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Primary fibrosarcoma of the diaphragm
BY. J. Dis.
PRIMARY
FIBROSARCOMA DIAPHRAGM
COSTAS G. SBOKOS, F. D. SALAMA, Wessex
Cardiac
V. POWELL
and Thoracic
Centre,
Chest
(1977)
71, 49
OF THE AND I. K. R. MCMILLAN Southampton
Summary Primary tumours of the diaphragm are rare. Benign neoplasms occur more malignant ones. Among the latter, fibrosarcoma is the most common. A case sarcoma of the diaphragm is presented with long survival and recurrence 10 years removal. At re-operation, a huge tumour was excised arising from a small part The histology of the recurrence was the same as the original tumour. The patient signs and symptoms nine months after the second operation.
frequently than of primary fibroafter first surgical of the diaphragm remained free of
INTRODUCTION
Any of the normal malignant tumour. tumours from the porting a case of collecting another 3 : 2, although it is when they involve
tissue components of the diaphragm may give rise to a benign or Wiener and Chou (1965) collected 70 cases of primary diaphragmatic world literature and added another one. Olafsson et al. (1971), reundifferentiated sarcoma of the diaphragm, reviewed the literature, 13 cases. The ratio of benign to malignant tumours appears to be about admittedly difficult in special cases to draw a sharp line between them the diaphragm.
Case Report A 51-year-old woman was admitted to hospital on 10 August 1965 complaining of pain in the lower left chest, worse on inspiration. Physical examination and laboratory studies were normal. Chest radiograph showed a left pleural effusion and a rounded basal shadow situated in the left cupola anteriorly. On fluoroscopic examination, the lesion moved with the diaphragm. Another chest radiograph five days later showed that this effusion had disappeared, but the shadow (an oval, well-defined mass) was considerably larger. Bronchoscopy, respiratory function and bronchogram were normal. At operation (V. P.), a slightly pear-shaped tumour, measuring 6 x 7 x 6 cm and arising from the diaphragm, was resected with part of the left hemidiaphragm and adherent flattened edge of lung and pericardium. There was no invasion of adjacent organs. The tumour had a thin capsule. Histology revealed a cellular differentiated fibrosarcoma without invasion of adjacent tissues. The general features suggested a low grade of malignancy. The patient remained well for nine years. However, progressive increase in shadowing at the left base was noticed on successive chest radiographs taken over the last two years before admission to the hospital. Symptoms began to recur over the last few months, with pain in the lower chest, a sense of fullness and heartburn on eating and occasional vomiting. The pain was a continuous dull ache. On examination, the patient appeared to be healthy. A very large mass was easily palpable in her left hypochondrium, extending upwards to invade the anteriolateral chest wall. The tumour was not mobile or tender. There was no clubbing or hypertrophic osteoarthropathy. I
Costas G. Sbokas, F. D. Salama, V. Powell and I. K. R. MeMilan A chest radiograph demonstrated a dense opacity at the left lower chest, extending mainly into the abdomen. Intravenous pyelogram was normal, although the kidney was a little displaced medially. Bronchoscopy, oesophagoscopy and gastroscopy were normal. An aortogram was performed and showed no involvement of any of the major vessels. A barium meal showed the stomach to be pushed over to the right but not invaded (Fig. 1).
Fig. 1. A barium
meal
revealed
displacement hypochondria1
of the mass
stomach
to the
right
by
a left
Exploration was carried out, through a left thoracoabdominal approach (I.K.M., January 1975). An enormous ovoid lobulated tumour, measuring 22 x 22 x 13 cm was found in the upper abdomen, extending up into the left chest. The surrounding structures were only adherent to it, but not invaded. The stomach, the omentum, the spleen,’ the left lobe of the liver and the left lower lobe of the lung were peeled off it without difficulty. It arose from a small area in the peripheral left diaphragm and appeared to be thinly encapsulated over most of its surface. No glands were involved and there was no evidence of metastasis or any other tumour in the abdomen or the left chest. It weighed 4 kg (Fig. 2). Histology revealed a fibrosarcoma with a liposarcomatous element. It was composed of interweaving spindle cells of pleomorphic appearance. A moderate degree of acid mucopolysaccharide was present in the stroma and typical of connective tissue mucin. Mitoses were fairly frequent and avascular necrosis was occurring (Fig. 3). The patient stood the operation well and made an uneventful recovery. She is fit and well at the time of writing.
51
Primary Fibvosavcoma of the Diaphragm
2. External
appearance muscular
of the tumour. A very large tissue of the diaphragm is seen
ovoid lobulated at the resected
Fig. 3. Histological
of the
appearance
tumour
tumour. margin
Portion
of
1:he
52
Costas G. Sbokas, F. D. Salama, V. Powell and I. K. R. McMillan DISCUSSION
Fibrosarcoma is the commonest of malignant diaphragmatic tumours, constituting 27-30% of cases. It may occur at any age, although it is more common in middle age. There is no sex prevalence. It is slightly commoner on the left. Pleuritic chest pain or pain referred to the epigastrium is the most common complaint. The onset, duration and severity vary considerably. The pain is usually aggravated by deep breathing or physical activity and may radiate to the shoulder or the neck, suggesting phrenic nerve irritation. Gastrointestinal symptoms may be caused by left-sided tumours and this was the most disturbing symptom in our case. Hypertrophic pulmonary osteoarthropathy is not very common, occuring in 10% of the cases (Nicholson & Whitehead 1956; Wiener & Chou 1965; Olafsson et al. 1971). Such tumours are usually detected in chest radiographs but their radiological appearances are not characteristic. The usual picture is that of an intrathoracic lesion adjacent to the diaphragm or a subdiaphragmatic condition elevating or deforming the diaphragm. Pneumoperitoneum can be very helpful in the differential diagnosis, particularly from big diaphragmatic hernias (Crimm & Liechle 1952; Sarot et al. 1969). Fibrosarcoma of the diaphragm has been reported to be a relatively small tumour, growing rapidly and metastatizing early (Crimm & Liechle 1952; Wiener & Chou 1965). The longest survival recorded is five years (Drewes & Willmann 1955). Our case survived for 10 years after the first operation and the recurrence, although enormous in size, was not accompanied by any distant metastases. The presence of a liposarcomatous element is an interesting histological feature in our case; this variety has not been recorded before (Kalen 1970). Radiotherapy is not usually beneficial. As it is impossible to decide before operation about the true nature of diaphragmatic tumours, and as their size and even recurrence is no indication of inoperability or the degree of malignancy, surgical intervention is considered to be the treatment of choice. ACKNOWLEDGEMENT
We would like to thank Dr James Bowe who referred the patient.
Requests for reprints should be addressed to I. K. R. McMillan, Hospital, Oakley Road, Southampton SO9 4 WQ.
Southampton
Western
REFERENCES P. 0. & LIECHLE, F. L. (1952) Fibrosarcoma of the diaphragm. J. thorac. Surg. 23, 360. J. & WILLMANN, K. H. (1955) Die Primiiren Tumoren des Zwerchfalls. Thoraxchivurgie 3, 75. KALEN, N. A. (1970) Lipoma of the diaphragm. Scund.3’. resp. LG. 51,28. NICHOLSON, F. & WHITEHEAD, R. (1956) Tumours of the diaphragm. BY. J. Swg. 43, 633. OLAFSSON, G., RAUSLING, A. & OLEN, 0. (1971) Primary tumours of the diaphragm. Chest 59, 568. SAROT, I. A., SCHWIMMER, D. & SCHLECHTER, D. G. (1969) Primary neurilemmoma of the diaphragm. N. Y. St.J. Med. 69, 837. WIENER, M. F. & CHOU, W. H. (1965) Primary tumours of the diaphragm. Archs Surg., Chicago 90, 143. CRIMM, DREWES,
PRIMARY
FIBROSARCOMA DIAPHRAGM
COSTAS G. SBOKOS, F. D. SALAMA, Wessex
Cardiac
V. POWELL
and Thoracic
Centre,
Chest
(1977)
71, 49
OF THE AND I. K. R. MCMILLAN Southampton
Summary Primary tumours of the diaphragm are rare. Benign neoplasms occur more malignant ones. Among the latter, fibrosarcoma is the most common. A case sarcoma of the diaphragm is presented with long survival and recurrence 10 years removal. At re-operation, a huge tumour was excised arising from a small part The histology of the recurrence was the same as the original tumour. The patient signs and symptoms nine months after the second operation.
frequently than of primary fibroafter first surgical of the diaphragm remained free of
INTRODUCTION
Any of the normal malignant tumour. tumours from the porting a case of collecting another 3 : 2, although it is when they involve
tissue components of the diaphragm may give rise to a benign or Wiener and Chou (1965) collected 70 cases of primary diaphragmatic world literature and added another one. Olafsson et al. (1971), reundifferentiated sarcoma of the diaphragm, reviewed the literature, 13 cases. The ratio of benign to malignant tumours appears to be about admittedly difficult in special cases to draw a sharp line between them the diaphragm.
Case Report A 51-year-old woman was admitted to hospital on 10 August 1965 complaining of pain in the lower left chest, worse on inspiration. Physical examination and laboratory studies were normal. Chest radiograph showed a left pleural effusion and a rounded basal shadow situated in the left cupola anteriorly. On fluoroscopic examination, the lesion moved with the diaphragm. Another chest radiograph five days later showed that this effusion had disappeared, but the shadow (an oval, well-defined mass) was considerably larger. Bronchoscopy, respiratory function and bronchogram were normal. At operation (V. P.), a slightly pear-shaped tumour, measuring 6 x 7 x 6 cm and arising from the diaphragm, was resected with part of the left hemidiaphragm and adherent flattened edge of lung and pericardium. There was no invasion of adjacent organs. The tumour had a thin capsule. Histology revealed a cellular differentiated fibrosarcoma without invasion of adjacent tissues. The general features suggested a low grade of malignancy. The patient remained well for nine years. However, progressive increase in shadowing at the left base was noticed on successive chest radiographs taken over the last two years before admission to the hospital. Symptoms began to recur over the last few months, with pain in the lower chest, a sense of fullness and heartburn on eating and occasional vomiting. The pain was a continuous dull ache. On examination, the patient appeared to be healthy. A very large mass was easily palpable in her left hypochondrium, extending upwards to invade the anteriolateral chest wall. The tumour was not mobile or tender. There was no clubbing or hypertrophic osteoarthropathy. I
Costas G. Sbokas, F. D. Salama, V. Powell and I. K. R. MeMilan A chest radiograph demonstrated a dense opacity at the left lower chest, extending mainly into the abdomen. Intravenous pyelogram was normal, although the kidney was a little displaced medially. Bronchoscopy, oesophagoscopy and gastroscopy were normal. An aortogram was performed and showed no involvement of any of the major vessels. A barium meal showed the stomach to be pushed over to the right but not invaded (Fig. 1).
Fig. 1. A barium
meal
revealed
displacement hypochondria1
of the mass
stomach
to the
right
by
a left
Exploration was carried out, through a left thoracoabdominal approach (I.K.M., January 1975). An enormous ovoid lobulated tumour, measuring 22 x 22 x 13 cm was found in the upper abdomen, extending up into the left chest. The surrounding structures were only adherent to it, but not invaded. The stomach, the omentum, the spleen,’ the left lobe of the liver and the left lower lobe of the lung were peeled off it without difficulty. It arose from a small area in the peripheral left diaphragm and appeared to be thinly encapsulated over most of its surface. No glands were involved and there was no evidence of metastasis or any other tumour in the abdomen or the left chest. It weighed 4 kg (Fig. 2). Histology revealed a fibrosarcoma with a liposarcomatous element. It was composed of interweaving spindle cells of pleomorphic appearance. A moderate degree of acid mucopolysaccharide was present in the stroma and typical of connective tissue mucin. Mitoses were fairly frequent and avascular necrosis was occurring (Fig. 3). The patient stood the operation well and made an uneventful recovery. She is fit and well at the time of writing.
51
Primary Fibvosavcoma of the Diaphragm
2. External
appearance muscular
of the tumour. A very large tissue of the diaphragm is seen
ovoid lobulated at the resected
Fig. 3. Histological
of the
appearance
tumour
tumour. margin
Portion
of
1:he
52
Costas G. Sbokas, F. D. Salama, V. Powell and I. K. R. McMillan DISCUSSION
Fibrosarcoma is the commonest of malignant diaphragmatic tumours, constituting 27-30% of cases. It may occur at any age, although it is more common in middle age. There is no sex prevalence. It is slightly commoner on the left. Pleuritic chest pain or pain referred to the epigastrium is the most common complaint. The onset, duration and severity vary considerably. The pain is usually aggravated by deep breathing or physical activity and may radiate to the shoulder or the neck, suggesting phrenic nerve irritation. Gastrointestinal symptoms may be caused by left-sided tumours and this was the most disturbing symptom in our case. Hypertrophic pulmonary osteoarthropathy is not very common, occuring in 10% of the cases (Nicholson & Whitehead 1956; Wiener & Chou 1965; Olafsson et al. 1971). Such tumours are usually detected in chest radiographs but their radiological appearances are not characteristic. The usual picture is that of an intrathoracic lesion adjacent to the diaphragm or a subdiaphragmatic condition elevating or deforming the diaphragm. Pneumoperitoneum can be very helpful in the differential diagnosis, particularly from big diaphragmatic hernias (Crimm & Liechle 1952; Sarot et al. 1969). Fibrosarcoma of the diaphragm has been reported to be a relatively small tumour, growing rapidly and metastatizing early (Crimm & Liechle 1952; Wiener & Chou 1965). The longest survival recorded is five years (Drewes & Willmann 1955). Our case survived for 10 years after the first operation and the recurrence, although enormous in size, was not accompanied by any distant metastases. The presence of a liposarcomatous element is an interesting histological feature in our case; this variety has not been recorded before (Kalen 1970). Radiotherapy is not usually beneficial. As it is impossible to decide before operation about the true nature of diaphragmatic tumours, and as their size and even recurrence is no indication of inoperability or the degree of malignancy, surgical intervention is considered to be the treatment of choice. ACKNOWLEDGEMENT
We would like to thank Dr James Bowe who referred the patient.
Requests for reprints should be addressed to I. K. R. McMillan, Hospital, Oakley Road, Southampton SO9 4 WQ.
Southampton
Western
REFERENCES P. 0. & LIECHLE, F. L. (1952) Fibrosarcoma of the diaphragm. J. thorac. Surg. 23, 360. J. & WILLMANN, K. H. (1955) Die Primiiren Tumoren des Zwerchfalls. Thoraxchivurgie 3, 75. KALEN, N. A. (1970) Lipoma of the diaphragm. Scund.3’. resp. LG. 51,28. NICHOLSON, F. & WHITEHEAD, R. (1956) Tumours of the diaphragm. BY. J. Swg. 43, 633. OLAFSSON, G., RAUSLING, A. & OLEN, 0. (1971) Primary tumours of the diaphragm. Chest 59, 568. SAROT, I. A., SCHWIMMER, D. & SCHLECHTER, D. G. (1969) Primary neurilemmoma of the diaphragm. N. Y. St.J. Med. 69, 837. WIENER, M. F. & CHOU, W. H. (1965) Primary tumours of the diaphragm. Archs Surg., Chicago 90, 143. CRIMM, DREWES,