Efficacy of Paclitaxel in a Patient with Inoperable Pulmonary Vein Leiomyosarcoma

Pulmonary vein leiomyosarcoma is extremely rare and has a poor prognosis. Surgical resection with a wide margin seems to offer the only chance of cure. The role of adjuvant therapy is controversial, and the exact efficacy of chemotherapy has not been observed. In this report, we present an 18-year-old male patient with pulmonary vein leiomyosarcoma in whom the use of paclitaxel (PAX) proved to be effective. Because the tumor originated from the left superior pulmonary vein and diffused into the left atrial wall and the junction of the right superior pulmonary vein and left atrium, the en bloc excision of the tumor was impossible. The first-line chemotherapy, including ifosfamide, doxorubicin, and dacarbazine, in conjugation with radiation therapy could not demonstrate any effect on the tumor size. However, the following PAX-containing regimen provided complete regression of the tumor. After PAX-based high-dose chemotherapy with autologous peripheral blood stem cell transplantation, the patient showed complete remission for 2 years. Although he suffered metastatic recurrences and died 4 years after the onset of symptoms, our patient’s clinical course clearly reveals the efficacy of PAX.

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Perforated High-Grade Mixed Neuroendocrine Nonneuroendocrine Neoplasm of Cecum: Unusual Presentation of Rare Disease

Gastrointestinal Tumors ◽

10.1159/000512237 ◽

2021 ◽

pp. 1-6

Author(s):

Gunasekaran Gopalakrishnan ◽

Bheemanathi Hanuman Srinivas ◽

Biju Pottakkat ◽

Senthil Gnanasekaran ◽

Raja Kalayarasan

Keyword(s):

Rare Case ◽

Histopathological Examination ◽

Psoas Muscle ◽

Right Hemicolectomy ◽

Resected Specimen ◽

Radiological Findings ◽

En Bloc ◽

En Bloc Excision ◽

Bloc Excision ◽

The Right

Mixed neuroendocrine nonneuroendocrine neoplasms (MiNENs) are rare neoplasms of the gastrointestinal tract, where the neuroendocrine as well as the nonneuroendocrine components each comprise at least 30% of the tumor. Of all cases of colorectal malignancies, MiNENs constitute around 3–9.6%, with only a few cases reported to be arising in the cecum. Since majority present with nonspecific clinical and radiological findings, its diagnosis preoperatively is almost impossible and these are usually diagnosed after histopathological examination of the resected specimen. Owing to the rarity of these tumors as well as lack of complete molecular characterization, optimal treatment remains unestablished. We, here, report a rare case of MiNENs of the cecum infiltrating the right psoas muscle and presenting with perforation for which right hemicolectomy and en bloc excision of the involved psoas muscle was done followed by adjuvant chemotherapy.

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Recurrence of a complex anterior skull base tumour managed with an uncommon surgical approach

BMJ Case Reports ◽

10.1136/bcr-2018-228622 ◽

2019 ◽

Vol 12 (3) ◽

pp. e228622

Author(s):

Muhammad Ibaad ur Rehman Alvi ◽

Omar Pathmanaban ◽

Raj Bhalla ◽

Navin Mani

Keyword(s):

Visual Fields ◽

Three Dimensional ◽

Endoscopic Biopsy ◽

Low Grade ◽

En Bloc ◽

Craniofacial Resection ◽

Primary Surgery ◽

En Bloc Excision ◽

Bloc Excision ◽

The Right

A well 74 -year-old woman presented to routine ophthalmology clinic instituted following treatment of a frontoethmoidal sarcoma initially excised in 1989 and diagnosed then as a sinus mucosal melanoma. At review in ophthalmology clinic, a reduction in right visual fields was noted. CT scan showed recurrence of a mass now involving the frontoethmoidal region, frontal sinus and abutting the cribiform plate. Endoscopic biopsy confirmed the recurrence as a low-grade biphenotypic sarcoma. This was discussed at the sarcoma multidisciplinary meeting. Using a three-dimensional printed model of the patients skull for planning, primary surgery with craniofacial resection combining intracranial and transfacial approaches with reconstruction was decided on. The implications of no treatment would be tumour involvement of the dura and brain as well as the right only-seeing eye. Craniofacial surgery would involve otolaryngolical, neurosurgical and maxillofacial multispecialty involvement and close teamwork. The goal was en bloc excision with negative surgical margins.

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Iliac wing osteochondroma in a 16-year-old boy: a case report

International Journal of Research in Orthopaedics ◽

10.18203/issn.2455-4510.intjresorthop20205585 ◽

2020 ◽

Vol 7 (1) ◽

pp. 171

Author(s):

Shrihari L. Kulkarni ◽

Sunil Mannual ◽

Manjunath S. Daragad ◽

Sharan H. Girirajaiah

Keyword(s):

Bone Tumour ◽

Neurovascular Compression ◽

Rare Entity ◽

En Bloc ◽

Iliac Wing ◽

Benign Bone Tumour ◽

Rare Site ◽

En Bloc Excision ◽

Bloc Excision ◽

The Right

<p class="abstract">Osteochondroma is the most common benign bone tumour. Commonly seen in the long bones of the lower extremity. Ilium is a rare site. We present a 16-year-old boy with swelling in the right groin for 8 months. After clinical and radiological evaluation, it was diagnosed as osteochondroma of right iliac wing. Patient underwent en-bloc excision of the lesion and histopathology confirmed the diagnosis. Pelvic osteochondroma is a rare entity but not unusual. Extra periosteal en-bloc excision is the management of choice in patients with cosmetic deformity, neurovascular compression and malignant transformation with very low recurrence rate.</p>

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