Spontaneous regression in alveolar soft part sarcoma: case report and literature review
© BaniHani and Al Manasra; licensee BioMed Central Ltd. 2009
Received: 11 March 2009
Accepted: 10 June 2009
Published: 10 June 2009
Sarcomas are a type of malignant tumors that arise from connective tissue. They are most of the time found in extremities
We are presenting a case of adult male patient, who was found to have huge abdominal mass and multiple gastric and duodenal polyps. Pathological diagnosis for all lesions was Alveolar soft part sarcoma. Although he complained from metastasis to both lungs and right atrium, all these deposits regressed spontaneously. Patient stated that he used some herbs (Teucrium polium, Cat Thyme) prescribed for him. No chemotherapy or radiotherapy was given. The duration of regression was about 5 months before other lesions appeared. Later on, he died secondary to brain metastasis.
ASPS is a rare type of sarcomas that affect primarily the lower limbs. This tumor does rarely metastasize to the gastrointestinal tract.
Sarcomas are a type of malignant tumors that arise from connective tissue. They are most of the time found in extremities. The term -Soft Part- is used to distinguish these sarcomas from bone sarcomas. Although soft tissue sarcomas are linked to the tissue of origin; alveolar description is based on the histological pattern of the tumor rather than the origin.
A 38 year old male married patient, medically free, referred to our center from a another hospital complaining from lower abdominal pain of few months duration, intermittent, vague, gripping in nature, associated with abdominal distention.
He didn't undergo any type of surgery previously. Past history of the patient was negative for any fever, sweating, vomiting, constipation, or weight changes. He also didn't suffer from obstructive urinary symptoms. Family history was negative too.
On examination, he actually was found to have large left, hypochondrial mass. It was painless & hard in consistency.
ASPS is a rare type of sarcomas, accounting for less than 1% of soft tissue sarcomas . It was firstly described by Christopherson et al in 1952 . The usual age for presentation is 15–35 years. The most common site for the tumor is lower extremities , representing about 60% of ASPS cases. Other sites included are head and neck, more commonly in children.
Gastrointestinal tract is also rarely involved by these malignancies. Only 3 cases were reported. In 2001, Michael S.et al reported a case of ASPS metastatic to small bowel mucosa causing polyposis and intussuseption in a 42 year old male with long history of ASPS metastasis to lungs and brain.  Later on; in 2003, Zilber S. et al reported another 43 year old woman who had a leg primary tumor more than 15 years ago and multiple lung and brain metastases. She also was found to have caecal metastases, revealed by an anemia, she was treated by laparoscopic right colectomy . Primary Gastrointestinal ASPS is extremely rare. Only one case was reported in 2000 by Yaziji H et al. He described a Primary ASPS of the stomach in a 54 year old Italian woman without evidence of primary neoplasm elsewhere ten years following the initial diagnosis .
In our case, screening for primary lesions in extremities was negative. Pathological wise, depending on the cellularity of the tumor, the primary source is more likely to be from mesenchymal cells of the mesentery than from gastric or duodenal polyps,. This means that it can be considered the first case of this origin type to be reported in the literature.
Spontaneous regression is a term that is used interchangeably with spontaneous remission. Tilden Everson and Warren Cole have defined spontaneous regression of cancer as the partial or complete disappearance of a malignant tumor in the absence of all treatment or in the presence of therapy which is considered inadequate to exert a significant influence on neoplastic disease. They stated that it is not implied that spontaneous regression need progress to complete disappearance of tumor nor that spontaneous regression is synonymous with cure, all cases in which a tumor underwent apparent spontaneous regression in one area but flourished unchecked in other areas or reappeared at a later date are considered as valid examples of this phenomenon in their opinion. Until now, this definition of spontaneous regression, with few additions, has remained the one in most common usage today . In fact, what happened in our patient case does fit to the criteria mentioned in their definition of spontaneous regression.
The highest incidence of this phenomenon was in tumors of genitourinary organs. According to Caryle Hirshberg , between 1918 and 1993, a total of 64 soft tissue sarcomas cases were reported to undergo spontaneous regression, however, spontaneous regression in the more specific alveolar pattern sarcomas has not been identified during that period.
To the best of our knowledge, only a single case of ASPS with spontaneous regression (of pulmonary metastases) has been published. In 2003, A 14 year old male patient was described to have ASPS in femoral region. A combined chemotherapy (radiotherapy, hyperthermia and chemotherapy) was performed before operation. Tumor wide resection was carried out, however, a metastatic lesion revealed in the lung continued to increase in number and size during the progress after operation, however, it decreased in size and number after several years .
In 1988, Pang and colleagues have report a case of female patient with ASPS who survived for over 9 years after detection of pulmonary metastases regressed when she was treated with oral and topical Chinese herbs. She had excision of a right buttock mass in 1975. She was given cytotoxic chemotherapy, but there was no radiological response. Thereafter; she took herbal mixture from March 1979 to July 1982. Chest X-rays in 1983 showed complete disappearance of some shadows and shrinkage of others. 
In a study conducted by Kebudi R. et al between 1989 and 2002, the incidence of brain metastases in extracranial solid tumors was studied in pediatric age group. Only 16 out of 1100 children with extracranial solid tumors developed brain mets. One of the 16 was found to be alveolar soft part sarcoma. 
The aim of publishing our case, the second of its type in the literature, was to encourage the study of biological behavior of ASPS as well as common molecular characteristics that could be present in other tumors that tend to regress alone.
ASPS is a rare type of sarcomas that affect primarily the lower limbs. This tumor does rarely metastasize to the gastrointestinal tract. We can only speculate on the unusual spontaneous regression reported in this case. We do not have any solid information of a cytotoxic effect of the herb used, so we speculate that the regression was spontaneous probably mediated by the immunologic system of the host. However, this effect was short termed as the patient has died from further progression and metastasis of the tumor.
Written informed consent was obtained from the next of kin for reporting of this case, the copy of consent is available with editor in chief.
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