- Email: [email protected]
Primary neurogenic sarcoma of the forearm following trauma at golf
PRIMARY
NEUROGENIC
SARCOMA
FOLLOWING
TRAUMA
OF THE FOREARM AT GOLF
CASE REPORT SAUL Assistant
ALFRED
Attending NEW
I
YORK,
solitary neurogenic sarcoma of periphera1 nerves unassociated with von ReckIinghausen’s disease is uncommon. Its occurrence is even more unusua1 folIowing a definite history of trauma. The accessibiIity of these tumors generally leads one to minimize their serious nature and most often to mistake them for benign growths. It is rareIy diagnosed as maIignant until pathologica examination has been made and in many cases, the tumor smaI1 or Iarge is removed without considering the necessity for radica1 extirpation at the time. It is difhcuIt to determine whether these isoIated growths arise from the neuriIemma, perineurium or epineurium, but the connective tissue nature of these growths make it probabIe that they originate from either of these structures.’ Stout2 maintains that there are two classes of maIignant tumors that deveIop in periphera1 nerves; those of mesoblastic origin and those derived from neuro-epithelium. PenfieId3 and MaIIory4 beIieve that these tumors arise from the supporting endoneura1 connective tissue and are, therefore, of mesodermal origin. Masson and Verocay6 are of the opinion that the majority of these neopIasms originate from the Schwann ceIIs of nerve sheaths, which have their anIage from the neural crest, hence are of ectoderma1 origin. Neurofibrosarcoma usuaIIy deveIops in superficial and deep nerve trunks. The tumors are composed of varying numbers and sizes of ceIIs, the more ceIIuIar forms being the more maIignant. SOLATED
RITTER,
M.D.
Surgeon, Misericordia NEW
HospitaI
YORK
Quick and CutIer’ have graded these tumors into three different varieties, designating Grade I, II and III, depending on
FIG. I. Volar-lateral aspect of left forearm showing area excised and covered by a Wolf skin graft.
their ceIIuIar content. The more aceIIuIar group I, being the more benign type and grade III, being the most maIignant. Fischer,* in 1927, reviewed the Iiterature on von ReckIinghausen’s disease, and of the 466 cases reported up to that time found that 13 per cent were compIicated by sarcomatous degeneration in the nerve tumors.
502
American Journal of Surgery
Ritter-Sarcoma
between GarrGg studied th .e differences primary s:arcomas of ’ nerves and secondary von sarcomas compIic :at ;ing pre-existing
of Forearm neurosarcoma arm due to before.
MAY, 194.z
of the nnedian ler -ve of the definite traum a t wo Y ears
FIG. 2. Illustration showing a spindIe cell sarcoma with considerable mucoid degeneration between the celIs. Mitotic figures are not abundant. The arrangement of the ceIIs very strongIy suggests a neurogenic sarcoma and severa nerve bundIes, present in the substance of the tumor, are suggestive of the site of origin for the neoplasm.
ReckIinghausen’s disease and found the Iatter more prone to remain encapsuIated for Ionger periods of time. He reported the former to be of greater maIignancy and metastasize earIier to interna organs. Quick and CutIer,’ reported that fourteen patients out of seventy two with neurogenic sarcoma unassociated with von ReckIinghausen’s disease, gave a history of trauma, but stress that they beIieve that chronic irritation or repeated traumatic insuIts are more important if trauma as an etioIogy can be considered. Stewart and CopeIand,‘O on the other hand, record eight instances of trauma among their eighty-three cases, but they beIieve “in no case is the anaIysis suffxcient to make the traumatic etioIogy secure beyond doubt.” Cramer, l1 in 1929, showed that chronic irritation by tar painting produced this form of tumor which recurred after excision. Biggs12 reported the occurrence of a
Most authors insist that radica1 onestage wide excision or amputation shouId be performed and unIess compIete extirpation is done earIy, there is great danger of recurrence and metastasis. Many authors on this subject point to the reIative rarity of the neurogenic sarcomas and their benign appearance and that the genera1 surgeon does not encounter them frequentIy enough to be famiIiar with their highIy malignant nature. Quick and CutIer’ out of twenty-four cases had a mortaIity (excIusive of tumors of the thigh) of 50 per cent from metastases. Of the cases of tumors of the thigh the result was still worse; onIy two out of fifteen are stiI1 Iiving from three to nine years after operation. Twenty per cent of a11 their patients with neurogenic sarcoma died from puImonary metastases. The case of soIitary neurogenic sarcoma of the forearm which I wish to report is unique and significant. It foIIowed a
NEW
SERIES
Ritter-Sarcoma
VOL. LVI, No. 2
definite trauma to the left forearm the patient was playing golf. CASE
whiIe
REPORT
RI. B., twenty-five years of age, American, complained of pain and swelIing on the volar aspect of the left forearm, since striking it with the end of a golf club while driving a golf bail five w-eeks before. He experienced pains in the fIexor aspect of the Ieft forearm which radiated up to the arm and down to the fingers. At first he paid Iittle attention to the injury. He did notice, however, the deveIopment of a small nodule on the volar aspect of the forearm. It was painfu1 and tender. Upon examination, I found at the volar surface of the Ieft forearm four inches proximal to the wrist. joint, a firm, tender, subcutaneous tumor, the size of a smaI1 haze1 nut. It was fixed to the underIying tissues but the skin over it was freely movable. There was no adenopathy of the epitrochIear, axillary, subclavicular or cervica1 gIands. On October 15, 1938, under IocaI anesthesia, a biopsy of the growth was made. The specimen was submitted for pathoIogica1 examination to Dr. R. C. Scleussner who reported spindIe cel1 sarcoma. Sections were reviewed by Dr. Francis Carter Wood, Dr. PauI Klemperer and Dr. H. R. MuIIer who confirmed the pathoIogica1 findings of spindIe ceI1 sarcoma of neurogenic origin. On November 3, 1938, at Doctors Hospital, I performed a wicIe excision of the tissues of the volar-IateraI region of the forearm, encircIing the site of the biopsy. The ova1 area excised measured 3)s by IO inches. It incIuded the skin, superficia1 fascia and nerves, deep fascia and a portion of the muscuIar tissue at the site of the tumor. It was excised en masse. There were gray strands of neopIastic tissue invading the deep fascia at the site of the tumor. A Wolf skin graft was removed from the proxima1 aspect of the left thigh and grafted over the exposed region of the forearm. It was sutured by fine silk interrupted atraumatic sutures. A spIint was appIied to the posterior aspect of the arm which was flexed at right angles. The Wolf skin graft heaIed compIeteIy within two weeks with the exception of a few smaI1 hemorrhagic bIebs which became necrotic.
of Forearm
American
Journal
of Suryery
303
These areas were Iater covered with smaI1 Thiersch grafts. CompIete heaIing resuIted shortly thereafter. The skin over the operated area became pigmented but was eIastic and freeIy movabIe. There were no adhesions between the origina skin graft and the ffexor tendons of the forearm. There was fuI1 mobility of the fingers and wrist. Two and a half years after operation, the patient has some periodic neuraIgic pains in the Ieft forearm. There remains cutaneous anesthesia over the grafted area, but he has normal use of the Iimb. Roentgen ray examination of the bones of the body does not revea1 any metastases. There is no adenopathy and the patient is in apparent good health. SUMMARY I. A unique case of solitary neurogenic sarcoma of the left forearm, folIowing a definite history of trauma at goIf, is reported. 2. An early biopsy and radica1 excision were performed. 3. Two and a half years following operation there is no evidence of metastases. 4. No postoperative radiation was administered because of insensitivity of this neoplasm to radiation.
5. EarIy recognition and radicaI extirpation of neurogenic spindIe ceII sarcoma of a limb seem to obviate the necessity for amputation in these highly maIignant Iesions. REFERENCES I.
2.
3. 4. 5, 6. 7.
VON
RECKLINGHAUSEN,
F.
Uber
die
multiplen
Fibrome der Haut und irhre Beziehune zu den multipIen Neuron. Berlin, 1882. A. Hir&hwald. STOUT, A. P. Am. .I. Cancer, 25: I, 1935. PENFIELD. Surg., Cynec. e’” Oh., 45 : 178, 1927. MALLORY, A. J. Metab. Research, 16: 34. 1920. hbtSSON, P. Am. J. Patb., 3: 367, 1932. VERVCAY, J. Beitr. z. patb. Anat. und. x. a&g. Path., 48: I, ,910. QLXK, D. and CLTLER, M. Ann. Surp., 86: 810,
1927. 8. FISCHER, G. A. Dermat.
Wcbnscbr., 84: 9. GARR~, C. Beitr. z. klin. Cbir., 9: 465, STEWART, F. W. and COPELAND, M. 10. Cancer, 15: 1235, 1931. I I. CRAMER, W. Brit. J. Expel. Patb., IO:
89, 1927. 1892. hf. Am. J. 335,
1929.
NEUROGENIC
SARCOMA
FOLLOWING
TRAUMA
OF THE FOREARM AT GOLF
CASE REPORT SAUL Assistant
ALFRED
Attending NEW
I
YORK,
solitary neurogenic sarcoma of periphera1 nerves unassociated with von ReckIinghausen’s disease is uncommon. Its occurrence is even more unusua1 folIowing a definite history of trauma. The accessibiIity of these tumors generally leads one to minimize their serious nature and most often to mistake them for benign growths. It is rareIy diagnosed as maIignant until pathologica examination has been made and in many cases, the tumor smaI1 or Iarge is removed without considering the necessity for radica1 extirpation at the time. It is difhcuIt to determine whether these isoIated growths arise from the neuriIemma, perineurium or epineurium, but the connective tissue nature of these growths make it probabIe that they originate from either of these structures.’ Stout2 maintains that there are two classes of maIignant tumors that deveIop in periphera1 nerves; those of mesoblastic origin and those derived from neuro-epithelium. PenfieId3 and MaIIory4 beIieve that these tumors arise from the supporting endoneura1 connective tissue and are, therefore, of mesodermal origin. Masson and Verocay6 are of the opinion that the majority of these neopIasms originate from the Schwann ceIIs of nerve sheaths, which have their anIage from the neural crest, hence are of ectoderma1 origin. Neurofibrosarcoma usuaIIy deveIops in superficial and deep nerve trunks. The tumors are composed of varying numbers and sizes of ceIIs, the more ceIIuIar forms being the more maIignant. SOLATED
RITTER,
M.D.
Surgeon, Misericordia NEW
HospitaI
YORK
Quick and CutIer’ have graded these tumors into three different varieties, designating Grade I, II and III, depending on
FIG. I. Volar-lateral aspect of left forearm showing area excised and covered by a Wolf skin graft.
their ceIIuIar content. The more aceIIuIar group I, being the more benign type and grade III, being the most maIignant. Fischer,* in 1927, reviewed the Iiterature on von ReckIinghausen’s disease, and of the 466 cases reported up to that time found that 13 per cent were compIicated by sarcomatous degeneration in the nerve tumors.
502
American Journal of Surgery
Ritter-Sarcoma
between GarrGg studied th .e differences primary s:arcomas of ’ nerves and secondary von sarcomas compIic :at ;ing pre-existing
of Forearm neurosarcoma arm due to before.
MAY, 194.z
of the nnedian ler -ve of the definite traum a t wo Y ears
FIG. 2. Illustration showing a spindIe cell sarcoma with considerable mucoid degeneration between the celIs. Mitotic figures are not abundant. The arrangement of the ceIIs very strongIy suggests a neurogenic sarcoma and severa nerve bundIes, present in the substance of the tumor, are suggestive of the site of origin for the neoplasm.
ReckIinghausen’s disease and found the Iatter more prone to remain encapsuIated for Ionger periods of time. He reported the former to be of greater maIignancy and metastasize earIier to interna organs. Quick and CutIer,’ reported that fourteen patients out of seventy two with neurogenic sarcoma unassociated with von ReckIinghausen’s disease, gave a history of trauma, but stress that they beIieve that chronic irritation or repeated traumatic insuIts are more important if trauma as an etioIogy can be considered. Stewart and CopeIand,‘O on the other hand, record eight instances of trauma among their eighty-three cases, but they beIieve “in no case is the anaIysis suffxcient to make the traumatic etioIogy secure beyond doubt.” Cramer, l1 in 1929, showed that chronic irritation by tar painting produced this form of tumor which recurred after excision. Biggs12 reported the occurrence of a
Most authors insist that radica1 onestage wide excision or amputation shouId be performed and unIess compIete extirpation is done earIy, there is great danger of recurrence and metastasis. Many authors on this subject point to the reIative rarity of the neurogenic sarcomas and their benign appearance and that the genera1 surgeon does not encounter them frequentIy enough to be famiIiar with their highIy malignant nature. Quick and CutIer’ out of twenty-four cases had a mortaIity (excIusive of tumors of the thigh) of 50 per cent from metastases. Of the cases of tumors of the thigh the result was still worse; onIy two out of fifteen are stiI1 Iiving from three to nine years after operation. Twenty per cent of a11 their patients with neurogenic sarcoma died from puImonary metastases. The case of soIitary neurogenic sarcoma of the forearm which I wish to report is unique and significant. It foIIowed a
NEW
SERIES
Ritter-Sarcoma
VOL. LVI, No. 2
definite trauma to the left forearm the patient was playing golf. CASE
whiIe
REPORT
RI. B., twenty-five years of age, American, complained of pain and swelIing on the volar aspect of the left forearm, since striking it with the end of a golf club while driving a golf bail five w-eeks before. He experienced pains in the fIexor aspect of the Ieft forearm which radiated up to the arm and down to the fingers. At first he paid Iittle attention to the injury. He did notice, however, the deveIopment of a small nodule on the volar aspect of the forearm. It was painfu1 and tender. Upon examination, I found at the volar surface of the Ieft forearm four inches proximal to the wrist. joint, a firm, tender, subcutaneous tumor, the size of a smaI1 haze1 nut. It was fixed to the underIying tissues but the skin over it was freely movable. There was no adenopathy of the epitrochIear, axillary, subclavicular or cervica1 gIands. On October 15, 1938, under IocaI anesthesia, a biopsy of the growth was made. The specimen was submitted for pathoIogica1 examination to Dr. R. C. Scleussner who reported spindIe cel1 sarcoma. Sections were reviewed by Dr. Francis Carter Wood, Dr. PauI Klemperer and Dr. H. R. MuIIer who confirmed the pathoIogica1 findings of spindIe ceI1 sarcoma of neurogenic origin. On November 3, 1938, at Doctors Hospital, I performed a wicIe excision of the tissues of the volar-IateraI region of the forearm, encircIing the site of the biopsy. The ova1 area excised measured 3)s by IO inches. It incIuded the skin, superficia1 fascia and nerves, deep fascia and a portion of the muscuIar tissue at the site of the tumor. It was excised en masse. There were gray strands of neopIastic tissue invading the deep fascia at the site of the tumor. A Wolf skin graft was removed from the proxima1 aspect of the left thigh and grafted over the exposed region of the forearm. It was sutured by fine silk interrupted atraumatic sutures. A spIint was appIied to the posterior aspect of the arm which was flexed at right angles. The Wolf skin graft heaIed compIeteIy within two weeks with the exception of a few smaI1 hemorrhagic bIebs which became necrotic.
of Forearm
American
Journal
of Suryery
303
These areas were Iater covered with smaI1 Thiersch grafts. CompIete heaIing resuIted shortly thereafter. The skin over the operated area became pigmented but was eIastic and freeIy movabIe. There were no adhesions between the origina skin graft and the ffexor tendons of the forearm. There was fuI1 mobility of the fingers and wrist. Two and a half years after operation, the patient has some periodic neuraIgic pains in the Ieft forearm. There remains cutaneous anesthesia over the grafted area, but he has normal use of the Iimb. Roentgen ray examination of the bones of the body does not revea1 any metastases. There is no adenopathy and the patient is in apparent good health. SUMMARY I. A unique case of solitary neurogenic sarcoma of the left forearm, folIowing a definite history of trauma at goIf, is reported. 2. An early biopsy and radica1 excision were performed. 3. Two and a half years following operation there is no evidence of metastases. 4. No postoperative radiation was administered because of insensitivity of this neoplasm to radiation.
5. EarIy recognition and radicaI extirpation of neurogenic spindIe ceII sarcoma of a limb seem to obviate the necessity for amputation in these highly maIignant Iesions. REFERENCES I.
2.
3. 4. 5, 6. 7.
VON
RECKLINGHAUSEN,
F.
Uber
die
multiplen
Fibrome der Haut und irhre Beziehune zu den multipIen Neuron. Berlin, 1882. A. Hir&hwald. STOUT, A. P. Am. .I. Cancer, 25: I, 1935. PENFIELD. Surg., Cynec. e’” Oh., 45 : 178, 1927. MALLORY, A. J. Metab. Research, 16: 34. 1920. hbtSSON, P. Am. J. Patb., 3: 367, 1932. VERVCAY, J. Beitr. z. patb. Anat. und. x. a&g. Path., 48: I, ,910. QLXK, D. and CLTLER, M. Ann. Surp., 86: 810,
1927. 8. FISCHER, G. A. Dermat.
Wcbnscbr., 84: 9. GARR~, C. Beitr. z. klin. Cbir., 9: 465, STEWART, F. W. and COPELAND, M. 10. Cancer, 15: 1235, 1931. I I. CRAMER, W. Brit. J. Expel. Patb., IO:
89, 1927. 1892. hf. Am. J. 335,
1929.