Mucinous adenocarcinoma of the perianal region is an oncologic rarity posing a diagnostic and therapeutic dilemma for treating oncologists due to very few reported cases without definite therapeutic guidelines. It accounts for 2% to 3% of all gastrointestinal malignancies and are historically known to arise from chronic anal fistulas and ischiorectal or perianal abscesses. We hereby report a sporadic and interesting case of perianal mucinous adenocarcinoma in a 56-year-old male initially treated with alternative medicines and local surgery for recurrent fistula in ano of 2 years duration. He presented with complaints of discharging growth in perianal region, painful defecation associated with occasional blood mixed stools of 6 months duration. Incisional biopsy from the ulcer revealed mucinous adenocarcinoma.Contrast enhanced computed tomography (CT) scan and whole body positron emission tomography (PET) scan showed a localized perianal growth which was further confirmed with colonoscopy. With no pre-set treatment protocol for this rare entity, he was managed with neo-adjuvant concurrent chemoradiation (CCRT) followed by abdominoperineal resection (APR) and adjuvant chemotherapy. Presently he is on 3 monthly follow-up since last 1 year post APR and adjuvant chemotherapy without any evidence of recurrence or distant metastasis. To the best of knowledge, our report may be one of the rarest cases of this disease entity where the duration of anal fistula was merely 2 years in contrast to the established criteria that the fistula precedes carcinoma by at least 10 years.
Adenocarcinoma lung with upfront metastases to skeletal muscle is rarely encountered in clinical practice since skeletal muscles are highly resistant to dissemination from solid organs. Moreover, these muscle metastatic lesions generally present with pain and palpable mass to get detected clinically. However, silent skeletal muscle metastases without any symptoms or signs getting detected by functional imaging with whole body 18F-fluorodeoxyglucose positron emission/computed tomography (18FDG-PET/CT) scan have been scarcely described in literature, while we present such an interesting case in a 45-year-old female. She was diagnosed as a case of biopsy-proven metastatic adenocarcinoma lung after evaluation by 18FDG-PET/CT. Despite treatment with palliative chemoradiotherapy, her disease progressed, and she finally succumbed to her illness. This case is discussed to highlight an unusual scenario we encountered, the clinical course of the disease with its management and overall poor prognosis.
Malignant peripheral nerve sheath tumor (MPNST) is a neurogenic tumor arising from peripheral nerves or nerve sheaths. MPNSTs are highly aggressive sarcomas mainly associated with neurofibromatosis type-1 (NF-1) with high rates of local recurrence and distant metastasis carrying a dismal prognosis. Lung is the most common metastatic site. Bone metastasis although documented in the literature is still very rare, while dissemination to brain without the involvement of lungs and that too in a non-NF-1 case is extremely unusual. A 48-year-old female was diagnosed with a case of non-NF-1 MPNST left thigh with bone metastases including sphenoid. Despite showing complete resolution of skeletal and primary lesions postpalliative chemoradiotherapy, she developed brain metastases and succumbed to her disease. This case is discussed to highlight an unusual scenario we encountered, the clinical course of the disease with its management, and overall poor prognosis. To the best of our knowledge, this may be the earliest case of MPNST with sphenoid metastases detected by 18-fluorodeoxyglucose positron-emission computed tomography scan and a sporadic case of brain metastases reported in the world literature.
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