Journal of the Royal Society of Medicine Volume 84 August 1991
Liposarcoma of the spermatic cord
499
Case presented to Clinical Section, 1 8 June 1990
F R Goodman MA MBBS M D Staunton MCh FRCS H C Rees MB BS MRCPath St Bartholomew's Hospital, London EClA 7BE Keywords: liposarcoma; spermatic cord; scrotum
Clinical experience teaches that, whereas testicular swellings are commonly solid and malignant, paratesticular swellings are usually cystic and benign. We report an exception: a case of spermatic cord liposarcoma, of which only 92 other instances are known. Case report An obese 60-year-old Caucasian male was referred in October 1987 with a painless, slowly enlarging left scrotal swelling, present for over one year. Examination showed a slightly tender solid scrotal mass, measuring approximately 10 x 10 x 5 cm, with a normal testis beneath. Surgical exploration via a trans-scrotal incision revealeda soft, yellowish fatty mass, immediately above the testis. Exposure of the inguinal canal showed that the spermatic cord within the canal wai normal, and that the mass, which apparently arose from the intra-scrotal portion of the cord; was separate from the peritoneum and retroperitoneum. Left radical orchidectomy was performed, with total excision of the mass. Postoperatively, computed axial tomography scans of the abdomen and chest revealed no evidence of metastatic spread. Regular follow-up was instituted, and in December 1990, a small local recurrence was detected and excised. The patient remains free of metastatic disease. The first specimen (October 1987) consisted of a lobulated, reasonably well-circumscribed mass, 14x14x6 cm, attached to the testis, with a non-uniform cut surface, composed of soft yellowish and firmer whitish areas, Microscopy revealed a well-differentiated liposarcoma of the sclerosing subtype. Most areas contained lipoblasts resembling lipocytes, but, with pleomorphic hyperchromatic nuclei. Amongst these were multinucleate lipoblasts and arborizing small vessels. The whitish areas showed sclerosis and myxoid change, with pleomorphic spindle cells, scattered lipoblasts and a plexiform capillary network (see Figures l and 2). The testis showed no evidence of infiltration, and excision appeared complete. The second specimn (December 1990) likewise had the appearance of a well-differentiated liposarcoma.
Figure 1. Photomicrograph x50. H & E stain. Sclerotic area showing pleomorphic spindle-shaped cells, prominent capillaries and lipoblasts resembling lipocytes.
Correspondence to M Dudley Staunton, 80 Harley Street, London WlN 1DE
Figure 2. Photomicrograph x 125. H & E stain. Area showing multivacuolatedpleomorphic lipoblasts with hyperchromaticpleomorphic nuclei
Discussion The clinical picture presented here is highly unusual: a ma which was truly scrotal, separate from the testis, solid and malignant. Paratesticular malignancies are rare, comprising mainly sarcomas originating from spermatic cord mesenchyme'. Undifferentiated sarcoma and embryonal rhabdomyosarcoma predominate amongst children, while fibrosarcoma, leiomyosarcoma and malignant fibrous histiocytoma _predominate amongst adults. Liposarcoma, the commonest soft-tissue sarcoma of adult life, usually arises in the extremities and retroperitoneum2. Only 92 cases of spermatic cord liposarcoma have previously been reported3`8 (remaining references available on request). Patients are commonly in their fifties or sixties, with an age range of 16-88 years. The tumour occurs world-wide, although there is a remarkably high incidence amongst Japanese men, who account for 24 of the 93 published cases4". It almost always arises de novo: simple lipomas, which constitute about 80% of spermatic cord neoplasms, virtually never undergo malignant transformation2. The presentation described here is fairly typical, although the ma mnay be inguinal rather than scrotal, and may attain giant proportions (up to 13.5 kg). Clinical assessment is difficult, with inguinal hernia, hydrocele and chronic epididymitis being the most common differential diagnoses. An ultrasound scan is helpful prior to exploratory. surgery, to confirm that the mass is solid and paratesticular. Histologically, almost all spermatic cord liposarcomas are well-differentiated (as here), or, more rarely, myxoid. These are both relatively low-grade malignancies with little tendency to metastasize, although they may be locally invasive2. High-grade round cell and pleomorphic spermatic cord liposarcomas, which frequently metastasize via the regional lymph nodes, have been reported in only 16 patients, of whom four died3'6'9"0 The treatment of choice is radical orchidectomy- with high ligation of the spermatic cord3, as was performed in this case. Retroperitoneal lymph node dissection is not indicated unless there is evidence of-tumour spread. Surgical resection may be inadequate due to the presence of a 'pseudo-casule' of compressed normal tissue, through which tumour cells may infiltrate into adjacent structures. Twelve other local recurrences have been reported, between one and 16 years after surgery378, although the short follow-up period in most reports may mean that the true figure is higher. All were successfully treated. by further surgery, radiotherapy or chemotherapy. Spermatic cord liposarcoma is a rare low-grade malignancy with an excellent prognosis following radical orchidectomy. Nevertheless, in view of the risk of- local recurrence, longterm follow-up is mandatory. References 1 Gowing NFC, Morgan AD. Paratesticular tumours of connective tissue and muscle. Br J Urol 1964;34(suppl):78-84 2 Enziger FM, Weiss SW. Soft tiue tumours, 2nd edn. St Louis:
CV Mosby Co., 1988:346-82
0141-0768/91/ 080499-02/$02.00/0 © 1991 The Royal Society of Medicine
500
Journal of the Royal Society of Medicine Volume 84 August 1991 7 Chan YF, Yuen MYP, Tung Ma L, Li MK. Recurrent dedifferentiated liposarcoma of the spermatic cord simulating malignant fibrous histiocytoma. Pathology 1987;19:99-102 8 Rieu JP, Catala F, Boyer JL, Carton M. Liposarcoma of the spermatic cord. Ann Urol (Paris) 1988;22:137-8 9 D'Abrera VS, Burfitt-Williams W. A giant scrotal liposarcoma. Med J Aust 1973;2:854-6 10 Garcia AE, Martin GG, Torrenti H, Monserrat JM. Liposarcoma of the spermatic cord. Rev Argent Urol 1968;37:44-8
3 Vorstman B, Block NL, Politano VA. The management of spermatic cord liposarcomas. J Urol 1984;131:66-9 4 Ishida A, Takeuchi H, Tomoyoshi T. Giant liposarcoma of the spermatic cord: report of a case. Acta Urol Japan 1985;31: 1059-64 5 Hoshino T, Yajima M, Iwasaki A, Hirokawa M, Matsushita K. Liposarcoma of the spermatic cord: a case report. Acta Urol Japan 1987;33:1296-9 6 Mhiri MN, Sellami F, Sellami M, Ben Hamed Y, Smida ML. Malignant paratesticular tumours apropos of three unusual cases. Ann Urol (Paris) 1989;23:23-26
(Accepted 7 March 1991)
Neuroleptic malignant syndrome: a diagnostic dilemma
management only. The event was accompanied by a rise in the CPK to a peak of 1417 IU/l (normal range: 25-175).
S M Sagar BSc MRCP Department of Medicine, Greenwich District Hospital, Vanbrugh Hill, London SElO 9HE Keywords: neuroleptic; catatonia; rigidity; pyrexia; hypersensitivity
The neuroleptic malignant syndrome (NMS) is an idiosyncratic reaction following treatment with neuroleptic drugs such as haloperidol or fluphenazinel2. It is characterized by an extrapyramidal motor disorder resulting in muscular rigidity; altered thermoregulation resulting in pyrexia; and autonomic dysfunction causing sweating, tachycardia and labile blood pressure. There is often a rise in serum skeletal muscle creatinine phosphokinase (CPK)3. It has a mortality of 20%4 which is a consequence of late diagnosis since its features may be confused with signs of other organic and pyschiatric disorders5'6. Three cases of NMS diagnosed during one year at this district general hospital are reported.
Case reports Case 1 A 36-year-old man presented with sudden onset ofheadache. Examination revealed meningism and cerebral angiography demonstrated a subarachnoid haemorrhage. The patient became confused and aggressive and following a diagnosis of acute psychosis, 20 mg haloperidol 4 times daily for one week followed by 50 mg of fluphenazine intramuscularly with procyclidine were administered. After one week, he became catatonic and developed rigidity, pyrexia, diaphoresis, dehydration and a labile blood pressure. Serum urea and aspartate transaminase became elevated. No evidence of sepsis was found and a diagnosis of drug-induced extrapyramidal disorder was made. Despite intensive supportive therapy, the patient died.
Case 2 A 39-year-old man with Henoch-Schonlein purpura was treated with steroids and developed a paranoid psychosis. Twenty-five milligrams of fluphenazine was administered intramuscularly and repeated after 2 weeks. Two days afterwards, he became confused, pyrexial and developed a tachycardia, neck stiffness and catatonia. A lumbar puncture was normal. He recovered after one week with supportive
Correspondence to S M Sagar, Newfoundland Cancer Clinic, Health Sciences Centre, Prince Phillip Drive, St John's Newfoundland, AlB 3V6, Canada
Case 3 A 46-year-old schizophrenic man became acutely agitated. Remission had previously been maintained with intramuscular fluphenazine. This acute episode was treated with chlorpromazine and intravenous haloperidol with procyclidine. Five days later, he became drowsy, pyrexial and developed increasing muscle tone and coma. A pyrexia of 39.5°C, hypotension of 85/60 mmHg and a tachycardia of 130 beats per minute were recorded. He became dehydrated, unresponsive to painful stimuli and developed a generalized lead-pipe rigidity with a pill-rolling tremor. Further investigations included: serum urea 43.9 mmol/l, creatinine 464 mmol/l, haemoglobin 19.9 g/dl, white cell count 7.9x 10l/l. Blood cultures grew no organisms and a lumbar puncture was normal. Serum CPK rose to a peak of 1030 IU/1 and aspartate transaminase rose to 332 IU/1 (normal range: 10-40). A diagnosis of NMS was made and managed with intensive supportive care. After 10 days, the pyrexia remitted and renal function normalized, but catatonia persisted and the CPK remained elevated. Treatment with bromocriptine and dantrolene was commenced. After 5 weeks, the CPK had returned to normal, autonomic instability had resolved, but the patient remained rigid, mute and withdrawn. He was reviewed by a consultant psychiatrist who diagnosed a psychotic catatonic stupor and prescribed electroconvulsive therapy (ECT). After a single treatment, the rigidity diminished and the patient developed spontaneous movements and recovered his speech. After five ECT treatments, rigidity completely resolved and the patient became less withdrawn and could deliver a rational conversation.
Discussion NMS contains features of catatonia, but paradoxically is precipitated by a neuroleptic drug which would otherwise resolve a catatonic state secondary to a mental psychosis. In its extreme form, catatonia is a life-threatening condition which can be precipitated by psychiatric or organic disorders through an effect on limbic and striatal dopamine neuronal pathways7. Hypothalamic malfunction results in labile temperature and blood pressure control, and sustained muscle rigidity results in an elevated CPK and myoglobinaemia. An accurate psychiatric diagnosis is essential before commencing treatment with potent neuroleptics. However, the author has not seen NMS develop after administration of neuroleptic drugs as antiemetics in cancer patients treated with opiates or cytotoxic agents, although a case has recently been reported8. The development of signs which indicate NMS should be critically assessed by the physician. In particular, deterioration in conscious state and development of neck stiffness require the exclusion of meningitis or a subarachnoid haemorrhage. Pyrexia and hypotension resulting from autonomic instability must be differentiated from
0141-0768/91/ 080500-02/$02.00/0 © 1991
The Royal Society of Medicine
Liposarcoma of the spermatic cord
499
Case presented to Clinical Section, 1 8 June 1990
F R Goodman MA MBBS M D Staunton MCh FRCS H C Rees MB BS MRCPath St Bartholomew's Hospital, London EClA 7BE Keywords: liposarcoma; spermatic cord; scrotum
Clinical experience teaches that, whereas testicular swellings are commonly solid and malignant, paratesticular swellings are usually cystic and benign. We report an exception: a case of spermatic cord liposarcoma, of which only 92 other instances are known. Case report An obese 60-year-old Caucasian male was referred in October 1987 with a painless, slowly enlarging left scrotal swelling, present for over one year. Examination showed a slightly tender solid scrotal mass, measuring approximately 10 x 10 x 5 cm, with a normal testis beneath. Surgical exploration via a trans-scrotal incision revealeda soft, yellowish fatty mass, immediately above the testis. Exposure of the inguinal canal showed that the spermatic cord within the canal wai normal, and that the mass, which apparently arose from the intra-scrotal portion of the cord; was separate from the peritoneum and retroperitoneum. Left radical orchidectomy was performed, with total excision of the mass. Postoperatively, computed axial tomography scans of the abdomen and chest revealed no evidence of metastatic spread. Regular follow-up was instituted, and in December 1990, a small local recurrence was detected and excised. The patient remains free of metastatic disease. The first specimen (October 1987) consisted of a lobulated, reasonably well-circumscribed mass, 14x14x6 cm, attached to the testis, with a non-uniform cut surface, composed of soft yellowish and firmer whitish areas, Microscopy revealed a well-differentiated liposarcoma of the sclerosing subtype. Most areas contained lipoblasts resembling lipocytes, but, with pleomorphic hyperchromatic nuclei. Amongst these were multinucleate lipoblasts and arborizing small vessels. The whitish areas showed sclerosis and myxoid change, with pleomorphic spindle cells, scattered lipoblasts and a plexiform capillary network (see Figures l and 2). The testis showed no evidence of infiltration, and excision appeared complete. The second specimn (December 1990) likewise had the appearance of a well-differentiated liposarcoma.
Figure 1. Photomicrograph x50. H & E stain. Sclerotic area showing pleomorphic spindle-shaped cells, prominent capillaries and lipoblasts resembling lipocytes.
Correspondence to M Dudley Staunton, 80 Harley Street, London WlN 1DE
Figure 2. Photomicrograph x 125. H & E stain. Area showing multivacuolatedpleomorphic lipoblasts with hyperchromaticpleomorphic nuclei
Discussion The clinical picture presented here is highly unusual: a ma which was truly scrotal, separate from the testis, solid and malignant. Paratesticular malignancies are rare, comprising mainly sarcomas originating from spermatic cord mesenchyme'. Undifferentiated sarcoma and embryonal rhabdomyosarcoma predominate amongst children, while fibrosarcoma, leiomyosarcoma and malignant fibrous histiocytoma _predominate amongst adults. Liposarcoma, the commonest soft-tissue sarcoma of adult life, usually arises in the extremities and retroperitoneum2. Only 92 cases of spermatic cord liposarcoma have previously been reported3`8 (remaining references available on request). Patients are commonly in their fifties or sixties, with an age range of 16-88 years. The tumour occurs world-wide, although there is a remarkably high incidence amongst Japanese men, who account for 24 of the 93 published cases4". It almost always arises de novo: simple lipomas, which constitute about 80% of spermatic cord neoplasms, virtually never undergo malignant transformation2. The presentation described here is fairly typical, although the ma mnay be inguinal rather than scrotal, and may attain giant proportions (up to 13.5 kg). Clinical assessment is difficult, with inguinal hernia, hydrocele and chronic epididymitis being the most common differential diagnoses. An ultrasound scan is helpful prior to exploratory. surgery, to confirm that the mass is solid and paratesticular. Histologically, almost all spermatic cord liposarcomas are well-differentiated (as here), or, more rarely, myxoid. These are both relatively low-grade malignancies with little tendency to metastasize, although they may be locally invasive2. High-grade round cell and pleomorphic spermatic cord liposarcomas, which frequently metastasize via the regional lymph nodes, have been reported in only 16 patients, of whom four died3'6'9"0 The treatment of choice is radical orchidectomy- with high ligation of the spermatic cord3, as was performed in this case. Retroperitoneal lymph node dissection is not indicated unless there is evidence of-tumour spread. Surgical resection may be inadequate due to the presence of a 'pseudo-casule' of compressed normal tissue, through which tumour cells may infiltrate into adjacent structures. Twelve other local recurrences have been reported, between one and 16 years after surgery378, although the short follow-up period in most reports may mean that the true figure is higher. All were successfully treated. by further surgery, radiotherapy or chemotherapy. Spermatic cord liposarcoma is a rare low-grade malignancy with an excellent prognosis following radical orchidectomy. Nevertheless, in view of the risk of- local recurrence, longterm follow-up is mandatory. References 1 Gowing NFC, Morgan AD. Paratesticular tumours of connective tissue and muscle. Br J Urol 1964;34(suppl):78-84 2 Enziger FM, Weiss SW. Soft tiue tumours, 2nd edn. St Louis:
CV Mosby Co., 1988:346-82
0141-0768/91/ 080499-02/$02.00/0 © 1991 The Royal Society of Medicine
500
Journal of the Royal Society of Medicine Volume 84 August 1991 7 Chan YF, Yuen MYP, Tung Ma L, Li MK. Recurrent dedifferentiated liposarcoma of the spermatic cord simulating malignant fibrous histiocytoma. Pathology 1987;19:99-102 8 Rieu JP, Catala F, Boyer JL, Carton M. Liposarcoma of the spermatic cord. Ann Urol (Paris) 1988;22:137-8 9 D'Abrera VS, Burfitt-Williams W. A giant scrotal liposarcoma. Med J Aust 1973;2:854-6 10 Garcia AE, Martin GG, Torrenti H, Monserrat JM. Liposarcoma of the spermatic cord. Rev Argent Urol 1968;37:44-8
3 Vorstman B, Block NL, Politano VA. The management of spermatic cord liposarcomas. J Urol 1984;131:66-9 4 Ishida A, Takeuchi H, Tomoyoshi T. Giant liposarcoma of the spermatic cord: report of a case. Acta Urol Japan 1985;31: 1059-64 5 Hoshino T, Yajima M, Iwasaki A, Hirokawa M, Matsushita K. Liposarcoma of the spermatic cord: a case report. Acta Urol Japan 1987;33:1296-9 6 Mhiri MN, Sellami F, Sellami M, Ben Hamed Y, Smida ML. Malignant paratesticular tumours apropos of three unusual cases. Ann Urol (Paris) 1989;23:23-26
(Accepted 7 March 1991)
Neuroleptic malignant syndrome: a diagnostic dilemma
management only. The event was accompanied by a rise in the CPK to a peak of 1417 IU/l (normal range: 25-175).
S M Sagar BSc MRCP Department of Medicine, Greenwich District Hospital, Vanbrugh Hill, London SElO 9HE Keywords: neuroleptic; catatonia; rigidity; pyrexia; hypersensitivity
The neuroleptic malignant syndrome (NMS) is an idiosyncratic reaction following treatment with neuroleptic drugs such as haloperidol or fluphenazinel2. It is characterized by an extrapyramidal motor disorder resulting in muscular rigidity; altered thermoregulation resulting in pyrexia; and autonomic dysfunction causing sweating, tachycardia and labile blood pressure. There is often a rise in serum skeletal muscle creatinine phosphokinase (CPK)3. It has a mortality of 20%4 which is a consequence of late diagnosis since its features may be confused with signs of other organic and pyschiatric disorders5'6. Three cases of NMS diagnosed during one year at this district general hospital are reported.
Case reports Case 1 A 36-year-old man presented with sudden onset ofheadache. Examination revealed meningism and cerebral angiography demonstrated a subarachnoid haemorrhage. The patient became confused and aggressive and following a diagnosis of acute psychosis, 20 mg haloperidol 4 times daily for one week followed by 50 mg of fluphenazine intramuscularly with procyclidine were administered. After one week, he became catatonic and developed rigidity, pyrexia, diaphoresis, dehydration and a labile blood pressure. Serum urea and aspartate transaminase became elevated. No evidence of sepsis was found and a diagnosis of drug-induced extrapyramidal disorder was made. Despite intensive supportive therapy, the patient died.
Case 2 A 39-year-old man with Henoch-Schonlein purpura was treated with steroids and developed a paranoid psychosis. Twenty-five milligrams of fluphenazine was administered intramuscularly and repeated after 2 weeks. Two days afterwards, he became confused, pyrexial and developed a tachycardia, neck stiffness and catatonia. A lumbar puncture was normal. He recovered after one week with supportive
Correspondence to S M Sagar, Newfoundland Cancer Clinic, Health Sciences Centre, Prince Phillip Drive, St John's Newfoundland, AlB 3V6, Canada
Case 3 A 46-year-old schizophrenic man became acutely agitated. Remission had previously been maintained with intramuscular fluphenazine. This acute episode was treated with chlorpromazine and intravenous haloperidol with procyclidine. Five days later, he became drowsy, pyrexial and developed increasing muscle tone and coma. A pyrexia of 39.5°C, hypotension of 85/60 mmHg and a tachycardia of 130 beats per minute were recorded. He became dehydrated, unresponsive to painful stimuli and developed a generalized lead-pipe rigidity with a pill-rolling tremor. Further investigations included: serum urea 43.9 mmol/l, creatinine 464 mmol/l, haemoglobin 19.9 g/dl, white cell count 7.9x 10l/l. Blood cultures grew no organisms and a lumbar puncture was normal. Serum CPK rose to a peak of 1030 IU/1 and aspartate transaminase rose to 332 IU/1 (normal range: 10-40). A diagnosis of NMS was made and managed with intensive supportive care. After 10 days, the pyrexia remitted and renal function normalized, but catatonia persisted and the CPK remained elevated. Treatment with bromocriptine and dantrolene was commenced. After 5 weeks, the CPK had returned to normal, autonomic instability had resolved, but the patient remained rigid, mute and withdrawn. He was reviewed by a consultant psychiatrist who diagnosed a psychotic catatonic stupor and prescribed electroconvulsive therapy (ECT). After a single treatment, the rigidity diminished and the patient developed spontaneous movements and recovered his speech. After five ECT treatments, rigidity completely resolved and the patient became less withdrawn and could deliver a rational conversation.
Discussion NMS contains features of catatonia, but paradoxically is precipitated by a neuroleptic drug which would otherwise resolve a catatonic state secondary to a mental psychosis. In its extreme form, catatonia is a life-threatening condition which can be precipitated by psychiatric or organic disorders through an effect on limbic and striatal dopamine neuronal pathways7. Hypothalamic malfunction results in labile temperature and blood pressure control, and sustained muscle rigidity results in an elevated CPK and myoglobinaemia. An accurate psychiatric diagnosis is essential before commencing treatment with potent neuroleptics. However, the author has not seen NMS develop after administration of neuroleptic drugs as antiemetics in cancer patients treated with opiates or cytotoxic agents, although a case has recently been reported8. The development of signs which indicate NMS should be critically assessed by the physician. In particular, deterioration in conscious state and development of neck stiffness require the exclusion of meningitis or a subarachnoid haemorrhage. Pyrexia and hypotension resulting from autonomic instability must be differentiated from
0141-0768/91/ 080500-02/$02.00/0 © 1991
The Royal Society of Medicine
