Dermatofibrosarcoma protuberance: a recrudescent dermal malignancy

Dermatofibrosarcoma protruberens is a rare soft tissue tumour of low malignant potential, commonly seen on the trunk and extremities. It is a slow growing sarcoma, with a tendency for recurrence. Rarely, it may be a high-grade tumour. It responds well to wide local excision. Radiotherapy is beneficial for margin positive and high-grade tumours. We present a case of a 22 years old male with a dermatofibrosarcoma protruberens presenting as a swelling over the left inguinal region since, 4 years, that was excised with a 2cm margin. Patient was followed up for six months without any recurrence.

Management of idiopathic granulomatous mastitis: a surgical experience of ten cases

Granulomatous mastitis is a chronic inflammatory condition of the breast that is rarely described in literature. It mimics breast abscess on clinical presentation giving dilemma to the surgeon. The etiology can be infectious, autoimmune or idiopathic. The various modalities of treatment for idiopathic granulomatous mastitis includes drainage of abscess, antibiotics and corticosteroid therapy. This is a case series of ten cases of diagnosed with granulomatous mastitis treated in the period of 2 years from 2018 and 2019 in Department of General Surgery, MGM Hospital Navi Mumbai. The data of all the patients were collected from records. They were following at regular intervals in the outpatient department to assess recurrence. The mean age of the patients was 41 years (range 26–47 years). Six patients gave history of breast-feeding in the last 5 years, one was lactating at the time of presentation. One of the patients was treated empirically for Tuberculous mastitis for the opposite breast. Others had no history suggestive of tuberculosis or other connective tissue diseases, such as rheumatoid arthritis or sarcoidosis USG breast was performed in all cases. Hypoechoic abscess was seen in 5 cases. The time taken for complete resolution of the disease was ranging from 2 weeks to 2 months. All patients had an uneventful recovery and no recurrences reported till now. GM is a rare benign breast disease that is difficult to distinguish from other inflammatory breast diseases or cancer. The diagnosis of GM must be based on a multidisciplinary approach. Surgical management followed by antibiotics was found to be sufficient to treat the condition in our patients. Corticosteroids need not be administered in all cases of this benign breast disease.

Melanoma of anal canal

Melanomas are primarily tumours of the skin, but rarely occur at other sites like retina and anal canal. Anorectal melanoma is an uncommon condition associated with a very poor prognosis. The patient usually presents with per rectal bleed or mass. These are often misdiagnosed on presentation. Diagnosis is confirmed by biopsy. Treatment is abdominoperineal resection or wide local excision if tumour free margins can be obtained. We present a case of a 60 years old female who presented to the outpatient department with per rectal mass and bleed since 4 to 5 months and was diagnosed with melanoma anal canal on biopsy. CT scan abdomen and pelvis was done. Patient underwent abdominoperineal resection as wide local excision with sphincter saving was not possible due to the location and extent of tumour as seen on CT scan. Patient had an uneventful recovery after the procedure.

A rare case of perineal endometriosis with anal sphincter involvement

Perineal endometriosis is a rare entity which can be explained by direct implantation of endometriotic cells over the fresh episiotomy wound and subsequent development of scar endometriosis. Perineal scar endometriosis incidence is reported to be 0.3% to 1%. 28 years old, P1L1, presented with pain and swelling near episiotomy site which is associated with menstruation. Examination during menstruation revealed swelling was tender, erythematous and slightly increased in size. Clinical diagnosis of scar endometriosis was made after clinical examination. Mass excised and sent for histopathological examination. Although diagnosis essentially remains clinical, preoperative evaluation with perineal ultrasound and MRI was performed. Wide local excision remains treatment of choice and follow up for recurrence is recommended. Histopathological examination is obligatory to exclude rare possibility of malignant changes.

Analysis of Surgical Resection and Prognostic Factors of Primary Chest Wall Chondrosarcomas

Introduction: Primary chest wall tumours are very rare.Chondrosarcoma represents 40% of all chest wall tumours.Wide local excision with tumour free margins has been thegold standard therapy. We evaluated this therapy in relationto various prognostic factors for anticipating the recurrenceof the tumour.Material and methods: 22 cases of Primary chest wallchondrosarcoma was operated upon from 2009 to 2019 withwide local excision with adequate margins. Male constituted77.27% and females 22.73% with the tumour size rangingfrom 5 to 30 cm in size(median 7cm).Results: 19 patients were subjected to lateral chest wallresection. 3 cases underwent partial sternectomies. Resectionwas extended to lungs in 7 cases, diaphragm in 2 cases,vertebral body in 2 cases and clavicle in 2 cases. Reconstructionwas done with polypropylene or titanium mesh and a muscleflap coverage. There was no perioperative mortality. Postoperative complications occurred in 13.6%(n=3). Therewas recurrence in 4 cases within 5 years. 5year disease freesurvival rate was 81.81%.Conclusions: Wide local excision with tumours free marginsstill remains the standard form of therapy. The reconstructiveprocedures have evolved largely to cover the huge defectsfollowing surgery. Post operative adjuvant therapy is guidedby various prognostic factors.

Primary Pleomorphic Adenoma of the External Auditory Canal-A Case Report and Literature Review

Tumours primarily arising from the external auditory canal are very uncommon and 5% of these originate from the glands present in the skin. Primary Pleomorphic adenomas arising from the External Auditory Canal are extremely rare and are difficult to diagnose preoperatively. They have clinically unremarkable presentation, and are treated by wide local excision with adequate normal margins. These tumours are mostly diagnosed postoperatively by histopathological examination and requires a long term follow up. In this case report we describe one such unusual case of primary Pleomorphic adenoma of the external auditory canal along with the literature review.

Surgical treatment of anorectal malignant melanoma from single tertiary center: A retrospective analysis of 91 cases / 中国实用外科杂志

OBJECTIVE:To investigate the surgical treatment of anorectal malignant melanoma(ARMM) and the prognostic factors that affect patients' overall survival(OS).METHODS:Theclinical and pathological data of 91 patients diagnosed as ARMM, who underwent surgery in the Gastrointestinal Cancer Center of Peking University Cancer Hospital from January 2003 to January 2018 were retrospectively analyzed. The effects of different clinicopathological factors on OS of the patients, and difference surgery, abdominal-perineal resection(APR) and wide local excision(WLE) on patients' survival were compared.RESULTS:Of the 91 patients with ARMM, 35(38.5%) were male, 56(61.5%) were female, and the ratio of male to female was 1:1.6. 12 patients(13.2%) underwent wide local excision and 79 patients underwent abdominal-perineal resection(86.8%); there were 19 cases(20.9%) diagnosed with distant metastasis and 72 cases(79.1%)without distant metastasis. The of 1-, 3-, and 5-year survival rates of ARMM patients in this cohort were 74%, 23% and9%; and the median survival of the patients was 18.7 months. The univariate analysis of the clinicopathological features of the patients demonstrated that the preoperative distant metastases, tumor diameters(>2 cm), tumor invasion depth(>2 mm) and lymph node metastasis were the prognostic factors of OS; multivariate analysis showed that preoperative distant metastasis and lymph node metastasis were independent factors affecting OS.The median survival of the patients admitted APR and WLE were both 21 months. There was no significant difference in survival between the WLE and APR group(P=0.94).CONCLUSION:Preoperative distant metastasis and lymph node metastasis are independent prognostic factors for overall survival of ARMM patients.Comparing with WLE, APR could not bring survival benefits.

Cystosarcoma phyllodes: Pathological enigma: A retrospective review of 162 cases.

PURPOSE: Phyllodes tumor (PT) is a rare fibroepithelial neoplasm comprising <1% of all breast tumors. Clinical spectrum ranges from benign (B), borderline (BL), and locally recurrent to malignant (M) and metastatic type. The aim of our study was to analyze the clinicopathological factors, compare treatment options, and evaluate outcome in patients with PT. METHODS: We retrospectively reviewed 162 women with PT. The surgical intervention varied from simple excision (lumpectomy)/wide local excision (WLE) in benign cases to simple/modified radical or radical mastectomy (SM/MRM/ RM) in malignant and recurrent tumors. RESULTS: Out of 162 patients, B, BL, and M were 95 (58.64%), 29 (18%), and 38 (23.45%), respectively. Mean age, duration of lump, and size were 38 ± 8 years, 28 ± 10 months, and 12 ± 5 cm, respectively. Recurrence rate with B, BL, and M was 15.78%, 41.37%, and 55.26%, respectively (P = 0.00001). As compared to WLE (22%), SM (23.8%), and MRM/RM (14.2%), recurrence was higher with lumpectomy (48.9%) (P = 0.004). Positive correlation was found between recurrence rate with the size of tumor (P = 0.008) and also number of recurrence with holoprosencephaly (P = 0.047). There was no association between the number of recurrences and size of tumor (P = 0.63). Malignant PT was seen in 38 (24%) and distant metastasis was seen in 7 (18%). Mean duration of follow‑up was 42 months. CONCLUSION: WLE with negative margins should be the initial surgery for PT. The role of adjuvant radiotherapy and chemotherapy is uncertain. PT is pathological enigma. Till date, no factors can accurately predict the recurrence and outcome. PT is known for unpredictable behavior and high recurrence rates, hence long‑term follow‑up is advised.

Primary Anorectal Malignant Melanoma Treated With Endoscopic Mucosal Resection

Anorectal melanoma is a rare neoplasm that accounts for less than 1-4% of anorectal malignant tumors. The main therapeutic modality for anorectal melanoma is surgical treatment, with abdominoperineal resection or wide local excision being the most common approaches. A 77-year-old male with a history of cerebral infarction and hypertension presented with anal bleeding. Here, we report a case of anorectal melanoma treated by endoscopic mucosal resection with adjuvant interferon therapy rather than surgical resection. The patient has been disease-free for 5 years after endoscopic treatment.

The Surgical Management of Dermato Fibro Sarcoma Protuberance.

Background: Dermatofibrosarcoma protuberance is relatively uncommon low to intermediate grade malignant neoplasm with characteristic cytogenetic features. It constitutes less than 0.1% of all malignant neoplasms. Although metastasized rarely, it is locally aggressive tumour with high recurrence rate. It is a one of the rare type of law grade sarcomas that occur s anywhere in the body, usually arise from trunks and extremities. It rarely arises from abdominal wall. Objectives: The main objective of the present study was to study the outcome of surgical treatment of dermatofibrosarcoma protuberance. Methods: This study included 4 patients all retrospective and prospective from September 2008 to December 2013. They were subjected to detailed history taking and examination with relevant investigations and were subjected to surgery. They were followed up for at least 36-month period to assess for any recurrence. Results and Interpretation: Out of 4 patients, all were males. Mean age of presentation was 32 years. Site distribution was 25% trunk, 50% extremities and 25% neck. All patients underwent wide local excision. At the end of follow-up period of up to 3 years after surgery Overall recurrence rate was nil. Because of the potential of local recurrence, therapy for DFSP should be directed toward adequate local excision of the primary lesion. Minimal resection should include a surrounding margin, comprising 3-cm margin of normal skin and removal of underlying deep fascia. Compromising on margins invites higher chances of local recurrence. Conclusion: DFSP behaves like a locally infiltrating neoplasm. Despite their locally aggressive behaviour, distant metastasis occurs with extreme rarity. Because of this, it is reasonable that therapy should be directed towards adequate local excision of the primary lesion. Compromising on margins always invites higher chances of local recurrence.

The clinical features and optimal treatment of anorectal malignant melanoma

Anorectal malignant melanoma (AMM) is a very rare and aggressive disease. The purpose of this article is to review the clinical features of AMM, to understand treatment options, and optimal therapy by reviewing pertinent literature. Traditionally an abdominoperineal resection (APR) sacrificing the anal sphincter has been performed for radical resection of cancer, but recently, wide excision of AMM is attempted since quality of life after surgery is an important issue. Some authors reported that there was no difference in five-year survival between the patient who underwent an APR and wide excision. The goal of both APR and wide excision was to improve survival with R0 resection. Adjuvant chemoradiation therapy can be performed to achieve an R0 resection. AMM shows very poor prognosis. At this time, research on AMM is insufficient to suggest a treatment guideline. Thus, treatment options, and a therapeutic method should be selected carefully.

A Case of Merkel Cell Carcinoma: Treated with Wide Excision and Adjuvant Radiotherapy

Merkel cell carcinoma is a rare aggressive primary cutaneous neuroendocrine skin cancer arising from the basal epidermis. Although the majority of patients with non-melanoma skin cancer are cured by local treatment, patients with Merkel cell carcinoma have a poor outcome characterized by locoregional and distant relapse. No standard treatment protocol for Merkel cell carcinoma exists. But, there is mounting evidence that combined treatment, incorporating adjuvant radiotherapy, improves the outcome (locoregional control and disease-free survival) compared with surgery alone in most patients. Herein we report a case of Merkel cell carcinoma treated with wide local excision and post-operative radiotherapy.

A Case of Malignant Melanoma of Vulva / 대한산부인과학회잡지

Malignant melanoma of the vulva is a rare neoplasm and accounts for approximately 10% of all vulvar malignancies. Five-year survival rates range from 8% to 54%, with occuring predominantly in postmenopausal white women. As compared with malignant melanoma of the skin, vulvar melanoma infiltrates early deep into the tissues with abundant lymphatic channels and blood vascularization, so its prognosis is mostly poor. Depth of invasion has been reported as the single most important indicator of prognosis. Historically the treatment of choice for vulvar melanoma has been en bloc radical vulvectomy with bilateral inguinofemoral lymphadenectomy. But recently there have been reports suggesting that acceptable survival may be achieved by less radical operation. The authors report a 84-year old woman with malignant melanoma of the vulva. She underwent wide local excision of the lesion. Now we are following her up with no clinical evidence of cancer recurrence and remnant.

A Case of Primary Malignant Melanoma of the Vagina: Trial of a Wide Local Excision of Vagina and Rectum / 대한산부인과학회잡지

Primary malignant melanoma of the vagina is an exceedingly rare entity, accounting for about 3% of all primary vagina1 malignant tumors and 0.3% of all melanoma. The overall 5-year survival in patients with primary vaginal melanoma is notoriously poor, estimated to be between 13% and 19%. A case of primary malignant melanoma of the vagina treated with the wide local excision of vagina and rectum was presented with a brief review of literature.