A RARE CASE REPORT OF ALVEOLAR RHABDOMYOSARCOMA IN VAGINA OF YOUNG FEMALES

Alveolar rhabdomyosarcoma in a rare aggressive tumour which manifests in children and young adults. The site of alveolar rhabdomyosarcoma in vagina is also extremely rare. We presented a case report of 38 yrs.lady who was having complain of intermenstrual bleeding & discharge per vaginum from last 6 months. A large fleshy infiltrative bluish friable mass (3*3cm) present at the junction of middle rd rd and upper 1/3 and upper 1/3 of posterior vaginal wall . Another similar mass (2* 2cm) was also present in the lateral vaginal wall. Wide excision of both growth was done & postoperatively chemotherapy was given.

Upper lip squamous cell carcinoma arising from discoid lupus erythematosus treated with standard wide excision

@#Introduction: Squamous cell carcinoma (SCC) is a rare complication of long-standing discoid lupus erythematosus (DLE). The reported risk factors associated with the malignant transformation include chronic inflammation, scarring, immunosuppression, and the dysfunctional healing already evident in discoid lesions. If underdiagnosed and left untreated, this may lead to higher rates of recurrences, metastases and death. Objective: The main objective of this article is to report the diagnosis and management of a DLE-associated SCC on the upper lip of an elderly Filipino female. A short discussion on the pathogenesis and treatment of this disease entity is also presented.ugs/topical medications, family history and accompanying systemic symptoms were reviewed and analyzed. Case: A 78-year-old female, pawid maker, developed a rapidly growing verrucous tumor measuring 4.0 x 2.0 x 2.0 cm from a depigmented atrophic plaque located on the upper lip. Initial histopathologic findings were consistent with SCC and DLE. Considering the tumor size and the challenging anatomical location, the surgical oncology and plastic surgery team carried out a wide excision with 5 mm margin of the entire upper lip mass and surrounding plaque, followed by lip reconstruction. Biopsy findings of the excised mass were consistent with the initial findings. Three months post-operation, there was significant wound healing with no recurrences. Conclusion: Although the incidence is rare, SCC occurring in DLE is considered to be high-risk and should warrant early recognition and prompt treatment. In this paper, the authors also highlight the use of a standard wide excision in lieu of Mohs Microscopic Surgery as an alternative option where resources are limited.

Aneurysmal fibrous histiocytoma

Aneurysmal fibrous histiocytoma is rare clinicopathological variant of Cutaneous Fibrous Histiocytoma. The clinical manifestation of Aneurysmal Fibrous Histiocytoma is often confusing to distinguish from other skin lesions. Most of the cases showed rapid increase in size or a history of recurrence, however histologically all are almost similar. Rarity of aneurysmal fibrous histiocytoma and high numbers of recurrence rate poses a big diagnostic challenge. Late treatment will result in a decrease until loss of function of the affected region.  In this article, author reported the case of aneurysmal fibrous histiocytoma of the hand in 7 years old girl with restricted at 2nd-3rd metacarpophalangeal joints. The patient had undergone a series of investigations until finally a wide excision was carried out. Excision tissue was performed CD 68 and CD 34  immunocytochemical smear to establish the diagnosis. It was not simple to make diagnosis aneurysmal fibrous histiocytoma. While it is benign, the lesion can appear malignant, and one should consider an excisional biopsy to rule out malignant conditions. The diagnosis had to be confirmed by histopathological and performed immunocytochemical smear. It was often necessary to take aggressive actions with wide excision and reconstruction.

Pitfall management of dermatofibrosarcoma protuberans on right leg

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally invasive soft tissue sarcoma. The local recurrence rate is high, in some studies upwards of 60%, likely reflecting a failure to remove occult extensions of tumor. Surgical excision has been the treatment of choice for the resection of DFSP. Any pitfall on management therapy of DFSP not only increase the recurrence rate but also add new problems to patients with DFSP. 58-year-old male patient, came with a local recurrent of DFSP on his right leg. Then performed excision with margin one centimetre, and closing defect using pedicle sural flap, durante surgery turned pedicle on the flap is too short and because of poor soft tissue handling pedicle was injured. It consulted into the vascular division for evaluated and treatment pedicle. Postoperative evaluation, the flap experiences bluish due to vascularization compromised. It was decided to expose the flap and the defect was covered with skin graft. 2 months postoperative evaluation found local recurrent on skin graft area. Excision margins between 2 to 5 cm can reduce the recurrence rate. Proper planning in designing flaps to cover defects and the ability of good soft tissue handling is required to avoid new problems on management DFSP.

A Case of Basal Cell Carcinoma on the Left Cheek in a 62-Year-Old Woman, Operated by Wide Excision with V-Y Subcutaneous Tissue Pedicle Island Advancement Flap Under Bilevel Surgical Tumescent Anesthesia

Basal cell carcinoma (BCC) is a nonmelanocytic skin cancer that arises from basal cells, slow-growingtumors that almost never metastasize. Untreated BCCs have the potential to continue to grow anddestroy surrounding skin and nearby structures leading to physical deformity. The key to treatmentis to ensure that there is no more cancer left in the skin. Reconstruction with V-Y skin flaps can beperformed in several areas of the face. We reported a 62-year-old female patient with pigmentednodular BCC tumor on left cheek who was successfully treated with wide excision with V-Y skin flapunder bilevel surgical tumescent anesthesia.

Poorly Differentiated Synovial Sarcoma over Tendo Achilles: A Rare Case.

Synovial sarcomas of Tendo Achilles are rare tumors. The name “synovial sarcoma” is a misnomer, as it does not arise from synovial membrane. These tumors occur usually in the upper and lower limbs, more often in lower limbs in the distal parts. Distal extremities are more commonly associated with this tumor. It is seen to affect mostly tendon sheath, bursa, and joint capsule; knee being the commonly affected large joint. The common age group is 15-40 years. In our case, the initial pathology report came as hemanioma to us, but the immunohistochemistry report revealed it to be poorly differentiated synovial sarcoma.

Congenital Dermatofibrosarcoma Protuberans Treated with Wide Excision / 대한피부과학회지

Dermatofibrosarcoma protuberans is a fibrohistiocytic tumor with intermediate malignancy that usually appears in adults. The congenital type is very rare, and only three cases have been reported so far in the Korean literature. A 2-month-old girl presented with a firm reddish-gray nodule on the middle of her back that had been present since birth. Histopathologically, the lesion was composed of fascicles of densely packed uniform spindle cells arranged in a storiform (mat-like) pattern. The cells had hyperchromatic nuclei and rare mitotic figures. On immunohisto-chemistry, the tumor cells were positive for CD34. Based on these findings, we made the diagnosis of congenital dermatofibrosarcoma protuberans. She underwent wide excision, and there was no evidence of recurrence during 24 months of follow-up. Our report is of interest because of the rarity of congenital dermatofibrosarcoma protuberans and its earliest detection reported in Korea.

Delayed Reconstruction for the Non-Amputative Treatment of Subungual Melanoma

BACKGROUND: In cases of early stage subungual melanoma (SUM), conservative treatment with non-amputative wide excision of the nail unit and subsequent skin graft is preferred over amputation to preserve the involved digit. OBJECTIVE: We report a series of patients with SUM treated with conservative surgery and suggest an effective supplementary treatment process. METHODS: We retrospectively reviewed 10 patients (2 males, 8 females) who were diagnosed with in situ or minimally invasive SUM on the first biopsy and underwent non-amputative wide excision of the nail unit. All patients underwent secondary intention healing during the histopathological re-evaluation of the entire excised lesion, and additional treatment was administered according to the final report. RESULTS: In two of 10 patients, amputation was performed because of the detection of deep invasion (Breslow thickness: 4.0, 2.3 mm) from the final pathologic results, which differed from the initial biopsy. In six patients who received delayed skin graft, the mean total time required for complete healing after secondary intention healing and the skin graft was 66.83+/-15.09 days. As a result of this delayed skin graft, the final scarring was similar to the original shape of the nail unit, scored between 5 and 10 on a visual analogue scale. Most patients were satisfied with this conservative surgery except one patient, who had volar portion involvement and received an interpolated flap instead of a skin graft. CONCLUSION: Our treatment process can reduce the risk of incomplete resection and improve cosmetic outcomes in patients with SUM.

An Unusual Intranasal Mass: Pleomorphic Adenoma of the Nasal Septum.

Pleomorphic adenomas are the most common salivary glands tumors that occur predominantly in major salivary glands with highest incidence in the parotid gland. Rarely, they may also be seen arising from the minor salivary glands present in the upper aerodigestive tract. Though they can occur at any age, the peak incidence is in 3rd-6th decade and are seen more commonly in females. A wide variety of tumors arise in the nasal cavity. A majority of them develop from the nasal mucosa; however, some also arise from the olfactory, neuroendocrine or minor salivary gland tissue. Pleomorphic adenoma is a tumor arising from the salivary glandular tissue with wide cytomorphologic diversity. Recognition of an epithelial, myoepithelial and a stromal component on histopathology is essential for diagnosis. Nasal pleomorphic adenomas are extremely rare, more so at a younger ages. Accurate clinical diagnosis is difficult owing to overlapping presenting features with other nasal tumors. Herewith we discuss clinical presentation, histopathologic peculiarities, diagnosis and management of nasal pleomorphic adenoma.

Study on the Comparison between Wide Excision and Mohs Micrographic Surgery for the Management of Dermatofibrosarcoma Protuberans: A Single Institution Experience / 대한피부과학회지

BACKGROUND: Dermatofibrosarcoma protuberans is a mesenchymal tumor of the skin of intermediate-grade which is a rare condition. The slow growing and aggressive invasion on local tissues are characteristic features of dermatofibrosarcoma protuberans. The treatment for dermatofibrosarcoma protuberans is mainly a surgical excision such as a wide excision and Mohs micrographic surgery. OBJECTIVE: The aim of this study was to compare the result of wide excision and Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans at a single institution in Korea. METHODS: A retrospective review was done for 24 patients diagnosed with dermatofibrosarcoma protuberans and treated surgically from 1999 to 2010 at Chonbuk National University Hospital. Patient demographics, tumor features, surgical features, and recurrence during the follow-up period were evaluated. RESULTS: 13 patients were treated with wide excision, and 11 with Mohs micrographic surgery. There was no metastasis for all the cases. Mean operation time for the wide excision group was 83 minutes whereas 182 minutes for the Mohs micrographic surgery group, and it was a statistically significant difference. However, no significant difference was observed in post-operative defect size, advanced surgical repair and local recurrence in our study. CONCLUSION: We suggest that wide excision and Mohs micrographic surgery are both successful modalities for the surgical treatment of dermatofibrosarcoma protuberans. Hence, individualized patient and tumor characteristics should be concerned when determining the surgical options for dermatofibrosarcoma protuberans.

Non-amputative Surgical Management of Subungal Melanoma in Situ / 대한피부과학회지

Subungual melanoma is a variant of acral lentiginous melanoma and is thought to carry a poor prognosis due to difficulties in early diagnosis. Classical management of subungual melanoma is based on the radical surgery of distal phalanx amputation. Instead, conservative treatment with non-amputative wide excision of the nail unit followed by reconstruction has been insufficiently reported, especially in Korea. A 71-year-old woman presented with dark brownish longitudinal bands on her right thumbnail. Punch biopsies from the nail matrix and nail bed showed proliferation of atypical melanocytes without dermal involvement. We describe a case of subungual melanoma in situ of the right thumb, and a non-amputative surgical management which aims to provide adequate clearance of the lesion with minimal morbidity and satisfactory preservation of function for the patient.

Effectiveness of Wide Excision of Subconjucntival Fibrovascular Tissue with Conjunctivo-Limbal Autograft in Pterygium Surgery

PURPOSE: To evaluate the efficacy of wide excision of subconjucntival fibrovascular tissue after conjunctivo-limbal autograft of primary and recurrent pterygium. METHODS: Sixty-five eyes of 59 patients underwent pterygium excision with conjunctivo-limbal autograft. Thirty-six eyes of 33 patients had wide excision of pterygium, 29 eyes of 26 patients did not have wide excision of pterygium. All patients underwent follow-up for more than one year. Complications, recurrence rate, and operation time were evaluated. RESULTS: With a minimum of one year of follow-up, there was no recurrence in either group. Six cases (17%) of subconjunctival fibrosis at the donor site, one case (3%) of subconjunctival neovascularization at the donor site, and one case (3%) of wound gapping at the recipient site were noted in the wide excision group. In the non-wide excision group, there were two cases (7%) of subconjunctival fibrosis at the donor site, one case (3%) of granuloma at the donor site, and one case (3%) of retention cyst at the recipient site. Mean operating time in the non-wide excision group (45.2 +/- 6.9 minutes) was significantly shorter than that in the wide excision group (62.4 +/- 6.2 minutes). CONCLUSIONS: Non-wide excision with conjunctivo-limbal autograft in pterygium surgery can be considered an effective treatment with shorter operating time than the wide excision method. Both of these methods show low recurrence rates and few complications for both primary and recurrent pterygia.

Axillary Hidradenitis Suppurativa Treated with a Split Thickness Skin Graft after Wide Excision / 대한피부과학회지

Hidradenitis suppurativa is a chronic, relapsing suppurative disease of the apocrine sweat glands that principally affects the axillary and anogenital regions. The etiology of this disorder remains unknown. As conservative treatment usually does not prevent recurrence, surgical treatment is the method of choice, particularly for advanced case. We report a 25-year-old male patient who presented with a 7-year history of abscesses with foul-smelling discharge from his axilla. After failed medical therapy, he was referred for surgery. We performed a wide excision of the axilla with a split thickness skin graft after 1 week. The patient has been doing well, and there has been no relapse for about ayear after the operation.

Treatment Outcomes of Anorectal Melanoma

PURPOSE: An anorectal melanoma (AM) is a very rare tumor. However, sufficient data supporting effective surgical options for the disease do not exist. This retrospective review aimed to analyze treatment outcomes for an AM. METHODS: From June 1999 to December 2008, we retrospectively reviewed a prospectively collected consecutive series of 19 patients who had undergone a surgical resection for an AM at a single institute. Surgical method and clinicopathological factors were analyzed. RESULTS: The median age was 61.4 years (range, 46 to79 years). Main symptoms were an anal mass, hematochezia, perianal pain, tenesmus, fecal incontinence, and bowel habit change. The average duration of symptoms before diagnosis was 7.8 months (range, 1 to 36 months). S-100 and HMB-45 were positive in all patients, even in non-melanin pigmentation. There were 12 abdominoperineal resections (APRs) and 7 wide local excisions (WEs). The APR showed longer overall survival when compared with the WE (64.1 months vs. 10.9 months, P < 0.001). No patients who underwent a WE survived more than 13 months. CONCLUSION: A high index of suspicion is necessary to establish the diagnosis for an AM in patients with anal symptoms, and S-100 and HMB-45 can be useful markers for an AM. Even with the small number of cases and the short follow-up, our data suggest that an APR for an AM may provide longer survival than a WE.

Malignant Fibrous Histiocytoma Originating in the Short-term after Excision

MFH is rare with just a few thousand cases diagnosed each year. We report a case of right flank malignant fibrous histiocytoma (MFH) in the short-term after tumor excision. A 47-year-old woman visited hospital after being diagnosed with MFH. Resection margin was positive, so we planned for wide excision. While awaiting surgery, 1.5 cm sized new mass occurred adjacent to the incision site. The patient underwent wide excision. Postoperative pathologic findings observed a 1.0 cm diameter mass with infiltrative borders in the subcutaneous fat. It was composed of spindle or epithelial cell showing severe polymorphism. Many mitotic cells were observed including atypia. In immunohistochemical study, tumor cells were negative for smooth muscle actin, desmin, myoglobin, S100, cytokeratin, and positive for CD68, and thus diagnosed as pleomorphic MFH. With the purpose of improving local tumor control, radiation therapy was performed after wide excision.

A Case of Pilonidal Sinus Mimicking Inflammatory Epidermal Cyst / 대한피부과학회지

Pilonidal sinus is one of a follicular occlusion tetrad, and is a chronic inflammatory disease that is associated with hair; a midline hairy patch or pit in the sacral region with a sinus orifice in the bottom, or a cyst beneath it, in which hair is growing. The exact pathogenesis of pilonidal sinus remains unknown, but hair insertion may be the essential cause of the disease. It is usually treated by radical excision, marsupialization or incision and curettage. However, these surgical options often lead to a high recurrence rate, especially for hirsute patients. Wide excision and simple primary closure of the pilonidal sinus is an easy, simple method and the recurrence rate is low. We report here the case of an 18-year-old male patient with recurrent pilonidal sinus mimicking inflammatory epidermal cyst. The pilonidal sinus was treated by wide excision and primary closure.

Sebaceous Carcinoma Treated with Various Flaps in Head and Facial Regions

PURPOSE: Sebaceous carcinoma is a rare malignant tumor that occurs mostly in head and neck regions. Early diagnosis and treatment are necessary because it tends to be locally aggressive and goes through distal metastasis with fast progression. This study presents reliable surgical methods for sebaceous carcinoma in head and neck regions. METHODS: Three patients were included in this study. First, a 61-year-old woman visited the hospital with a yellow-colored, slowly growing mass on the left ala. A 54-year-old woman had a brown-colored mass on her right preauricle. Last case was a 62-year-old man who had a yellow-colored mass on his scalp. CT scan and punch biopsy were done. All cases were diagnosed as sebaceous carcinoma. The lesions were resected with 10 mm safety margin and various regional flaps were used for reconstruction. RESULTS: Histological examination revealed sebaceous differentiation and local invasions. Postoperatively, all patients did not suffer from complication and no recurrence was found. CONCLUSION: We recommend early wide excision with an enough safety margin, and a regional flap as a treatment of sebaceous carcinoma.

Treatment of Heterotopic Calcification with Ulceration in Burn Scar

PURPOSE: Heterotopic calcification is a pathological event in which deposits of calcium salts build up, usually in the joint area or soft tissues. It can occur under many conditions and in some rare cases may develop in burn scars. In particular, ulcerations in burn scars accompanied by heterotopic calcification are difficult to treat through conservative treatment. This study reports methods for accurate diagnosis and adequate treatment of ulceration in burn scars accompanied by heterotopic calcification. METHODS: Fourteen patients who visited our hospital from March 2008 to January 2010 were subjected to this study. Their sex, age, modes of burn, degree, TBSA(%), ulcerated area, the time of occurrence of the ulcerations in the burn scars were investigated. In addition, radiological examination and biopsy was performed to diagnose heterotopic calcification. RESULTS: Among the 14 cases, 6 were male and 8 were female. The average age of the patients was 48.2(27-69 yrs). As for the mode of burn, 11 were flame burns and 3 were scalding burns. The average time of occurrence of the ulcerations in the burn scars was 4.5 months. The ulcerated areas were situated in the legs in 12 cases, arms in 1 case, and torso in 1 case. The diagnosis was confirmed through X-ray and biopsy, and skin graft was performed after wide excision. CONCLUSION: Diagnosis of the ulceration in burn scars accompanied by heterotopic calcification is possible through radiological and pathologic studies. Surgical treatment is the most reliable method of treatment, and we chose to perform skin graft after wide excision. Also, we learned that the complete removal of the calcified tissue and the inflammatory fibrotic tissues is crucial in preventing recurrence. Also, in contrast to Marjolin's ulcer, heterotopic calcification had a small size, little or no granulation tissue, and lacked fungating type ulceration. Therefore, favorable prognosis could be achieved through adequate treatment.

Formidable Angiosarcoma of the Scalp

PURPOSE: Angiosarcoma is a tumor of mesenchymal origin with an extremely high rate of metastasis and invasiveness. This tumor is notorious for its very poor prognosis, although surgical excision followed by radiation therapy is considered to be effective by many. The authors experienced three angiosarcoma patients with their tumors removed and wounds covered with split-thickness skin grafts and/or latissimus dorsi free flaps. METHODS: Three patients were admitted to our hospital showed plaques of different morphology. Based on their medical records, these patients were classified by sex, age, type of reconstruction, recurrence, and further treatment after surgical removal. RESULTS: All patients were male, with a mean age of 72 years (range, 66 to 77 years). Split-thickness skin grafts with latissimus dorsi free flaps were performed on two cases, and of these two cases, cervical lymph node biopsy was done in one case, and radical neck dissection was done in the other. In all cases, radiation therapy was done within two weeks of tumor removal. Distant metastasis occurred without local recurrence in two of the cases. Lung was the first organ affected by metastasis. In the remaining case, the tumor recurred locally 6 times, and additional excision was necessary. All patients died due to local recurrence and lung metastasis. CONCLUSION: Irregular margins and high recurrence and metastasis rates cause a poor prognosis in large angiosarcoma of the scalp. Radiotherapy and chemotherapy should be strongly considered in large angiosarcomas.

Secondary Chondrosarcoma Arising from Osteochondroma(tosis) / 대한골관절종양학회지

PURPOSE: To analyze clinical, radiological and pathological features as well as clinical outcome after surgical treatment of patients with secondary chondrosarcoma arising from osteochondroma(tosis). MATERIALS AND METHODS: We retrospectively reviewed clinical records, radiographs, pathologic slides of 14 patients. Nine patients were male and five were female. The mean age was 34 years. The mean follow-up period was 54 months. RESULTS: All patients had a history of previous mass since childhood or puberty. Preexisted osteochondroma was single in 3 patients and multiple in 10. Remaining 1 patient had multiple osteochondromatosis with enchondromatosis. MRI clearly provided thickness of cartilage cap, which was over 2 cm except in 2 cases. Chondrosarcoma was grade 1 in all except 1 case, which was grade 2. Wide excision was performed in 10 patients, marginal excision in 3 and amputation in 1. Twelve patients were doing very well without evidence of disease. Among 3 patients with marginal excision, 1 patient had local recurrence and 1 patient died of disease. CONCLUSION: Comprehensive understanding of clinical, radiological and pathological features of secondary chondrosarcoma is warranted for accurate diagnosis. The best result can be expected with early recognition of malignant change of osteohcondroma(tosis) and wide excision.