when

the lesions were not thought to originate in bowel or abdominal vis-cera. Hence, an extrarenal angiomyolipoma was included, whereas alymphangioma of the spleen was not. Lesions arising in the chest andabdominal walls and paraspinal region were also included, as theyare frequently within the purview ofthe musculoskeletal radiologist.

Computer diagnoses were accessed under the Pathology NaturalLanguage Retrieval System (PANLARS) and were individuallyreviewed and standardized in accordance with the classification sys-tem used by the World Health Organization [4] (as modified by Enz-inger and Weiss [5]). No attempt was made to reclassify computerdiagnoses, and histologic material was not reexamined. A lesion diag-nosed as a “lipoma with areas of hibernomatous change” was codedas such and not as a “hibernoma.” Lesions were subcategorized whenpossible and when such information was clinically relevant. All soft-tissue tumors and tumomlike lesions were placed in one of 121 majordiagnostic categories. Lesions were coded to 32 anatomic locationssuch as hand, wrist, forearm, arm, and so on. For purposes of analy-sis, all lesions were placed in one of 10 categories: hand and wrist,upper extremity, proximal limb girdle (axilla and shoulder), foot andankle, lower extremity, hip and buttocks region, head and neck, trunk,retroperitoneum, and other lesions. This last category included lesionscoded as abdomen, pelvis, mediastinum, or location unknown.

Age was recorded to the nearest year for all patients more than 1year old. Patients less than 1 year old were grouped into newborn (1day or less), 1-10 days, 11-28 days, 29 days through 2 months, 3-5

months, and 6-1 1 months of age. In addition, the patient’s sex andrace were recorded for each case. In total, the records of 42,490lesions occurring in 38,484 patients were reviewed. Multiple lesions

were seen in 639 patients (2%), including 592 patients with two

lesions, 39 patients with three lesions, seven patients with fourlesions, and one patient with five lesions. Sequential biopsy speci-mens were found in 3311 cases. This often involved an initial mci-sional biopsy specimen and subsequent material from definitivesurgery. Such cases were counted only once, so as not to falselyincrease the number of a particular lesion. A total of 39,179 soft-tis-sue tumors (and tumorlike masses) were available for detailed anal-ysis. From this group, 8132 nonmesenchymal lesions wereexcluded. This represented approximately 21% of all lesions andconsisted of 3370 malignant and 4762 benign lesions: 1487 casesof carcinoma; 564 cases of malignant melanoma; 472 cases of lym-phoma; 75 other malignant tumors (e.g. , seminoma, plasmacytoma,germ cell tumor, and malignant teratoma); 772 malignant tumorsthat could not be further classified; 2932 proliferative, reactive, andinflammatory lesions; 543 nonmesenchymal benign lesions (e.g.,teratoma, hamartoma, histiocytosis, pilomatnixoma, and syningoma);160 benign lesions that could not be further classified; and 1127miscellaneous lesions (e.g. , fat necrosis, foreign body reaction,hematoma, lipogranuloma, and thrombus).

The study group consisted of 31 047 lesions: 12,370 malignant

and 18,677 benign. Borderline and low-grade malignant lesions,such as dermatofibrosarcoma protuberans, atypical fibroxanthoma,angiomatoid malignant fibrous histiocytoma, infantile fibrosarcoma,and so on, were classified as malignant tumors. Superficial and deep(musculoaponeurotic) fibromatosis were considered benign lesions.

A total of 30,597 patients were in the study group: 16,727 men,1 3,611 women, and 259 whose sex was unknown. The patient’s age

was known in 30,244 cases and ranged from newborn to 97 years.In 26,854 cases (10,184 malignant and 16,670 benign), the

patient’s age was known and the lesion was located in one of theanatomic categories listed above (hand and wrist, upper extremity,etc.), excluding “other” lesions. The seven most frequent malignantlesions were then identified for each of the nine anatomic areas forages 0-5 years, 6-15 years, 16-25 years, 26-45 years, 46-65years, and for patients more than 65 years old.

Results

There were 18,677 benign mesenchymal lesions. Approxi-mately 70% of benign lesions were classified into eightpathologic diagnostic categories: lipoma and lipoma variants(16%), fibrous histiocytoma (13%), nodular fasciitis (11%),hemangioma (8%), fibromatosis (7%), neurofibroma (5%),schwannoma (5%), and giant cell tumor of the tendon sheath(4%). A summary of the benign lesions is given in Table 1,including patients’ age distribution and mean age, sex, andskeletal distribution of lesions for all histologic diagnoses.

The number and percentage of the most common benignlesions for each age and location are shown in Table 2. Allhemangiomas have been grouped together fonthis analysis, ashave lymphangiomas, and superficial and deep fibromatoses.Lipoma, lipomatosis, spindle cell lipoma, pleomomphic lipoma,and intramuscular lipoma have been combined and classifiedas lipoma. In total, 52 diagnostic categories were used for thisanalysis. Approximately 80% of all benign tumors can beplaced in the seven most common diagnostic categories foreach age and location.

Discussion

Radiologic detection and evaluation of soft-tissue masseshave become increasingly important with the advent of CTand MR imaging. Unfortunately, with the exception of a minor-ity of lesions (e.g., lipoma, hemangioma, subacute hematoma,pigmented villonodulam synovitis), the madiologic appearanceof most soft-tissue masses remains nonspecific [6]. Conse-quently, an appropriately ordered differential diagnosis basedon a lesion’s nadiologic appearance is difficult, if not impossi-ble. This difficulty is compounded by the seemingly endless listof diagnostic possibilities presented in the literature. In anattempt to provide a framework from which to approach thisproblem, a retrospective review of all soft-tissue lesions seenby our soft-tissue pathology department was undertaken todetermine the prevalence and distribution of each lesion, aswell as the tumor distribution for specific age groups and loca-tions. To my knowledge, an analysis of this type and scopehas not been previously reported.

A number of difficulties are inherent in a review of thisnature. The large number of patients and the extended periodoven which they were seen in consultation make it virtuallyimpossible for a single pathologist to assume responsibility forall histologic diagnoses on to review the histologic material forthe entire study group. All material was, however, reviewed bya staff pathologist in the Department of Soft-Tissue Pathology,Armed Forces Institute of Pathology, who has expertise in theevaluation of soft-tissue tumors. No histologic material wasreviewed for this study, and diagnoses are as coded by theoriginal pathologist. No attempt was made to reclassify lesionsor to change diagnoses.

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AJR:164, February 1995 BENIGN SOFT-TISSUE TUMORS 401

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402 KRANSDORF AJR:164, February 1995

There is an inherent bias in any referral population. Theconsultative nature of the cases also introduces the potentialfor a significant bias with a preference for difficult case mate-rial. This may be responsible for the relatively high percentageof malignant tumors (approximately 38%). This percentage isgreater than the 15.5% noted by Lattes [7], citing records fromColumbia University during the 45 1/2 years from February 1,1 906, to September 1 , 1951 (1 349 malignant and 7337 benignlesions), and considerably greater than the 5.1% reported byMyhne-Jensen [8] during the 7-year period from April 1970 toApril 1977 (72 malignant and 1331 benign lesions), atthe Uni-versity Institute of Pathology, Aarhus, Denmark. This discrep-

ancy was especially great in lesions such as lipoma andganglion. In the series reported by Myhne-Jensen [8], lipomarepresented almost half of all lesions. Lipomas represent 16%of the lesions in the current study. Ganglion has been reportedto represent as many as 33-60% [9, 10] of all hand tumors,but comprised only 3% of the hand and wrist lesions, and lessthan 1% of all lesions in this study. It is not surprising that suchlesions would be uncommon in a referral population.

Review of the relative predilection of tumors for specificlocations and age groups shows that 80% of lesions (range,

63-100%), can be placed in seven diagnostic groups. Forexample, in the retnopenitoneum, lipoblastoma is the mostcommon benign tumor in children 5 years old or less. Althoughlipoblastoma is a relatively rare lesion, comprising only 0.6%of all benign tumors, it makes up 37% of all benign tumors inthis age group. Some of the diagnoses listed as “common”may be unfamiliar to radiologists. It must be emphasized thatthe data in Tables 1 and 2 reflect lesions found at biopsy. Manysmall superficial lesions are excised on sampled without imag-ing. Lesions in this group include fibrous histiocytoma, nodularfasciitis, granuloma annulare, and so forth.

The purpose of this article is to establish the relative prey-alences of benign soft-tissue tumors and to identify prefenen-

tial locations and age groups for specific entities. When alesion’s imaging appearance is nonspecific, knowledge ofthe tumor’s prevalence, the patient’s age, and the location ofthe lesion will allow a suitably ordered differential diagnosis.

ACKNOWLEDGMENT

I am greatly in debt to Jeanne M. Meis-Kindblom for her assis-tance in the preparation of this manuscript. I also thank Joseph A.

Utz and James S. Jelinek for their editorial assistance, and TheaAmendola, Sheela Rao, and Pamela Kransdorf for their untiningresearch assistance.

REFERENCES

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sue masses with MR imaging: can benign masses be differentiated frommalignant ones? Radiology 1992:185:581-586

2. Berquist TH, Ehman RL, King BF, Hodgman CG, llstrup DM. Value of MRimaging in differentiating benign from malignant soft-tissue masses: studyof 95 lesions. AJR 1990;155:1251-1 255

3. Kransdort MJ, Jelinek JS, Moser RP, et al. Soft-tissue masses: diagnosis

using MR imaging. AJR1989;153:541-5474. Enzinger FM, Lattes R, Tonloni R. Histological typing of soft tissue tumors.

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6. Sundanam M, McLeod RA. MR imaging of tumor and tumorlike lesions ofbone and soft tissue. AJR 1990:155:817-824

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tumors: clinicopathologic data-comparison with sarcomas. Acta Orthop

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1975;108:214-22210. Butler ED, Hamill JP, Seipel RS, De Lormien AA. Tumors of the hand: a

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