|Year : 2015 | Volume
| Issue : 5 | Page : 663-666
Dermatofibrosarcoma protuberans: A rare presentation with lung and abdominal metastasis
Rupam Kumar Ta, Sourindra Nath Banerjee
Department of Chest Medicine, Burdwan Medical College and Hospital, Burdwan, West Bengal, India
|Date of Web Publication||10-Sep-2015|
Rupam Kumar Ta
120, Vivekananada Road, Chotonilpur, P.O. - Sripally, Burdwan - 713 103, West Bengal
Source of Support: Government Hospital and Self-funded., Conflict of Interest: None declared.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally invasive, slow-growing tumor of the skin and the subcutaneous tissue. The skin lesion appears initially as a single, raised, red to bluish, firm cutaneous nodule or plaque with surrounding discoloration. It occurs usually at middle age with a male preponderance with an annual incidence of nearly 0.8 cases/million. The tumor occurs predominantly on the trunk followed by the proximal extremities and in the head and neck area. Even after wide surgical excision which is the standard treatment, it often recurs and becomes progressive with reported recurrence rate in the literature between 10% and 80% of cases. Distant metastasis is rarely observed with a rate of 4-6% and occurs mostly after repeated local recurrence. The lung is the most common site of distal metastasis. Metastasis to abdominal as well peri-intestinal site with serosal implant was rarely reported. Metastasis was histologically confirmed. We are reporting this case as a rare presentation of DFSP with distal multiple metastases to the lung and abdomen occurring 8 years after initial diagnosis and so emphasize on long-term regular follow-up.
Keywords: Dermatofibrosarcoma protuberans, metastasis, recurrence, swelling
|How to cite this article:|
Ta RK, Banerjee SN. Dermatofibrosarcoma protuberans: A rare presentation with lung and abdominal metastasis. Med J DY Patil Univ 2015;8:663-6
| Introduction|| |
Dermatofibrosarcoma protuberans (DFSP) was first described in 1924 as a progressive and recurrent dermatofibroma. It is an uncommon locally invasive, slow-growing tumor of the subcutaneous tissue (soft tissue sarcoma) that often recurs even after wide surgical excision. DFSP typically occurs with a male preponderance and an annual incidence of nearly 0.8 cases/million was reported.
It usually occurs in adults aged 20-50 years, but it has been reported to occur rarely at extreme of ages. The progression to a high-grade fibrosarcomatous (FS) component was rare. The tumor occurs predominantly on the trunk followed by the proximal extremities and also the head and neck area. Distant metastasis is rare. Nearly <5% of patients of DFSP cases may show distant metastasis many years after occurrence of the initial lesion. The standards treatment is complete wide surgical excision with appropriate reconstruction. When resection is limited or incomplete or margins are positive, recurrence rate becomes high.,, The general immuno-staining pattern of DFSP shows CD34-positivity. We are reporting this case as a rare presentation of distal multiple metastasis to lungs and abdomen 8 years after initial diagnosis and also emphasize on long-term regular follow-up to detect metastasis.
| Case Report|| |
A 45-year-old male patient presented to our department with complains of shortness of breath (SOB) on exertion and weakness for last 4 weeks and abdominal pain for 2 weeks. A big firm swelling was present over his right upper arm for last 2 months along with a wide postoperative scar with irregular nodular surface over his upper back since 2006. He came with an abnormal chest X-ray showing multiple nodular opacity. His recent SOB was gradually progressive, Grade II modified Medical Research Council scale of breathlessness, mostly with exertion without any other symptom(s) of chest diseases and any kind of addiction. He became weak and lethargic over last 1-month. He had no prior cardiac disease. A dull aching constant, noncolicky, nonradiating abdominal pain was present for last 2 weeks. Intensity of pain was moderate 4/10 visual analogue scale. It was nocturnal and was not related to any food intake. Relief of pain would occur with opoids in addition to nonsteroidal antiinflammatory drugs but not with any antacid. Nausea or vomiting and abdominal swelling was absent. A big firm painless swelling appeared on his left thoraco-lumber area for 1-month. On enquiry, the patient told that he first noticed a globular firm swelling of skin and soft tissue on his upper back in 2006. Initially, the swelling was 1 cm × 2 cm in size, discrete, painless and mobile with demarkable margin. It enlarged gradually with its irregular and nodular overlying surface without any overlying local discharge or hemorrhage. He initially consulted a general surgeon who advised for fine needle aspiration cytology (FNAC) from the lesion. FNAC of the swelling was suggestive of malignancy of soft tissue origin. He was underwent wide surgical excision with reconstruction of overlying skin and soft tissue first time in 2006. A histopathological diagnosis of DFSP was made from the first postoperative tissue biopsy specimen. Immunohistochemistry (IHC) was done at that time showing positivity for CD34 staining. The lesion in upper back recurred after 12 months at same site after initial resection. Simultaneously, he also developed a soft tissue swelling near left thoraco-lumbar area, which was proved as metastatic local deposit by biopsy. He underwent a second wide excision with skin grafting. Thereafter, several other relapses at same site of the tumor with repeated local recurrences occurred in the year 2008, 2011, 2012. Surgical re-resections were done and local adjuvant radiotherapy (RT) was given in 2008 but still recurred. Histopathological biopsy report of all the repeatedly excised surgical specimen came out as DFSP with tumor free margin. Recently, another new gradually increasing globular hard painful swelling of 4 cm × 4.5 cm size was noted over his right upper arm for last 2 months [Figure 1]. The overlying skin gradually became tensed, glossy and skin surface was irregular with violacious color. He also noticed gradually developing multifocal painless firm nodular soft tissue swelling of varying size on the upper portion of the back of the neck, scalp and below right axilla near the posterior axillary line. Personal and past medical and family history were not significant. His general survey examination was unremarkable except for pallor. His vitals were stable. Examination of the respiratory system was unremarkable. Abdominal examination revealed firm nontender hepatomegaly of one inch along right midclavicular line. Splenomegaly or ascites was not detected per abdomen. Other system examination was within normal limit. Routine investigation of blood, urine and other relevant serum biochemistry were normal except low-hemoglobin level which was 8.6 g%. Red blood cells were hypochromic and microcytic suggestive of iron deficiency anemia. Serum iron was low. His stool was negative for occult blood. Recent chest-X-ray showed multiple nodular lesions of variable size in right lower zone [Figure 2]a. Contrast-enhanced computed tomography (CECT) scan of thorax also revealed multiple nodular deposits in the right lung without any mediastinal involvement and pleural effusion [Figure 2]b. CT guided FNAC from of lung nodule concluded metastatic deposits from DFSP lesion. Ultrasonography of the abdomen revealed a nearly 7 cm × 8 cm heterogeneous mass adjacent to the left adrenal gland and hepatomegaly with homogenous echo texture but no ascites. CECT scan of the abdomen also showed 8 cm × 9 cm heterogeneous soft tissue mass seen in left adrenal region with few areas of degeneration. Left paraspinal (back) muscle was bulky, heterogeneous with few areas of hypodensity suggestive of degeneration [Figure 3]. Few exophytic mass was seen attached to the small intestine with serosal implant without features of obstruction [Figure 3]. This mass explained the occurrence of persistent pain abdomen. FNAC again was done from abdominal mass and was suggestive of metastatic deposit from the dental follicle stem cell. CECT scan of the brain was normal. His general condition was not bad with a body mass index of 18.4 kg/m2 even with so much recurrent tumor burden for last 8 years. Biopsy report from swelling of his right upper arm was done, and it revealed malignant FS transformation of DFSP. But this time IHC report could not be done for financial constrain. Imatinib mesylate (400 mg) once daily was started as per advice of RT department and the patient was under follow-up now. Written informed consent was taken for publication of this case from the patient including images.
|Figure 2: (a) Chest X-ray and (b) contrast enhanced computed tomography thorax showing lung metastasis and (c) histology showing dermatofibrosarcoma protuberans with typical storiform pattern of spindle cells (H and E, original magnification ×100)|
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|Figure 3: Contrast enhanced computed tomography abdomen showing heterogenous mass near left adrenal gland and bulky left paraspinal swelling with peri-intestinal metastasis with peritoneal implant|
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| Discussion|| |
Dermatofibrosarcoma protuberans is the most frequent skin sarcoma and it accounts for 2-6% of all soft tissue sarcomas., It appears initially as a skin lesion described as a single, raised, red to bluish, firm cutaneous nodule or plaque with surrounding discoloration. The lesion is usually painless and becomes indurated but is extremely infiltrative and has a locally destructive growth potential so that it can invade the underlying deeper structures such as fascia, muscles or bones.,,, The tumor is often covered with a brown-yellow, red-tinged, sclerodermiform or teleangiectatic and atrophic skin as seen in our case. A history of trauma, burn or surgical scar may be present. Our case had no such history. Histological biopsy of DFSP revealed monomorphous proliferation of cytological spindle-shaped cells with a visible storiform or whorled (rushmat-like) architecture,, [Figure 2]c. Other characteristic features low mitotic activity and deep, honeycomb infiltration into subcutaneous adipose tissue. The expression of CD34 was positive on IHC and thus differentiated the lesion as DFSP from the other causes like benign fibrous histiocytoma, dermatofibroma and other soft tissue tumors.,,, The sensitivity of CD34 staining in DFSP ranges from 85% to 100%. Mohs micrographic surgery is the treatment of choice for DFSP. The alternative treatment of DFSP, regardless of its location, is wide and deep excision with adequate tumor-free margins. However, our patient had the latter. The type of the surgical procedure has an impact on the risk of recurrence. The success of the initial surgical excision has a major effect on its outcome also. In fact, if surgical procedure fails, the tumor would recur and would lead to an uncontrollable local growth as seen in our patient. The proper excision with clean margin is directly related to the risk of recurrence of the disease despite adjuvant therapy.,,, The involvement of the deep fascia and muscles require excision of these structures.,
Radiotherapy and chemotherapy were not used initially because the incision margin of the tumor was negative on histopathology. In 2008 adjuvant local RT was given but local recurrence still occurred. Hence, our case did not match with earlier studies, which suggested that additional postoperative RT reduces the risk of local recurrence in patients with questionable or positive surgical margins., Expression of tentacle-like projections from the tumor underneath healthy skin usually causes difficulty to remove the tumor completely, and this might have occurred with our case and this was probably the reason of high local recurrence rate. Due to this, our patient might have experienced repeated excisions. Its recurrence rate in the literature was between 10% and 80%. The risk of recurrence was 41% when the excision margin was <2 cm and 24% when it was equal to or more than 2 cm. Some cases of DFSP could relapse as late as 5 years after surgery. The indolent nature of DFSP requires lifelong surveillance for recurrence. Chest x ray are indicated yearly during a follow-up period up to 10 years for the detection of metastatic disease.
Despite local invasiveness and recurrence, distant metastasis is rarely observed with a rate of 4-6%, predominantly to the lungs.,, The lungs are the most common site of distant metastasis via hematogenous dissemination. Usually, metastatic disease is preceded by multiple local recurrences, as seen in our case. Metastases usually occur within 6 years after initial lesion. The prognosis in cases of metastasis is poor because death often ensues within 1-year after metastasis. Gloster et al. reported two separate episodes of pulmonary metastasis 9 and 10 years respectively, after initial DFSP.
So, as seen in the present case, locally recurrent DFSP can be devastating. In our department, three units of packed red blood cells were transfused and there after his exertional SOB was relieved much. His subjective wellbeing also improved. Symptomatic treatment was given to palliate discomfort. He is now under oral chemotherapy with imatinib mesylate 400 mg daily and kept under follow-up also. We reported this case of recurrent and infiltrative DSFP that failed conventional therapeutic options. Repeated surgical resection in relapsed lesions would increase the risk of metastatic potential. Clinicians should be aware of the re-occurrences of DFSP at postsurgical local area and it's potential for distal metastasis to lung and abdomen as long as 8 years after initial diagnosis despite its rare possibility. Abdominal metastasis was very uncommon. Multisite visceral metastasis (lung, abdomen) particularly peri-intestinal metastasis with serosal implant is very rare and also not reported, so far our knowledge goes. However, treatment was challenging with multidisciplinary team support and response to treatment also was not consistent in all cases.
| Acknowledgment|| |
General support from our departmental staffs for technical and material support and no Conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]